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There are a few types of tumor that fall within a category somewhere between benign and malignant. Carcinoid tumors are the most often occurring of these rare types of midway growths. They have been called "cancers in slow motion" because even though they usually have the potential for ultimately being fatal, they mostly tend to grow slowly so that people afflicted with these tumors usually live for many years, sometimes even for a normal lifetime.
Incidence; Causes & Development
Carcinoid tumours are rare neuroendocrine lesions. Approximately 1,000 cases are seen each year in the United Kingdom. Most arise from the gastrointestinal tract. The commonest sites of the primary tumour are the appendix (30%), small bowel (20%), colon (20%) and stomach less than 10%. Tumours can also arise from the bronchial tree.
Carcinoid tumors arise from glandular endocrine-hormone producing cells that are widely distributed within the body but found in greatest amounts in the small intestine and then in decreasing amounts in the appendix, rectum, lung, pancreas and very rarely in the ovaries, testes, liver, bile ducts or other locations. The prognosis depends on where the original tumor is located, the likelihood of metastases becoming greater with increasing tumor size.
Signs & Symptoms
Carcinoid cells are capable of producing hormones. When carcinoid tumours have metastasised, those that produce large amounts of hormones and other potent chemical substances, and which often have spread to the liver, can cause "carcinoid syndrome" -- hot red flushing of the face, diarrhea, and asthma-like wheezing attacks. Episodes of "carcinoid crisis" may be very infrequent and last only a few minutes at first but gradually occur more often as the disease progresses. Episodes are usually associated with abrupt low blood pressure and even fainting, although in a few cases the attacks are accompanied by high blood pressure. In many cases the symptoms of Carcinoid Syndrome, resulting from the hormones and chemicals produced, are worse than the symptoms from the growth of the tumor itself. Flushing may be precipitated by the ingestion of either chocolate or alcohol and is often associated with palpitations and episodes of hypotension. Clinical examination is often normal.
Not all functioning carcinoid tumors produce the same large variety of chemicals and hormones and it is not yet entirely clear which of the substances are responsible for each of the symptoms. Almost all of these tumors make serotonin and bradykinin.
Patients often present with vague right-sided abdominal pain which has often been present for a number of years, suggesting a right-sided abdominal mass or hepatomegaly. Many patients have had numerous normal investigations. Urgent surgery, has often been precipitated by the onset of intestinal obstruction.
In those with carcinoid syndrome, features of pellagra, tricuspid regurgitation or pulmonary stenosis may also be present.
Diagnosis & Tests
Non-functioning carcinoid tumors are so slow-growing that many years may pass between the onset of any symptoms and the diagnosis. They can cause intermittent abdominal pain and then a change in bowel habits that may lead to intestinal obstruction. In some cases they cause obscure intestinal bleeding or sometimes don't declare themselves until they cause painful enlargement of the liver due to large deposits of carcinoid metastases that have spread to that organ. The diagnosis is not usually suspected prior to surgery but is then established by biopsy.
Carcinoid Syndrome, due to the presence of a functioning carcinoid tumor, is easily diagnosed when all of the features of the syndrome are present. However, the biggest impediment to making the diagnosis is not thinking of it because of its rarity: when it is considered, just one or two of the main symptoms may be enough to confirm suspicions.
Standard X-ray and imaging techniques can be helpful in finding a carcinoid tumor and identifying its spread. This could include routine chest X-ray, CT scans, MRI, barium enema or upper GI and small bowel X-ray studies. Sometimes upper and lower GI tract endoscopy is also helpful.
A now universally approved (though costly) way of finding carcinoid tumors, as well as other neuroendocrine tumors, is the OctreoScan. It is successful in 85% of carcinoids and consists of a (probably) harmless injection of a minute dose of a short duration radioactive isotope which is specifically attracted to, and concentrated in, carcinoid tumor tissue (and any other neuroendocrine tumor) where it lights up during a radiation scan of the entire body.
In most cases a firm diagnosis has not been established before an operative procedure has been performed.
Laboratory Findings:- Increased urine 5-hydroxyindoleacetic acid (5-HIAA), a metabolite of serotonin - occurs only when hepatic metastases are present
- Increased blood and urine serotonin, found only after hepatic metastases of intestinal carcinoid tumor and in gastric carcinoid tumors
- Laboratory findings of diarrhea.
Treatment & Prevention
There are many conventional treatments for carcinoid tumors and syndrome, although the choice of treatment and their applications can be quite complex. Carcinoid tumors vary greatly in their size, location, symptoms and growth, and therefore the treatment in each case should be individualized to what is best for each particular patient. The wide variety of treatments now available makes the outlook for most victims of the more aggressive carcinoids more hopeful than it used to be.
Surgery is often recommended and can be highly successful. In cases where somewhat larger tumors have spread to local tissues and local lymph nodes but are, along with these locally invaded tissues, still totally surgically removable, the average survival has been 8 years ranging up to 23 years.
Chemotherapy, with many choices available, has been in use since the early 1980s. Radiation is useful only for pain relief and regressing tumors when they have spread to the skeletal system and when they are causing severe pain.
Antihistamