Peutz Jeghers syndrome
Related Terms
Cancer of the gastrointestinal tract, gastrointestinal polyps, GI polyps, gynecomastia, hamartomas, hereditary intestinal polyposis syndrome, Hutchinson Weber-Peutz syndrome, intestinal cancer, intestinal hamartomatous polyps in association with mucocutaneous melanocytic macules, intestinal lesions, intestinal polyposis-cutaneous pigmentation syndrome, intestinal polyps, mucocutaneous melanocytic macules, periorificial lentiginosis syndrome, Peutz-Jeghers polyposis, PJS, polyposis hamartomatous intestinal, polyposis intestinal II, polyps and spots syndrome, polyps-and-spots syndrome, serine/threonine kinase 11 gene, Sertoli cell tumors, STK11 gene mutation, STK11/LKB1 gene mutation, tumor suppressor gene.
Background
Peutz Jeghers syndrome (PJS) is a rare genetic condition marked by the development of multiple noncancerous polyps, or small growths, on the lining of the gastrointestinal tract. These polyps, called hamartomas, occur most frequently in the small intestine but are also found in the stomach and large intestine. Other symptoms of PJS include discoloration of the skin around the eyes, nose, mouth, anus, hands, and feet. People with PJS are at increased risk of developing intestinal and other cancers. The average age of diagnosis is 23 years. However, symptoms can be identified at birth.
It is believed that PJS is caused by mutations or defects in the serine/threonine kinase 11 gene (STK11 or STK11/LKB1), which plays a role in the suppression of tumor growth. The STK11 gene provides instructions for making the STK11 protein. The signaling pathway of the STK11 gene product has not yet been identified. Being a tumor suppressor gene means that the overexpression of STK11 may result in cell growth arrest. When the gene is mutated, the structure or function of the STK11 protein is altered or impaired. In some individuals with PJS, the STK11 gene is not mutated. In these individuals, the cause of the disease is unknown.
About half of PJS cases are inherited, or passed down among family members. In the other half, individuals have no family history of the disorder. These cases result from a spontaneous genetic mutation in the egg, sperm cells, or developing embryo.
The exact prevalence of PJS is unknown, but it is estimated to occur in about 1 in 25,000-300,000 births in the United States. The international prevalence of PJS (outside of the United States) is unknown. PJS does not appear to affect one sex or ethnicity more than another.
About half of those with PJS die of cancer-related complications by age 60. Other individuals with PJS may have a normal life expectancy. Given that PJS is a genetic disorder, there is no cure. Treatment aims to reduce symptoms and prevent complications.
Signs and symptoms
General: Peutz Jeghers syndrome (PJS) is a rare genetic condition marked by the development of multiple noncancerous polyps, or small growths, on the lining of the gastrointestinal tract. These polyps, called hamartomas, occur most frequently in the small intestine but are also found in the stomach and large intestine. Other symptoms of PJS include discoloration of the skin around the eyes, nose, mouth, anus, hands, and feet. People with PJS are at increased risk of developing intestinal and other cancers.
Skin discoloration: About 95% of people with PJS develop discoloration of the skin in and around the mouth (i.e., the lips, gums, and hard palate), near the nostrils and eyes, around the anus, and on the hands and feet. This discoloration, called mucocutaneous melanocytic macules, usually looks like small, flat, brown or dark blue freckle-like growths. They tend to appear during childhood and generally fade after puberty.
Polyps: Most people with PJS develop polyps, or noncancerous tumors, in the small intestine, but they have also been detected in the stomach and large intestine. These polyps tend to be noncancerous hamartomas. The development of polyps can vary dramatically among patients, and even among members of the same family. Most people with PJS during childhood and adolescence develop multiple polyps in the stomach and intestines. Polyps can cause medical problems such as recurrent bowel obstructions, chronic bleeding, and abdominal pain. There may be a significant risk of these polyps becoming cancerous. Some studies link PJS and cancers of the gastrointestinal tract, lung, breast, uterus, and ovaries.
Other: Females with PJS may enter puberty early and have irregular or heavy menstrual periods for unclear reasons. Males with PJS may develop testicular tumors called Sertoli cell tumors. These tumors secrete the female hormone estrogen, which can lead to the development of breast tissue in males.
Diagnosis
General: Peutz Jeghers syndrome (PJS) is a rare genetic condition marked by the development of multiple noncancerous polyps, or small growths, on the lining of the gastrointestinal tract. These polyps, called hamartomas, occur most frequently in the small intestine but are also found in the stomach and large intestine. Other symptoms of PJS include discoloration of the skin around the eyes, nose, mouth, anus, hands, and feet. People with PJS are at increased risk of developing intestinal and other cancers. The average age at diagnosis is about 23 years in males and about 26 years in females. Skin discoloration, which tends to develop in early childhood, may make earlier diagnosis of PJS possible. Signs and symptoms that may point to a diagnosis of PJS include a family history of the disorder, unexplained abdominal pain and unexplained gastrointestinal bleeding in young people, irregular menstruation in females, development of breast tissue in males, early puberty, and bowel obstruction. In addition, females should receive a thorough gynecological and breast exam, including mammography, as some studies link PJS and cancers of the breast, uterus, and ovaries, while males should undergo screening for testicular abnormalities.
Physical exam: Individuals suspected of having PJS should receive a thorough physical exam and provide a complete family history, including any history of cancer. Signs and symptoms that may point to a diagnosis of PJS include a family history of the disorder, unexplained abdominal pain and gastrointestinal bleeding in young people, irregular menstruation in females, development of breast tissue in males, early puberty, and bowel obstruction.
Dermatological exam: A dermatologist or skin specialist can evaluate the skin discolorations known as mucocutaneous melanocytic macules, which usually look like small, flat, brown or dark blue freckle-like growths in and around the mouth (i.e., the lips, gums, and hard palate), near the nostrils and eyes, around the anus, and on the hands and feet.
Blood tests: Samples of blood may be taken to measure hematocrit, the proportion of blood volume occupied by red blood cells and iron level. These values may be low if blood is being lost because of unexplained gastrointestinal bleeding, which has been known to occur in patients younger than 25 years of age, and which can cause anemia.
Fecal occult blood test: A fecal occult blood test (FOBT) may be done if gastrointestinal bleeding is suspected. This test analyzes a stool sample for the presence of blood because unexplained gastrointestinal bleeding has occurred in some patients younger than 25 years of age.
Carcinoembryonic antigen test: The carcinoembryonic antigen (CEA) test has been used by clinicians to detect and monitor the development of noncancerous growths due to their potential to become cancerous. This test measures blood levels of the CEA protein, which is normally produced during fetal development and is not generally present in the blood of healthy adults. Presence of CEA in the blood may indicate the presence and severity of certain cancers, changes in cancer following the use of medications or surgery, and the return of cancer.
Imaging studies: Upper and lower endoscopies, in which a scope is introduced via the mouth or anus to examine the inside of the gastrointestinal system, may also be performed to assess the presence and severity of polyps.
Genetic testing: If PJS is suspected, DNA sequencing may be performed to confirm a diagnosis. A sample of the patient's blood is taken and analyzed in a laboratory for the defect in the STK11 gene. If this defect is detected, a positive diagnosis is made.
Prenatal DNA testing: If there is a family history of PJS, prenatal testing may be performed to determine whether the fetus has the disorder. Amniocentesis and chorionic villus sampling (CVS) may be able to diagnose PJS. However, because there are serious risks associated with these tests, patients should discuss the potential health benefits and risks with a medical professional.
During amniocentesis, a long, thin needle is inserted through the abdominal wall and into the uterus, and a small amount of amniotic fluid is removed from the sac surrounding the fetus. Cells in the fluid are then analyzed for normal and abnormal chromosomes. This test is performed after 15 weeks of pregnancy. The risk of miscarriage is about one in 200-400 patients. Some patients may experience minor complications, such as cramping, leaking fluid, or irritation where the needle was inserted.
During chorionic villus sampling (CVS), a small piece of tissue (chorionic villi) is removed from the placenta between the ninth and 14th weeks of pregnancy. CVS may be performed through the cervix or through the abdomen. The cells in the tissue sample are then analyzed for the mutation in the STK11 gene. Miscarriage occurs in about 0.5-1% of women who undergo this procedure.
Complications
General: There is no cure for Peutz Jeghers syndrome (PJS). Instead, treatment aims to reduce symptoms and prevent complications. Patients with PJS should be regularly be seen by a pediatrician, dermatologist, gastroenterologist, oncologist, and various surgeons.
Anemia: Bleeding of gastrointestinal polyps may cause persistent bleeding and eventually lead to anemia, a blood disorder characterized by low levels of red blood cells.
Bowel obstruction: Almost half of those with PJS develop bowel obstruction caused by the polyps that result from the disorder. The severity of bowel obstruction may depend on the size and location of the polyps, and generally occurs in the small intestine.
Cancer: Individuals with PJS are about 15 times more likely than the general population to develop cancer during their lifetime. About half those with PJS develop cancer and die by age 60, and the cumulative risk of developing cancer is estimated to be 93% by 64 years of age. Cancers in PJS typically occur in the gastrointestinal tract (i.e., the stomach, colon, small intestine, and esophagus), breast, ovaries, lung, cervix, testes, and uterus.
Sertoli cell cancer in male patients with PJS
may result in the production of estrogen, thus causing feminization. Symptoms associated with Sertoli cell cancer include prominent external genitalia, pubic hair growth, accelerated skeletal and muscle development, and mature masculine voice in prepubertal males. Males may also show feminizing characteristics such as gynecomastia (breast tissue formation) and breast tenderness. Loss of libido, erectile dysfunction, and infertility have all been reported.
Pain: About one-quarter of individuals with PJS have abdominal pain caused by gastrointestinal polyps.
Treatment
General: There is no cure for Peutz Jeghers syndrome (PJS). Instead, treatment aims to reduce symptoms and prevent complications. Depending on individual symptoms and complications, individuals with PJS should be regularly seen by a geneticist, gynecologist, oncologist, dermatologist, and gastroenterologist.
Cancer treatment: People with PJS who develop cancer should receive standard cancer treatment. This often includes radiation and chemotherapy. By damaging the deoxyribonucleic acid (DNA) in the target cells, radiation therapy stops the growth of cancerous cells. While both normal and cancer cells are damaged by radiation, normal cells can typically recover, while cancer cells cannot. The goal of radiation therapy is to maximize damage to cancer cells and minimize damage to normal cells. About half of those with cancer receive radiation therapy in some form. Side effects of radiation therapy depend on the site of the radiation target and the type of cancer. For example, nausea, vomiting, and diarrhea may result from radiation therapy targeted at the stomach and related areas. Radiation may be used alone or in combination with other therapies, such as chemotherapy, which involves the use of drugs to stop cancerous cell growth. Again, however, chemotherapy can damage normal cells as well as cancerous cells. Side effects of chemotherapy may include nausea, vomiting, fatigue, pain, and loss of hair on the head and body.
Iron supplementation: Iron supplementation may be prescribed to individuals who develop iron deficiency anemia, a blood disorder characterized by low levels of red blood cells.
Surgery: Surgical removal of large polyps may be appropriate to reduce pain, bleeding, and risk of bowel obstruction. Surgery is also an option for the removal of cancerous growths.
Integrative therapies
Note: Currently there is a lack of scientific evidence on the use of integrative therapies for the treatment or prevention of Peutz Jeghers syndrome (PJS). The therapies listed below have been studied for cancer in general or anemia, should be used only under the supervision of a qualified healthcare provider, and should not be used in replacement of other proven therapies.
Strong scientific evidence:
Iron:
Iron is an essential mineral and an important component of proteins involved in oxygen transport and metabolism. Ferrous sulfate (Feratab?, Fer-Iron?, Slow-FE?) is the standard treatment for treating iron deficiency anemia.
Iron is a trace mineral, and hypersensitivity is unlikely. Avoid with known allergy or hypersensitivity to products containing iron. Avoid excessive intake. Avoid with blood disorders that require frequent blood transfusions. Use cautiously with history of kidney disease, intestinal disease, peptic ulcer disease, enteritis, colitis, pancreatitis, hepatitis, or alcoholism; and in patients who plan to become pregnant, or those who are over age 55 and have a family history of heart disease. Pregnant or breastfeeding women should consult a healthcare professional before beginning iron supplementation.
Good scientific evidence:
Greater celandine: UkrainT, a semisynthetic drug derived from greater celandine (
Chelidonium majus), has been studied in clinical trials of various types of cancer in general, with consistently positive outcomes. However, the quality of the research performed to date is inadequate, and higher-quality studies are needed.
Use cautiously in patients taking amphetamines, morphine, hexobarbital, MAOIs, or dopaminergic or serotonergic drugs, or in patients undergoing radiation therapy. Avoid in patients with liver disease or in pregnant and lactating women.
Guided imagery: Early research suggests that guided imagery may help reduce cancer pain. Further research is needed to confirm these results.
Guided imagery is usually intended to supplement medical care, not to replace it, and guided imagery should not be relied on as the sole therapy for a medical problem. Contact a qualified healthcare provider if mental or physical health is unstable or fragile. Never use guided imagery techniques while driving or doing any other activity that requires strict attention. Use cautiously with physical symptoms that can be brought about by stress, anxiety, or emotional upset, because imagery may trigger these symptoms. For those feeling unusually anxious while practicing guided imagery, or those with a history of trauma or abuse, speak with a qualified healthcare provider before practicing guided imagery.
Meditation: There is good evidence that various types of meditation may help improve quality of life in cancer patients. Studies have shown benefits for mood, sleep quality, and the stresses of treatment. The specific effects of meditation are not fully understood. Additional research is needed in this area.
Use cautiously with underlying mental illnesses. People with psychiatric disorders should consult with their primary mental healthcare professionals before starting a program of meditation, and they should explore how meditation may or may not fit in with their current treatment plan. Avoid with risk of seizures. The practice of meditation should not delay the time to diagnosis or treatment with more proven techniques or therapies and should not be used as the sole approach to illnesses.
Psychotherapy: Psychotherapy is an interactive process between a person and a qualified mental healthcare professional (psychiatrist, psychologist, clinical social worker, licensed counselor, or other trained practitioner). There is good evidence that psychotherapy may enhance quality of life in cancer patients by reducing emotional distress and aiding in coping with the stresses and challenges of cancer. Therapy may be supportive-expressive therapy, cognitive therapy, or group therapy. While some patients seek psychotherapy in hopes of extending survival, conclusive evidence of its effects on medical prognosis is currently lacking. Psychotherapy may help people come to terms with the fact that they may die of cancer, which is the fourth stage of dealing with a terminal illness (the stages are denial, anger, bargaining, and acceptance).
Psychotherapy is not always sufficient to resolve mental or emotional conditions. Psychiatric medication is sometimes needed. The reluctance to seek and use appropriate medication may contribute to worsening of symptoms or increased risk for poor outcomes. In order to be successful, psychotherapy requires considerable personal motivation and investment in the process. This includes consistent attendance and attention to treatment recommendations provided by the practitioner. Not all therapists are sufficiently qualified to work with all problems. The client or patient should seek referrals from trusted sources and should also inquire of the practitioner's training and background before committing to work with a particular therapist. Some forms of psychotherapy evoke strong emotional feelings and expression. This can be disturbing for people with serious mental illness or some medical conditions. Psychotherapy may help with postpartum depression but is not a substitute for medication, which may be needed in severe cases.
Yoga: Yoga is an ancient system of relaxation, exercise, and healing with origins in Indian philosophy. Several studies report enhanced quality of life in cancer patients, lower sleep disturbance, decreased stress symptoms, and changes in cancer-related immune cells after patients received relaxation, meditation, and gentle yoga therapy. Yoga is not recommended as a sole treatment for cancer but may be helpful as an adjunct therapy.
Yoga is generally considered to be safe in healthy individuals when practiced appropriately. Avoid some inverted poses with disc disease of the spine, fragile or atherosclerotic neck arteries, risk for blood clots, extremely high or low blood pressure, glaucoma, detachment of the retina, ear problems, severe osteoporosis, or cervical spondylitis. Certain yoga breathing techniques should be avoided in people with heart or lung disease. Use cautiously with a history of psychotic disorders. Yoga techniques are believed to be safe during pregnancy and breastfeeding when practiced under the guidance of expert instruction (the popular Lamaze techniques are based on yogic breathing). However, poses that put pressure on the uterus, such as abdominal twists, should be avoided in pregnancy.
Unclear or conflicting scientific evidence:
Acupuncture: Acupuncture, or the use of needles to manipulate the "qi" (or "chi"), or body energy, originated in China over 5,000 years ago. There has been limited research on acupuncture for cancer pain, and that research showed mixed results. More studies are needed to determine potential benefits. Evidence from several small studies supports the use of acupuncture at a specific point on the wrist (P6) to help chemotherapy patients reduce nausea and vomiting. Acupuncture may also reduce the pain associated with cancer.
Needles must be sterile in order to avoid disease transmission. Avoid with valvular heart disease, infections, bleeding disorders, medical conditions of unknown origin, or neurological disorders, or with anticoagulants (drugs that increase the risk of bleeding). Avoid on areas that have received radiation therapy. Avoid during pregnancy. Use cautiously with pulmonary disease, such as asthma or emphysema. Use cautiously in elderly or medically compromised patients, diabetics, or those with a history of seizures. Avoid electroacupuncture with arrhythmia (irregular heartbeat) or in patients with pacemakers.
Aloe: Transparent gel from the pulp of the meaty leaves of
Aloe vera has been used on the skin for thousands of years to treat wounds, skin infections, burns, and numerous other skin conditions. Dried latex from the inner lining of the leaf has traditionally been used as an oral laxative. Preliminary research suggests that aloe may help in the area of cancer prevention or may aid in the regression of cancerous tumors. Additional research is needed in this area.
Caution is advised when taking aloe supplements, as numerous adverse effects, including a laxative effect, cramping, dehydration, and drug interactions, are possible. Aloe should not be used if pregnant or breastfeeding, unless otherwise directed by a doctor.
American pawpaw: Evidence supporting the use of the American pawpaw (
Asimina triloba) tree for cancer treatment in humans is largely anecdotal and subjective. However, use in humans has reported minimal side effects, and evidence from animal and test tube studies suggest that American pawpaw extract does have some anticancer activity. Pawpaw standardized extract has been used for 18 months in patients with various forms of cancer. Well-designed studies on the long-term effects of pawpaw extracts are currently lacking. Pawpaw should not be used if pregnant or breastfeeding, unless otherwise directed by a doctor.
Antineoplastons: Antineoplastons are a group of naturally occurring peptide fractions, which were observed by Stanislaw Burzynski, MD, PhD, in the late 1970s to be absent in the urine of cancer patients. There is inconclusive scientific evidence regarding the effectiveness of antineoplastons in the treatment of cancer. Several preliminary human studies (case series, phase I/II trials) have examined antineoplaston types A2, A5, A10, AS2-1, and AS2-5 for a variety of cancer types. It remains unclear if antineoplastons are effective, or what doses may be safe. Until better research is available, no clear conclusion can be drawn.
Avoid if allergic or hypersensitive to antineoplastons. Use cautiously with high medical or psychiatric risk, an active infection due to a possible decrease in white blood cells, high blood pressure, heart conditions, chronic obstructive pulmonary disease, liver disease or damage, or kidney disease or damage. Avoid if pregnant or breastfeeding.
Arabinoxylan: Arabinoxylan is made by altering the outer shell of rice bran using enzymes from
Hyphomycetesmycelia mushroom extract. Arabinoxylan has been found to improve immune reactions in patients with cancer of various types. Arabinoxylan products may contain high calcium and phosphorus levels, which may be harmful for patients with compromised renal (kidney) function.
Caution is advised when taking arabinoxylan supplements, as numerous adverse effects, including drug interactions, are possible. Arabinoxylan should not be used if pregnant or breastfeeding, unless otherwise directed by a doctor.
Arginine: A combination of arginine and omega-3 fatty acids may reduce the length of hospital stays and infections for patients after gastrointestinal cancer surgery. Other research suggests that arginine, omega-3 fatty acids, and glutamine may boost the immune system and reduce inflammation after surgery. More research with arginine alone is needed.
Avoid if allergic to arginine, or with a history of stroke or liver or kidney disease. Avoid if pregnant or breastfeeding. Use caution if taking blood-thinning drugs (like warfarin or Coumadin?), blood pressure drugs, or herbs or supplements with similar effects. Check blood potassium levels. L-arginine may worsen symptoms of sickle cell disease. Caution is advised in patients taking prescription drugs to control sugar levels.
Aromatherapy: Healing with fragrant oils has been used for thousands of years. Aromatherapy is often used in people with chronic illnesses (frequently in combination with massage), with the intention to improve quality of life or well-being. There is currently not enough scientific evidence to form a firm conclusion about the effectiveness of aromatherapy for quality of life in cancer.
Essential oils should only be used on the skin in areas without irritation. Essential oils should be administered in a carrier oil to avoid toxicity. Avoid with a history of allergic dermatitis. Use cautiously if driving or operating heavy machinery. Avoid consuming essential oils. Avoid direct contact of undiluted oils with mucous membranes. Use cautiously if pregnant.
Art therapy: Art therapy involves the application of a variety of art modalities including drawing, painting, clay, and sculpture. Art therapy enables the expression of inner thoughts or feelings when verbalization is difficult or not possible. Limited evidence suggests that art therapy may be of benefit in cancer caregiving for families of cancer patients. Possible benefits include reduced stress, lowered anxiety, increased positive emotions, and increased positive communication with cancer patients and healthcare professionals. Art therapy may also reduce pain and other symptoms in cancer patients. More studies are needed to determine how best to use this form of intervention with this population.
Art therapy may evoke distressing thoughts or feelings. Use under the guidance of a qualified art therapist or other mental health professional. Some forms of art therapy use potentially harmful materials. Only materials known to be safe should be used. Related clean-up materials (like turpentine or mineral spirits) that release potentially toxic fumes should only be used with good ventilation.
Astragalus: Astragalus (
Astragalus membranaceus) has been used in Chinese medicine for centuries for its immune-enhancing properties. Although early laboratory and animal studies report immune stimulation and reduced cancer cell growth associated with the use of astragalus, reliable human evidence in these areas is currently lacking. In Chinese medicine, astragalus-containing herbal mixtures are also sometimes used with the intention to reduce side effects of chemotherapy and other cancer treatments. Due to a lack of well-designed research, a firm conclusion cannot be drawn.
Caution is advised when taking astragalus supplements, as numerous adverse effects, including drug interactions, are possible. Astragalus should not be used if pregnant or breastfeeding, unless otherwise directed by a doctor.
Baikal skullcap: Although the outcomes of early studies using Baikal skullcap for cancer are promising, high-quality clinical studies are needed in this area before a conclusion can be made. Avoid if allergic or hypersensitive to Baikal skullcap (
Scutellaria barbata), its constituents, or members of the Lamiaceae family. Use cautiously if taking sedatives and/or operating heavy machinery. Use cautiously if taking antineoplastic (anticancer) agents or agents metabolized by cytochrome P450 enzymes. Avoid if pregnant or breastfeeding. Baikal skullcap is an ingredient in PC-SPES, a product that has been recalled from the U.S. market and should not be used.
Bee pollen: Bee pollen is considered a highly nutritious food, because it contains a balance of vitamins, minerals, proteins, carbohydrates, fats, enzymes, and essential amino acids. Research has found that bee pollen may reduce some adverse effects of cancer treatment side effects. Additional research is needed before a firm recommendation can be made.
Caution is advised when taking bee pollen supplements, as allergic reactions may occur in sensitive individuals. Bee pollen should not be used if pregnant or breastfeeding, unless otherwise directed by a doctor.
Beta-glucan: Treatment with a beta-glucan, called lentinan, plus chemotherapy (S-1) may help prolong the lives of patients with cancer that has returned or cannot be operated on. More research is needed in this area.
Avoid if allergic or hypersensitive to beta-glucan. When taken by mouth, beta-glucan is generally considered safe. Use cautiously with AIDS or AIDS-related complex (ARC). Avoid using particulate beta-glucan. Avoid if pregnant or breastfeeding.
Bitter melon: Bitter melon (
Momordica charantia) is used in Ayurvedic (Indian) medicine to lower blood sugar levels. Research has also found that bitter melon extracts may be beneficial in cancer therapies. Laboratory studies have reported that MAP30, a protein isolated from bitter melon extract, possesses anticancer effects. Potential anticancer effects have not been studied appropriately in humans.
Caution is advised when taking bitter melon supplements, as numerous adverse effects, including blood sugar lowering and drug interactions, are possible. Bitter melon should not be used if pregnant or breastfeeding, unless otherwise directed by a doctor.
Black tea: Black tea (
Camellia sinensis) is from the same plant as green tea, but the leaves are processed differently. Black tea usually contains more caffeine than green tea. Several studies have explored a possible association between regular consumption of black tea and rates of cancer in several populations. This research has yielded conflicting results, with some studies suggesting benefits and others reporting no effects. Laboratory and animal studies report that components of tea, such as polyphenols, have antioxidant properties and effects against tumors. However, their effects in humans remain unclear, and these components may be more common in green tea than in black tea. Some animal and laboratory research suggests that components of black tea may actually be carcinogenic, or cancer causing, although effects in humans are not clear. Overall, the relationship of black tea consumption and human cancer prevention remains undetermined.
Although there is strong evidence from animal and laboratory studies that black tea may help prevent colorectal cancer, human studies are limited. Additional research is needed.
Avoid if allergic or hypersensitive to caffeine or tannins. Skin rash and hives have been reported with caffeine ingestion. Use caution with diabetes. Use cautiously if pregnant. Heavy caffeine intake during pregnancy may increase the risk of sudden infant death syndrome (SIDS). Very high doses of caffeine have been linked with birth defects. Caffeine is transferred into breast milk. Caffeine ingestion by infants can lead to sleep disturbances or insomnia. Infants nursing from mothers consuming greater than 500 milligrams of caffeine daily have been reported to experience tremors and heart rhythm abnormalities. Tea consumption by infants has been linked to anemia, decreased iron metabolism, and irritability.
Bovine cartilage: In early research, bovine tracheal cartilage (preparations such as Catrix? and VitaCarte?) has been studied for the treatment of cancer, with encouraging results. High-quality clinical research is needed to better determine the effectiveness of bovine tracheal cartilage preparations for cancer treatment.
Avoid if allergic or hypersensitive to bovine cartilage or any of its constituents. Use cautiously with cancer, renal (kidney) failure, or hepatic (liver) failure. Avoid if pregnant or breastfeeding.
Bromelain: Bromelain is a sulfur-containing digestive enzyme (a protein which helps with digestion) that is extracted from the stem and the fruit of the pineapple plant (
Ananas comosus)
. There is not enough information to recommend for or against the use of bromelain in the treatment of cancer, either alone or in addition to other therapies. One small study found that a bromelain supplement decreased tumor size in 12 breast cancer patients. Patients took the supplements for different periods of time, lasting from months to years.
Caution is advised when taking bromelain supplements, as numerous adverse effects, including blood thinning and drug interactions, are possible. Bromelain should not be used if pregnant or breastfeeding, unless otherwise directed by a doctor.
Calcium: Calcium is the most abundant mineral in the human body and has several important functions. Most large, prospective studies have found increased calcium intake to be only weakly associated with a decreased risk of colorectal cancer. Further studies are needed to verify these results.
Avoid if allergic or hypersensitive to calcium or lactose. High doses taken by mouth may cause kidney stones. Avoid with high levels of calcium in the blood, high levels of calcium in urine, hyperparathyroidism (overgrowth of the parathyroid glands), bone tumors, digitalis toxicity, ventricular fibrillation (rapid, irregular twitching of heart muscle), kidney stones, kidney disease, or sarcoidosis (inflammatory disease). Calcium supplements made from dolomite, oyster shells, or bone meal may contain unacceptable levels of lead. Use cautiously with achlorhydria or irregular heartbeat. Talk to a healthcare provider to determine appropriate dosing during pregnancy and breastfeeding.
Cat's claw: Originally found in Peru, the use of cat's claw (
Uncaria tomentosa) has been said to date back to the Inca civilization, possibly as far back as 2,000 years. Cat's claw has anti-inflammatory properties, and several low-quality studies suggest that cat's claw may slow tumor growth. However, this research is early and has not identified specific types of cancer that may benefit; thus, the results are not clear. A few studies suggest that cat's claw may also boost the immune system.
Caution is advised when taking cat's claw supplements, as numerous adverse effects, including blood thinning and drug interactions, are possible. Cat's claw should not be used if pregnant or breastfeeding, unless otherwise directed by a doctor.
Chaparral: Chaparral was used by the Native Americans for various health conditions. The chaparral component nordihydroguaiaretic acid (NDGA) has been evaluated as a treatment for cancer but, due to risk of toxicity, is considered unsafe and not recommended for use. Chaparral and NDGA have been associated with cases of kidney and liver failure, liver cirrhosis, kidney cysts, and kidney cancer in humans. In response to these reports, the U.S. Food and Drug Administration (FDA) removed chaparral from its "generally recognized as safe" (GRAS) list in 1970. Chaparral and NDGA are generally considered unsafe and are not recommended for use.
Avoid if allergic to chaparral or any of its components, including nordihydroguaiaretic acid. Use cautiously if taking anticoagulants (blood thinners), blood sugar medication, or drugs that are broken down by the liver (like amiodarone, phenobarbital, valproic acid). Stop use two weeks before surgery or dental or diagnostic procedures with bleeding risk, and do not use immediately after these procedures. Use cautiously if driving or operating heavy machinery. Avoid if pregnant or breastfeeding.
Chlorophyll: Preliminary evidence suggests that chlorophyll may aid in the reduction of side effects associated with photodynamic therapies, such as those used in the management of malignant tumors. Further research is required to support the use of chlorophyll as a laser therapy adjunct for cancer treatment.
Avoid if allergic or hypersensitive to chlorophyll or any of its metabolites. Use cautiously with photosensitivity, compromised liver function, diabetes, or gastrointestinal conditions or obstructions. Use cautiously if taking immunosuppressant agents or antidiabetes agents. Avoid if pregnant or breastfeeding.
Coenzyme Q10: Further research is needed to determine if coenzyme Q10 (CoQ10) may be of benefit for cancer when used with other therapies.
Allergy associated with coenzyme Q10 supplements has not been reported, although rash and itching have been reported rarely. Stop use two weeks before surgery or dental or diagnostic procedures with bleeding risk, and do not use immediately after these procedures. Use caution with a history of blood clots, diabetes, high blood pressure, heart attack, or stroke, or with anticoagulants (blood thinners), antiplatelet drugs (like aspirin, warfarin, clopidogrel (like Plavix?), or blood pressure, blood sugar, cholesterol or thyroid drugs. Avoid if pregnant or breastfeeding.
Copper: Copper is a mineral that occurs naturally in many foods, including vegetables, legumes, nuts, grains, and fruits (especially avocado), as well as shellfish and beef (organs such as liver). Preliminary research reports that lowering copper levels theoretically may arrest the progression of cancer by inhibiting angiogenesis (blood vessel growth). Copper intake has not been identified as a risk factor for the development or progression of cancer.
Copper is potentially unsafe when used orally in higher doses than the recommended dietary allowance (RDA). Copper supplements should not be used if pregnant or breastfeeding, unless otherwise directed by a doctor.
Cranberry: Several laboratory studies have reported positive effects of proanthocyanidins, flavonoid components of cranberry (
Vaccinium macrocarpon) and other fruits such as blueberries, grape seed, and pomegranate, on health. According to early laboratory research, cranberry has been proposed for cancer prevention. Additional research is needed in humans before a conclusion can be made.
Avoid if allergic to cranberries, blueberries, or other plants of the Vaccinium species. Sweetened cranberry juice may affect blood sugar levels. Use cautiously with a history of kidney stones. Avoid more than the amount usually found in foods if pregnant or breastfeeding.
Dandelion: Limited animal research does not provide a clear assessment of the effects of dandelion on tumor growth. Well-conducted human studies are needed to better determine dandelion's effects on cancer.
Avoid if allergic to chamomile, feverfew, honey, yarrow, or any related plants, such as aster, daisies, sunflower, chrysanthemum, mugwort, ragweed, or ragwort. Use cautiously with diabetes, bleeding disorders, gastroesophageal reflux disease (GERD), kidney or liver diseases, or a history of stroke or electrolyte disorders. Monitor potassium blood levels. Stop use two weeks before surgery or dental or diagnostic procedures with bleeding risk, and do not use immediately after these procedures. Avoid if pregnant or breastfeeding.
Echinacea: There is currently a lack of clear human evidence that echinacea affects any type of cancer. The evidence from a small number of clinical trials evaluating efficacy of echinacea in the treatment of radiation-induced leukopenia (decrease in white blood cells) is equivocal. Studies have used the combination product Esberitox?, which includes extracts of echinacea (
Echinacea purpurea and
pallida) root, white cedar (
Thuja occidentalis) leaf, and wild indigo (
Baptisia tinctoria)
root. Additional clinical studies are needed to make a conclusion.
Caution is advised when taking echinacea supplements, as numerous adverse effects, including drug interactions, are possible. Echinacea should not be used if pregnant or breastfeeding, unless otherwise directed by a doctor.
Essiac?: Essiac? contains a combination of herbs, including burdock root (
Arctium lappa), sheep sorrel (
Rumex acetosella), slippery elm inner bark (
Ulmus fulva), and Turkish rhubarb (
Rheum palmatum). The original formula was developed by the Canadian nurse Rene Caisse (1888-1978) and is thought to be effective in cancer therapies, although currently, there is not enough evidence to recommend for or against the use of this herbal mixture as a therapy for any type of cancer. Different brands may contain variable ingredients, and the comparative effectiveness of these formulas is not known. None of the individual herbs used in Essiac? has been tested in rigorous human cancer trials, although some components have shown antitumor activity in laboratory studies. Numerous individual patient testimonials and reports from manufacturers are available on the Internet, although these cannot be considered scientifically viable as evidence. Individuals with cancer are advised not to delay treatment with more proven therapies.
Caution is advised when taking Essiac? supplements, as numerous adverse effects, including drug interactions, are possible. Essiac? should not be used if pregnant or breastfeeding, unless otherwise directed by a doctor.
Focusing: Focusing (experiential therapy) is a method of psychotherapy that involves being aware of one's feelings surrounding a particular issue and understanding the meaning behind words or images conveyed by those feelings. Early evidence suggests that focusing may improve mood and attitude in cancer patients. Firm recommendations cannot be made until well-designed clinical trials are available.
Side effect reporting is rare, but patients should consult with a qualified healthcare practitioner before making decisions about medical conditions and practices. Individuals with severe emotional difficulties should not abandon proven medical and psychological therapies but rather choose focusing as a possible adjunct.
Folic acid: Folic acid, or folate, is a form of a water-soluble B vitamin needed for human health. Preliminary evidence suggests that folate may decrease the risk of several types of cancer. Additional research is needed to make a conclusion. Folic acid supplementation may mask the symptoms of pernicious, aplastic, or normocytic anemias caused by vitamin B12 deficiency and may lead to neurological damage.
Avoid if allergic or hypersensitive to folate or any folate product ingredients. Use cautiously if receiving coronary stents and with anemia and seizure disorders. It is recommended that pregnant women consume 400 micrograms daily in order to reduce the risk of fetal defects. Folate is likely safe if breastfeeding.
Gamma-linolenic acid (GLA): GLA is an omega-6 essential fatty acid. Some laboratory and human studies indicate that GLA may have antitumor activity and may be used as a cancer treatment adjunct. Additional research is needed in this area.
Caution is advised when taking GLA supplements, as numerous adverse effects, including an increased risk of bleeding and drug interactions, are possible. GLA should not be used if pregnant or breastfeeding, unless otherwise directed by a doctor.
Garlic: Preliminary human studies suggest that regular consumption of garlic (
Allium sativum) supplements may reduce the risk of developing several types of cancer. Some studies use multi-ingredient products, so it is difficult to determine if garlic alone may play a beneficial role in cancer prevention. Further well-designed human clinical trials are needed to conclude whether eating garlic or taking garlic supplements may prevent or treat cancer.
Caution is advised when taking garlic supplements, as numerous adverse effects, including an increased risk of bleeding and drug interactions, are possible. Garlic should not be used if pregnant or breastfeeding, unless otherwise directed by a doctor.
Ginkgo:
Ginkgo biloba exocarp polysaccharides (GBEP) capsule preparation has been studied for upper digestive tract malignant tumors of middle and late stages, with positive results. However, further research is needed to better understand the potential role of ginkgo for gastric cancer.
Avoid if allergic or hypersensitive to members of the Ginkgoaceaefamily.
If allergic to mango rind, sumac, poison ivy or oak, or cashews, then allergy to ginkgo is possible. Avoid with blood-thinners (like aspirin or warfarin (Coumadin?)) due to an increased risk of bleeding. Ginkgo should be stopped two weeks before surgical procedures. Ginkgo seeds are dangerous and should be avoided. Skin irritation and itching may also occur. Do not use ginkgo in supplemental doses if pregnant or breastfeeding.
Ginseng: Early studies report that ginseng taken by mouth may be of benefit in cancer prevention, especially if ginseng powder or extract is used. Early studies suggest that ginseng may decrease radiation therapy side effects and may be used as a chemotherapy adjunct to improve body weight, quality of life, and the immune response. There is currently not enough evidence to recommend the use of
Panax ginseng or American ginseng for these indications. Research results are unclear, and more research is needed before a clear conclusion can be reached.
Caution is advised when taking ginseng supplements, as numerous adverse effects, including an increased risk of drug interactions, are possible. Ginseng should not be used if pregnant or breastfeeding, unless otherwise directed by a doctor.
Goji: Polysaccharide constituents, such as alpha- and beta-glucans from a variety of plants, are reported to have immune system-enhancing properties. In clinical research,
Lycium barbarum polysaccharides (LBP) demonstrated a synergistic effect in various cancer treatments, when administered in conjunction with powerful immune stimulating drugs.
Use cautiously in patients who are taking blood-thinning medications, such as warfarin. Use cautiously in asthma patients and in patients with sulfite sensitivities. The New York Department of Agriculture has detected the presence of undeclared sulfites, a food additive, in two dried goji berry products from China. Avoid in patients who are allergic to goji, any of its constituents, or to members of the Solanaceae family.
Grape seed: There is currently little information available on the use of grape seed extract in the treatment of human cancer. Further research is needed before a recommendation can be made.
Avoid if allergic or hypersensitive to grapes or other grape compounds. Use cautiously with bleeding disorders or if taking blood thinners such as warfarin, aspirin, nonsteroidal anti-inflammatory drugs (NSAIDS), or antiplatelet agents. Use cautiously with drugs processed using the liver's cytochrome P450 enzyme system. Use cautiously with blood pressure disorders or if taking ACE inhibitors. Avoid if pregnant or breastfeeding.
Greater celandine: The majority of studies on the use of greater celandine (
Chelidonium majus) in the treatment of various types of cancer utilize the semisynthetic drug UkrainTM. Additional research is needed to better understand the role of this agent in patients with cancer.
Use cautiously in patients taking amphetamines, morphine, hexobarbital, MAOIs, or dopaminergic or serotonergic drugs, or in patients undergoing radiation therapy. Avoid in patients with liver disease or in pregnant or lactating women.
Green tea: Green tea is made from the dried leaves of
Camellia sinensis, a perennial evergreen shrub. Green tea has a long history of use in health and longevity, dating back to approximately 5,000 years ago in China. Although used for centuries to help prevent diseases, the relationship of green tea consumption and human cancer in general remains inconclusive. Evidence from well-designed clinical trials is needed before a firm conclusion can be made in this area.
Caution is advised when taking green tea supplements, as numerous adverse effects, including an increased risk of drug interactions, are possible. Green tea should not be used if pregnant or breastfeeding, unless otherwise directed by a doctor.
Healing touch: Preliminary data suggest that healing touch (HT) may increase quality of life in cancer patients. However, due to weaknesses in design and the small number of studies, the data are insufficient to make definitive recommendations. Studies with stronger designs are needed.
HT should not be regarded as a substitute for established medical treatments. Use cautiously if pregnant or breastfeeding.
Hoxsey formula: "Hoxsey formula" is a misleading name, because it is not a single formula, but rather a therapeutic regimen consisting of an oral tonic, topical (on the skin) preparations, and supportive therapy. The tonic is individualized for cancer patients based on general condition, the location of cancer, and previous history of treatment. An ingredient that usually remains constant for every patient is potassium iodide. Other ingredients are then added and may include licorice, red clover, burdock, stillingia root, berberis root, pokeroot, cascara, Aromatic USP 14, prickly ash bark, and buckthorn bark. A red paste may be used, which tends to be caustic (irritating) and contains antimony trisulfide, zinc chloride, and bloodroot. A topical yellow powder may be used and contains arsenic sulfide, talc, sulfur, and a "yellow precipitate." A clear solution may also be administered, which contains trichloroacetic acid.
Well-designed human studies available evaluating the safety or effectiveness of Hoxsey formula are currently lacking. Caution is advised when taking the Hoxsey formula supplements, as numerous adverse effects, including an increased risk of drug interactions, are possible. Hoxsey formula should not be used if pregnant or breastfeeding, unless otherwise directed by a doctor.
Hydrazine sulfate: Hydrazine is an industrial chemical marketed as having the potential to repress weight loss and cachexia (muscle wasting) associated with cancer, and to improve general appetite status. However, in large randomized controlled trials, hydrazine has not been proven effective for improving appetite, reducing weight loss, or improving survival in adults. The National Cancer Institute (NCI) sponsored studies of hydrazine sulfate that claimed efficacy in improving survival for some patients with advanced cancer. Trial results found that hydrazine sulfate did not prolong survival for cancer patients. The U.S. Food and Drug Administration (FDA) has received requests from individual physicians for approval to use hydrazine sulfate on a case-by-case "compassionate use" basis on the chance that patients with no other available effective cancer treatment options might benefit from this therapy. The controversy over the use of hydrazine sulfate is ongoing, and its relevance to clinical practice is unknown. The use of hydrazine sulfate needs to be evaluated further before any recommendations can be made.
Hydrazine sulfate may cause cancer. Avoid if allergic or hypersensitive to hydrazine sulfate or any of its constituents. Use cautiously with liver or kidney problems, psychosis, diabetes, or seizure disorders. Avoid if pregnant or breastfeeding. Side effects have been reported, including dizziness, nausea, and vomiting.
Iodine: Iodine is an element (atomic number 53), which is required by humans for the synthesis of thyroid hormones (triiodothyronine (T3) and thyroxine (T4)). The potential role of nonradioactive iodine in cancer care remains unknown. Antioxidant and antitumor effects have been proposed, based on laboratory research. In contrast, some scientists have asserted that tumors may uptake more iodine than normal tissues. It has been suggested that high rates of gastric (stomach) cancer or low rates of breast cancer in coastal Japan may be due to high iodine intake, although this has not been demonstrated scientifically. Povidone-iodine solutions have been used as a part of alternative cancer regimens, such as the Hoxsey formula. Preliminary research has also indicated povidone-iodine solution as a potential rectal washout for rectal cancer. Overall, no clear conclusion can be drawn based on the currently available evidence.
Reactions can be severe, and deaths have occurred with exposure to iodine. Avoid iodine-based products if allergic or hypersensitive to iodine. Do no use for more than 14 days. Avoid Lugol solution and saturated solution of potassium iodide (SSKI, PIMA) with hyperkalemia (high amounts of potassium in the blood), pulmonary edema (fluid in the lungs), bronchitis, or tuberculosis. Use cautiously when applying to the skin, because it may irritate or burn tissues. Use sodium iodide cautiously with kidney failure. Avoid sodium iodide with gastrointestinal obstruction. Iodine is safe in recommended doses for pregnant or breastfeeding women. Avoid povidone-iodine for perianal preparation during delivery or postpartum antisepsis.
Jiaogulan: Preliminary evidence indicates that gypenosides extracted from jiaogulan (
Gynostemma pentaphyllum) may decrease cancer cell viability, arrest the cell cycle, and induce apoptosis (cell death) in human cancer cells. Immune function in cancer patients has also been studied. Additional research is needed in this area.
Avoid if allergic or hypersensitive to jiaogulan (Gynostemma pentaphyllum), its constituents, or members of the Cucurbitaceae family. Use cautiously with blood disorders or if taking anticoagulants or antiplatelet drugs (blood thinners). Use cautiously with diabetes. Avoid if pregnant or breastfeeding.
Lavender: Perillyl alcohol (POH), derived from lavender (
Lavandula officinalis), may be beneficial in the treatment of some types of cancer. Preliminary small studies in humans, involving the use of POH, suggest safety and tolerability, but effectiveness has not been established.
Avoid if allergic or hypersensitive to lavender. Avoid with a history of seizures, bleeding disorders, eating disorders (anorexia, bulimia), or anemia (low levels of iron). Avoid if pregnant or breastfeeding.
Lutein: Currently, there is insufficient available evidence to recommend for or against the use of lutein for cancer. Available evidence in humans is conflicting.
Avoid if allergic or hypersensitive to lutein or zeaxanthin. Use cautiously if at risk for cardiovascular disease or cancer. Avoid if pregnant or breastfeeding.
Lycopene: High levels of lycopene are found in tomatoes and in tomato-based products. Tomatoes are also sources of other nutrients, such as vitamin C, folate, and potassium. Several laboratory and human studies examining tomato-based products and blood lycopene levels suggest that lycopene may be associated with a lower risk of developing cancer and may help stimulate the immune system. Studies have been conducted on the use of lycopene for prevention of various types of cancer including upper gastrointestinal tract and colorectal cancer. However, due to a lack of well-designed human research using lycopene supplements, its effectiveness for cancer prevention remains unclear.
Avoid if allergic to tomatoes or to lycopene. Due to a lack of conclusive data, avoid if pregnant or breastfeeding.
Maitake mushroom: Maitake is the Japanese name for the edible mushroom
Grifola frondosa. Maitake has been used traditionally both as a food and for medicinal purposes. Early studies in the laboratory as well as in humans suggest that beta-glucan extracts from maitake may increase the body's ability to fight cancer. However, these studies have not been well designed, and better research is needed before the use of maitake for cancer can be recommended.
Caution is advised when taking maitake supplements, as numerous adverse effects, including an increased risk of bleeding and drug interactions, are possible. Maitake should not be used if pregnant or breastfeeding, unless otherwise directed by a doctor.
Meditation: Not enough research has shown meditation to be of benefit in cancer prevention. More studies are needed.
Use cautiously with underlying mental illnesses. People with psychiatric disorders should consult with their primary mental healthcare professionals before starting a program of meditation, and should explore how meditation may or may not fit in with their current treatment plan. Avoid with risk of seizures. The practice of meditation should not delay the time to diagnosis or treatment with more proven techniques or therapies and should not be used as the sole approach to illnesses.
Melatonin: There are several early-phase and controlled human trials of melatonin in patients with various advanced-stage malignancies. There is currently not enough definitive scientific evidence to discern if melatonin is beneficial as a cancer treatment, whether it increases (or decreases) the effectiveness of other cancer therapies, or if it safely reduces chemotherapy side effects.
Melatonin is not to be used for extended periods of time. Caution is advised when taking melatonin supplements, as numerous adverse effects, including drug interactions, are possible. Melatonin is not recommended during pregnancy or breastfeeding, unless otherwise advised by a doctor.
Milk thistle: Milk thistle (
Silybum marianum) has been used medicinally in China for over 2,000 years, most commonly for the treatment of liver and gallbladder disorders. There are early reports from laboratory experiments that the components silymarin and silibinin found in milk thistle may reduce the growth of human cancer cells. However, effects have not been shown in high-quality human trials.
Caution is advised when taking milk thistle supplements, as numerous adverse effects, including an increased risk of bleeding and drug interactions, are possible. Milk thistle should not be used if pregnant or breastfeeding, unless otherwise directed by a doctor.
Mistletoe: Mistletoe is one of the most widely used unconventional cancer treatments in Europe. Mistletoe extracts have been studied for a variety of human cancers, as well as melanoma and leukemia. However, efficacy has not been conclusively proven for any one condition. In fact, some studies have shown lack of efficacy of certain preparations for a variety of cancers. Larger, well-designed clinical trials are needed.
Caution is advised when taking mistletoe supplements, as numerous adverse effects, including nausea, vomiting, and drug interactions, are possible. Mistletoe should not be used if pregnant or breastfeeding, unless otherwise directed by a doctor.
Moxibustion: Moxibustion is a healing technique employed across the diverse traditions of acupuncture and oriental medicine for over 2,000 years. Moxibustion uses the principle of heat to stimulate circulation and break up congestion or stagnation of blood and qi. Moxibustion is closely related to acupuncture, as it is applied to specific acupuncture points. Preliminary evidence suggests that moxibustion may reduce side effects of chemotherapy or radiation therapy. More studies are needed.
Use cautiously over large blood vessels, thin or weak skin, allergic skin conditions or ulcerated sores, or skin adhesions. Avoid the face, genitals, head, nipples, and skin adhesions. Avoid contraindicated acupuncture points, areas with an inflamed organ, and inflamed areas in general. Avoid with aneurysms, any kind of "heat syndrome," cardiac disease, convulsions, cramps, diabetic neuropathy, extreme fatigue, anemia, fever, or inflammatory conditions. Avoid if pregnant or breastfeeding. Avoid in patients who have just finished exercising or taking a hot bath or shower. Use cautiously with elderly people with large vessels. It is considered not advisable to bathe or shower for up to 24 hours after a moxibustion treatment.
Oleander: Laboratory studies of oleander (
Nerium oleander) suggest possible anticancer effects, although reliable research in humans is not currently available. There are reports that long-term use of oleander may have positive effects in patients several types of cancer. More research is needed.
Caution is advised when taking oleander supplements, as numerous adverse effects, including drug interactions, are possible. Oleander should not be used if pregnant or breastfeeding, unless otherwise directed by a doctor.
Omega-3 fatty acids: Omega-3 fatty acids are essential fatty acids found in some plants and fish. A balance of omega-6 and omega-3 fatty acids is advised for health. Several population studies report that dietary omega-3 fatty acids or fish oil may reduce the risk of developing several different types of cancer. Well-conducted clinical trials are necessary before a clear conclusion can be drawn regarding the use of omega-3 fatty acids for cancer prevention.
Caution is advised when taking omega-3 fatty acid supplements, as numerous adverse effects, including an increase in bleeding and drug interactions, are possible. Omega-3 fatty acid supplements should not be used if pregnant or breastfeeding, unless otherwise directed by a doctor.
Para-aminobenzoic acid: N-butyl-p-aminobenzoate (BAB) has been shown to be a lipid-soluble local anesthetic. Early research found significant pain relief in patients with intractable cancer pain after an epidural injection of BAB suspension. Larger-scale clinical research is needed to confirm these findings.
Avoid with known hypersensitivity to PABA or its derivatives. Avoid oral use in children and pregnant or nursing women. Use cautiously in patients with renal disease or bleeding disorders or in those taking anticoagulants. Avoid in diabetics or patients at risk for hypoglycemia. Discontinue use if rash, nausea, or anorexia occurs. Pharmaceutical doses of PABA and its derivatives should only be taken under appropriate medical supervision. PABA should not be given concurrently with sulfonamides.
Perillyl alcohol: Perillyl alcohol has been used to treat cancer. However, high-quality scientific studies are lacking. Further research is required before recommendations can be made.
Avoid if allergic or hypersensitive to perillyl alcohol. Avoid use in the absence of medical supervision. Use cautiously in patients under medical supervision. Avoid if pregnant or breastfeeding.
Prayer: Initial studies of prayer in patients with cancer (such as leukemia) report variable effects on disease progression or death rates when intercessory prayer is used. Better-quality research is necessary before a firm conclusion can be drawn.
Prayer is not recommended as the sole treatment approach for potentially serious medical conditions and should not delay the time it takes to consult with a healthcare professional or receive established therapies. Sometimes, religious beliefs come into conflict with standard medical approaches and require an open dialog between patients and caregivers. In clinical study, patients certain that they were receiving intercessory prayer had a higher incidence of complications following cardiac bypass surgery than those who did not know they were being prayed for.
PSK: Protein-bound polysaccharide (PSK) has been studied as a therapeutic adjuvant in various types of cancer including colorectal cancer, esophageal cancer, gastric cancer, and nasopharyngeal carcinoma. Well-designed clinical trials, with larger patient numbers, are needed to confirm available research results.
Avoid if allergic or hypersensitive to PSK, Coriolus versicolor, or any of its ingredients. Use cautiously with coronary artery disease. Avoid if pregnant or breastfeeding.
Psyllium: Psyllium, also referred to as ispaghula, is derived from the husks of the seeds of
Plantago ovata. According to early research, diets that include psyllium may reduce the risk of colon cancer. More studies are needed.
Avoid if allergic or hypersensitive to psyllium, ispaghula, or English plantains (Plantago lanceolata). Avoid in patients with esophageal disorders, gastrointestinal atony, fecal impaction, gastrointestinal tract narrowing, swallowing difficulties, and previous bowel surgery. Avoid ingestion of psyllium-containing products in individuals with repeated or prolonged psyllium exposure who have not manifested allergic or hypersensitive symptoms. Prescription drugs should be taken one hour before or two hours after psyllium. Adequate fluid intake is required when taking psyllium-containing products. Use cautiously with blood thinners, antidiabetic agents, carbamazepine, lithium, potassium-sparing diuretics, salicylates, tetracyclines, nitrofurantoin, calcium, iron, vitamin B12, other laxatives, tricyclic antidepressants (amitriptyline, doxepin, and imipramine), antigout agents, anti-inflammatory agents, hydrophilic agents, and chitosan. Use cautiously with diabetes and kidney dysfunction. Use cautiously if pregnant or breastfeeding.
Reiki: Reiki may contribute to reduced perception of pain, improved quality of life, and reduced fatigue in cancer patients. More studies are needed.
Reiki is not recommended as the sole treatment approach for potentially serious medical conditions and should not delay the time it takes to consult with a healthcare professional or receive established therapies. Use cautiously with psychiatric illnesses.
Reishi mushroom: Reishi (
Ganoderma lucidum) has been shown to have antineoplastic and immunomodulatory effects in animal studies. Human studies exist of advanced cancer patients using Ganopoly?, a
Ganoderma lucidum polysaccharide extract. Results show improved quality of life and enhanced immune responses, which are typically reduced or damaged in cancer patients receiving chemotherapy and/or radiation therapy. Well-designed long-term studies are needed confirm these results and to determine potential side effects.
Caution is advised when taking reishi supplements, as numerous adverse effects, including an increased risk of bleeding and drug interactions, are possible. Reishi should not be used if pregnant or breastfeeding, unless otherwise directed by a doctor.
Resveratrol: The effects of resveratrol cannot be adequately assessed from trials using foods, wine, or combination products containing resveratrol and other substances. Well-designed clinical trials of resveratrol alone are needed before a recommendation can be made in regard to cancer prevention and/or treatment.
Avoid if allergic or hypersensitive to resveratrol, grapes, red wine, or polyphenols. Resveratrol is generally considered safe and is commonly found in food and beverages. Use cautiously with bleeding disorders or abnormal blood pressure. Use cautiously with digoxin (or digoxin-like drugs) or drugs that are broken down by the body's cytochrome P450 system. Avoid if pregnant or breastfeeding.
Riboflavin: Riboflavin (vitamin B2) is a water-soluble vitamin that is involved in vital metabolic processes in the body and is necessary for normal cell function, growth, and energy production. Riboflavin supplementation has been studied in the prevention and treatment of esophageal cancer, mostly in China, with mixed results. No clear conclusion can be drawn at this time.
Avoid if allergic or hypersensitive to riboflavin. Since the amount of riboflavin a human can absorb is limited, riboflavin is generally considered safe. Riboflavin is generally regarded as safe during pregnancy and breastfeeding.
Seaweed: Bladderwrack (
Fucus vesiculosus) is a brown seaweed that grows on the northern coasts of the Atlantic and Pacific Oceans, and the North and Baltic Seas. Bladderwrack appears to suppress the growth of various cancer cells in animal and laboratory studies. However, reliable human studies to support a recommendation for use in cancer are currently lacking.
Caution is advised when taking bladderwrack supplements, as numerous adverse effects, including an increased risk of drug interactions, are possible. Bladderwrack should not be used if pregnant or breastfeeding, unless otherwise directed by a doctor.
Selenium: Selenium is a trace mineral found in soil, water, and some foods. It is an essential element in several metabolic pathways. Several studies suggest that low levels of selenium (measured in the blood or in tissues such as toenail clippings), may be a risk factor for developing cancer. Population studies suggest that people with cancer are more likely to have low selenium levels than healthy matched individuals, but in most cases, it is not clear if the low selenium levels are a cause or merely a consequence of disease. It currently remains unclear if selenium is beneficial for cancer prevention or cancer treatment.
Avoid if allergic or sensitive to products containing selenium. Avoid with a history of nonmelanoma skin cancer. Selenium is generally regarded as safe for pregnant or breastfeeding women. However, animal research reports that large doses of selenium may lead to birth defects.
Shark cartilage: For several decades, shark cartilage has been proposed as a cancer treatment. Studies have shown shark cartilage or the shark cartilage product AE-941 (Neovastat?) to block the growth of new blood vessels, a process called "antiangiogenesis," which is believed to play a role in controlling growth of some tumors. There have also been several reports of successful treatments of end-stage cancer patients with shark cartilage, but these have not been well designed and have not included reliable comparisons to accepted treatments. Many studies have been supported by shark cartilage product manufacturers, which may influence the results. In the United States, shark cartilage products are not allowed to claim to cure cancer, and the U.S. Food and Drug Administration (FDA) has sent warning letters to companies not to promote products in this way. Without further evidence from well-designed human trials, it remains unclear if shark cartilage is of any benefit in cancer, and patients are advised to check with their doctor and pharmacist before taking shark cartilage.
Shark cartilage available in Asian grocery stores and restaurants should not be eaten, due to declining populations of sharks. Caution is advised when taking shark cartilage supplements, as numerous adverse effects, including an increased risk of drug interactions, are possible. Shark cartilage should not be used by patients who are pregnant or breastfeeding, unless otherwise directed by a doctor.
Shiitake mushroom: Shiitake (
Lentinus edodes) has been taken by mouth for boosting the immune system, decreasing cholesterol levels, and promoting antiaging. Lentinan, derived from shiitake, has been injected as an adjunct treatment for cancer and HIV infection. Laboratory, animal, and human studies of lentinan have shown positive results in cancer patients when used as a chemotherapy adjunct. Further well-designed clinical trials on all types of cancer are required to confirm these results.
Caution is advised when taking shiitake supplements, as numerous adverse effects, including an increased risk of bleeding and drug interactions, are possible. Shiitake should not be used if pregnant or breastfeeding, unless otherwise directed by a doctor.
Slippery elm: Slippery elm is found as a common ingredient in a purported herbal anticancer product called Essiac? and a number of Essiac-like products. These products contain other herbs such as rhubarb, sorrel, and burdock root. Currently, there is not enough evidence to recommend for or against the use of this herbal mixture as a therapy for any type of cancer. Avoid if allergic or hypersensitive to slippery elm. Avoid if pregnant or breastfeeding.
Sorrel: Early evidence suggests that herbal formulations containing sorrel, such as Essiac?, do not shrink tumor size or increase life expectancy in patients with cancer. However, currently there is a lack of studies evaluating sorrel as the sole treatment for cancer. A conclusion cannot be made without further research.
Avoid with a known allergy or hypersensitivity to sorrel. Avoid large doses, due to reports of toxicity and death, possibly because of the oxalate found in sorrel. Many tinctures contain high levels of alcohol and should be avoided when driving or operating heavy machinery. Sorrel formulations may cause nausea or vomiting when taken with the prescription drugs metronidazole (Flagyl?) or disulfiram (Antabuse?). Avoid if pregnant or breastfeeding.
Soy: Soy (
Glycine max) contains compounds which have been reported to be effective as a cancer treatment. Genistein, an isoflavone found in soy, has been found in laboratory and animal studies to possess anticancer effects, such as blocking new blood vessel growth (called antiangiogenesis), acting as a tyrosine kinase inhibitor (a mechanism of many new cancer treatments), or causing cancer apoptosis (cell death). In contrast, genistein has also been reported to increase the growth of pancreas tumor cells in laboratory research. Until reliable human research is available, it remains unclear if dietary soy or soy isoflavone supplements are beneficial, harmful, or neutral in people with various types of cancer.
Caution is advised when taking soy supplements, as numerous adverse effects, including an increased risk of drug interactions, are possible. Soy should not be used if pregnant or breastfeeding, unless otherwise directed by a doctor.
Spiritual healing: Cancer patients, especially those who fear recurrence or are unhappy with their physicians, commonly use prayer and spiritual healing. More research is needed to address the effects of spiritual healing on anxiety, depression, and quality of life in patients with cancer.
Spiritual healing should not be used as the only treatment approach for medical or psychiatric conditions and should not delay the time it takes to consider more proven therapies.
Sweet annie: Certain constituents found in sweet annie show promise for use in cancer when used in combination with standard chemotherapy. However, currently there is not enough scientific evidence in humans to make a strong recommendation for this use.
Avoid if allergic or hypersensitive to sweet annie (Artemisia annua), its constituents, or members of the Asteraceae or Compositae family, such dandelion, goldenrod, ragweed, sunflower, and daisies. Use cautiously in patients who are taking angiogenic agents or recovering from surgery or other wounds. Use cautiously if taking cardiotoxic or neurotoxic agents or in those with compromised cardiac or neural function. Use cautiously if taking immunostimulants or quinolines. Avoid if pregnant or breastfeeding.
Taurine: For iron deficiency anemia, preliminary research suggests that the amino acid taurine aids in the ability of iron supplements to increase hemoglobin, red blood cell count, and serum ferritin. Additional research is needed before a conclusion can be made.
Allergies related to taurine are considered unlikely. However, allergies may occur from multi-ingredient products that contain taurine. Use cautiously in patients with high cholesterol, low blood pressure, coagulation disorders, potential for mania, or epilepsy. Avoid consumption of energy drinks containing taurine, caffeine, glucuronolactone, B vitamins, and other ingredients, then consuming alcohol or exercising. Use cautiously if pregnant or breastfeeding, because taurine is a natural component of breast milk.
Transcutaneous electrical nerve stimulation (TENS): Transcutaneous electrical nerve stimulation (TENS) is a noninvasive technique in which a low-voltage electrical current is delivered through wires from a small power unit to electrodes located on the skin. Although TENS has been used with some success in cancer pain, there is not enough reliable evidence to draw a firm conclusion in this area. TENS is often used in combination with acupuncture.
Avoid with implantable devices, such as defibrillators, pacemakers, intravenous infusion pumps, or hepatic artery infusion pumps. Use cautiously with decreased sensation, such as neuropathy, and with seizure disorders. Avoid if pregnant or breastfeeding.
Thiamin (Vitamin B1): Thiamin deficiency has been observed in some cancer patients, possibly due to increased metabolic needs. It is not clear if lowered levels of thiamin in such patients may actually be beneficial. Currently, it remains unclear if thiamin supplementation plays a role in the management of any particular types of cancer.
Thiamin is generally considered safe and relatively nontoxic. Avoid if allergic or hypersensitive to thiamin.
Rare hypersensitivity or allergic reactions have occurred with thiamin supplementation. Skin irritation, burning, or itching may rarely occur at injection sites. Large doses may cause drowsiness or muscle relaxation. Use cautiously if pregnant or breastfeeding.
Thymus extract: Preliminary evidence suggests that thymus extract may increase disease-free survival and immunological improvement in several types of cancer. Additional research is needed in this area.
Avoid if allergic or hypersensitive to thymus extracts. Use bovine thymus extract supplements cautiously due to potential for exposure to the virus that causes "mad cow disease." Avoid with an organ transplant or other forms of allografts or xenografts. Avoid if receiving immunosuppressive therapy or hormonal therapy. Avoid with thymic tumors, myasthenia gravis (neuromuscular disorder), or untreated hypothyroidism. Avoid if pregnant or breastfeeding.
Traditional Chinese medicine (TCM): The ancient Chinese philosophy of Taoism provides the basis for the development of Chinese medical theory. TCM uses over 120 different herbs in cancer treatment, depending on the type of cancer and its cause according to Chinese medical theory. Studies have reported significant benefits, including reducing tumors, reducing treatment side effects, and improving response to treatment. More studies of stronger design are needed before TCM can be recommended with confidence as an adjunct to cancer treatment, although centuries of traditional use in cancer cannot be discounted.
Chinese herbs can be potent and may interact with other herbs, foods, or drugs. Consult a qualified healthcare professional before taking. There have been reports of manufactured or processed Chinese herbal products being tainted with toxins or heavy metal or not containing the listed ingredients. Herbal products should be purchased from reliable sources. Avoid ma huang, which is the active ingredient in ephedra. Avoid ginseng if pregnant or breastfeeding.
Turmeric: Turmeric (
Curcuma longa) is commonly used for its anti-inflammatory properties. Several early animal and laboratory studies report anticancer (colon, skin, breast) properties of curcumin. Many mechanisms have been considered, including antioxidant activity, antiangiogenesis (the prevention of new blood vessel growth), and direct effects on cancer cells. Currently, it remains unclear if turmeric or curcumin has a role in preventing or treating human cancer. There are several ongoing studies in this area.
Caution is advised when taking turmeric supplements, as numerous adverse effects, including an increased risk of bleeding and drug interactions, are possible. Turmeric should not be used if pregnant or breastfeeding, unless otherwise directed by a doctor.
Vitamin A: Research results are not clear as to whether vitamin A is beneficial in the treatment or prevention of stomach cancer.
Vitamin A supplementation in combination with iron may have beneficial effects in patients with iron deficiency anemia, including children and pregnant women. It is not clear that there are benefits in individuals who are not vitamin A deficient. This area remains controversial, and further evidence is necessary before a clear conclusion can be drawn.
Avoid if allergic or hypersensitive to vitamin A. Vitamin A toxicity can occur if taken at high dosages. Use cautiously with liver disease or alcoholism. Smokers who consume alcohol and beta-carotene may have an increased risk for lung cancer or heart disease. Vitamin A appears safe in pregnant women if taken at recommended doses. Use cautiously if breastfeeding, because the benefits or dangers to nursing infants are not clearly established.
Vitamin C (ascorbic acid): Dietary intake of fruits and vegetables high in vitamin C has been associated with a reduced risk of various types of cancer in population studies (particularly cancers of the mouth, esophagus, stomach, colon, or lung). However, it is not clear that it is specifically the vitamin C in these foods that is beneficial, and vitamin C supplements have not been found to be associated with this protective effect. Experts have recommended increasing dietary consumption of fruits and vegetables high in vitamin C, such as apples, asparagus, berries, broccoli, cabbage, melon (cantaloupe, honeydew, watermelon), cauliflower, citrus fruits (lemons, oranges), kale, kiwi, potatoes, spinach, and tomatoes, as well as fortified breads, grains, and cereal. Vitamin C has a long history of adjunctive use in cancer therapy, and although there have not been any definitive studies using intravenous (or oral) vitamin C, there is evidence that it has benefit in some cases. Better-designed studies are needed to better determine the role of vitamin C in cancer prevention and cancer treatment.
Avoid if allergic or sensitive to vitamin C product ingredients. Vitamin C is generally considered safe in amounts found in foods. Vitamin C supplements are also generally considered safe in most individuals if taken in recommended doses. Large doses (greater than two grams) may cause diarrhea and gastrointestinal upset. Avoid high doses of vitamin C with glucose 6-phosphate dehydrogenase deficiency, kidney disorders or stones, cirrhosis (inflammation of the liver), gout, or paroxysmal nocturnal hemoglobinuria (a bleeding disorder). Vitamin C intake from food is generally considered safe if pregnant or breastfeeding. It is not clear if vitamin C supplements in doses higher than Dietary Reference Intake recommendations are safe for pregnant or breastfeeding women. Vitamin C is naturally found in breast milk.
Vitamin D: Limited research suggests that synthetic vitamin D analogs may play a role in the treatment of human cancers. However, it remains unclear if vitamin D deficiency raises cancer risk, or if an increased intake of vitamin D is protective against some cancers. Until additional trials are conducted, it is premature to advise the use of regular vitamin D supplementation for cancer prevention, such as in colorectal cancer.
Avoid if allergic or hypersensitive to vitamin D or any of its components. Vitamin D is generally well tolerated in recommended doses; doses higher than those recommended may cause toxic effects. Use cautiously with hyperparathyroidism (overactive thyroid), kidney disease, sarcoidosis, tuberculosis, and histoplasmosis. Vitamin D is safe in pregnant and breastfeeding women when taken in recommended doses.
Vitamin E: Reliable scientific evidence that vitamin E is effective as a cancer treatment is currently lacking. Vitamin E has been studied for use in various types of cancer including colon cancer and stomach cancer. Additional research is needed.
Caution is merited in people undergoing chemotherapy or radiation, because it has been proposed that the use of high-dose antioxidants may actually reduce the anticancer effects of these therapies. This remains an area of controversy, and studies have produced variable results. Patients interested in using high-dose antioxidants such as vitamin E during chemotherapy or radiation should discuss this decision with their medical oncologist or radiation oncologist. Caution is advised when taking vitamin E supplements, as numerous adverse effects, including an increased risk of bleeding and drug interactions, are possible. Avoid if allergic or hypersensitive to vitamin E. Avoid with retinitis pigmentosa (a loss of peripheral vision). Use cautiously with bleeding disorders or if taking blood thinners. Avoid use above the recommended daily level in pregnant women and breastfeeding women.
Fair negative scientific evidence:
Apricot: Available clinical trials on the use of whole apricots for cancer are currently lacking. However, some research has been conducted on LaetrileT, an alternative cancer drug marketed in Mexico and other countries outside of the United States. LaetrileT is derived from amygdalin, found in apricot pits and nuts such as bitter almond. There are multiple animal studies and initial human evidence to suggest that LaetrileT is not beneficial in the treatment of cancer. Based on a phase II trial in 1982, the U.S. National Cancer Institute concluded that LaetrileT is not an effective chemotherapeutic agent. Nonetheless, many people still travel to use this therapy outside the United States.
Multiple cases of cyanide poisoning, including deaths, have been associated with LaetrileT therapy. Avoid if allergic to apricot, its constituents, or members of the Rosaceae family, especially the Prunoideae subfamily of plants. Avoid eating excessive amounts of apricot kernels (about seven grams daily, or more than 10 kernels daily). Use cautiously with diabetes. Use cautiously when taking supplements containing beta-carotene, iron, niacin, potassium, thiamine, or vitamin C. Use cautiously when taking products that may lower blood pressure. Avoid if pregnant or breastfeeding.
Beta-carotene: While diets high in fruits and vegetables rich in beta-carotene have been shown to potentially reduce certain cancer incidences, results from randomized controlled trials with oral supplements do not support this claim.
There is some concern that beta-carotene metabolites with pharmacological activity can accumulate and potentially have carcinogenic (cancer-causing) effects. A higher, statistically significant incidence of lung cancer in male smokers who took beta-carotene supplements has been discovered. Beta-carotene and vitamin A supplements may have an adverse effect on the incidence of lung cancer and on the risk of death in smokers and asbestos-exposed people or in those who ingest significant amounts of alcohol. In addition, high-dose antioxidants theoretically may interfere with the activity of some chemotherapy drugs or radiation therapy. Therefore, individuals undergoing cancer treatment should speak with their oncologist if they are taking or considering the use of high dose antioxidants. Beta-carotene in the amounts normally found in food does not appear to have this adverse effect. Avoid if sensitive to beta-carotene, vitamin A, or any other ingredients in beta-carotene products.
Bitter almond: LaetrileT is an alternative cancer drug marketed in Mexico and other countries outside of the United States. LaetrileT is derived from amygdalin, found in the pits of fruits and nuts such as the bitter almond. Early evidence suggests that LaetrileT is not beneficial in the treatment of cancer. In 1982, the U.S. National Cancer Institute concluded that LaetrileT was not effective for cancer therapy. Nonetheless, many people still travel to use this therapy outside the United States.
Multiple cases of cyanide poisoning, including deaths, have been associated with LaetrileT therapy. Avoid if allergic to almonds or other nuts. Use cautiously if driving or operating machinery. Avoid if pregnant or breastfeeding, because of the risk of birth defects.
Hypnotherapy, hypnosis: Hypnosis did not reduce radiotherapy side effects such as anxiety and did not improve quality of life in patients undergoing curative radiotherapy in early high-quality studies.
Use cautiously with seizure disorders or with mental illnesses like psychosis, schizophrenia, manic depression, multiple personality disorder, or dissociative disorders.
Iridology: There are currently limited available data supporting iridology as a tool for cancer diagnosis. Additional research is needed.
Iridology should not be used alone to diagnose disease. Studies of iridology have reported incorrect diagnoses, and thus potentially severe medical problems may go undiagnosed. In addition, research suggests that iridology may lead to inappropriate treatment. Iridology is therefore not recommended as a sole method of diagnosis or treatment for any condition.
Vitamin E: At this time, based on the best available scientific evidence and recent concerns about the safety of vitamin E supplementation, vitamin E cannot be recommended for cancer prevention.
Avoid if allergic or hypersensitive to vitamin E. Avoid with retinitis pigmentosa (a loss of peripheral vision). Use cautiously with bleeding disorders or if taking blood thinners. Avoid above the recommended daily level in pregnant and breastfeeding women.
Prevention
General: Because Peutz Jeghers syndrome (PJS) is a genetic condition, there is currently no way to prevent the disease. However, a number of options are available for prospective parents with a family history of PJS.
Genetic testing and counseling: Individuals who have PJS may meet with a genetic counselor to discuss the risks of having children with the disease. Genetic counselors can explain the options and the associated risks of various tests, including preimplantation genetic diagnosis (PGD), amniocentesis, and chorionic villus sampling (CVS).
Preimplantation genetic diagnosis (PGD) may be used with artificial fertilization. In PGD, embryos are tested for the defective STK11 gene, and only the embryos that are not affected may be selected for implantation. Because PJS can be detected in a fetus, parents may choose whether to continue the pregnancy. Genetic counselors may assist parents with these difficult decisions.
Author information
This information has been edited and peer-reviewed by contributors to the Natural Standard Research Collaboration (www.naturalstandard.com).
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Causes
Genetic mutations: Peutz Jeghers syndrome (PJS) is usually caused by mutations or defects in the serine/threonine kinase 11 gene (STK11 or STK11/LKB1), which provides instructions for making the STK11 protein. The STK11 gene plays a role in the suppression of tumor growth. When the gene is mutated, the structure or function of the STK11 protein is altered, which changes its ability to regulate cell growth, possibly resulting in tumor formation.
Some researchers suggest that an additional genetic mutation may play a role in the development and progression of PJS. Whether this mutation occurs in the other STK11 gene or in a different gene altogether is currently unknown.
How these genetic mutations lead to the formation of hamartomatous polyps, which are noncancerous growths that occur in the intestines, and the characteristic skin discoloration found in PJS patients is not well understood. In some individuals with PJS, the STK11 gene is not mutated. In these individuals, the cause of the disease is unknown.
Autosomal dominant inheritance: About half of PJS cases are inherited, or passed down among family members, as an autosomal dominant trait. People inherit two copies of most genes, one from the mother and one from the father. To inherit an autosomal dominant trait, an individual needs to inherit only one defective copy of the causative gene. If one parent has the disorder, there is a 50% chance that his or her child will have the disorder. If both parents have the disorder, there is a 75% chance that their child will have the disorder. (By contrast, to inherit an autosomal recessive trait, an individual must inherit two defective copies of the causative gene.)
Random occurrence: The other half of PJS cases occur in individuals with no family history of the disorder. These cases result from a spontaneous genetic mutation in the egg, sperm cells, or developing embryo.
Risk factors
Peutz Jeghers syndrome (PJS) may be an inherited condition; therefore, a risk factor is a family history of the disease.
About half of PJS cases are inherited, or passed down among family members, as autosomal dominant traits. People inherit two copies of most genes, one from the mother and one from the father. To inherit an autosomal dominant trait, an individual needs to inherit only one defective copy of the causative gene. If one parent has the disorder, there is a 50% chance that his or her child will have the disorder. If both parents have the disorder, there is a 75% chance that their child will have the disorder.
The other half of PJS cases occur in individuals with no family history of the disorder. These cases result from a spontaneous genetic mutation in the egg, sperm cells, or developing embryo. There are no known risk factors for this method of occurrence.
The exact prevalence of PJS is unknown, but it is estimated to occur in about 1 in 25,000-300,000 births in the United States. The international prevalence of PJS (outside of the United States) is unknown. PJS does not appear to affect one sex or ethnicity more than another.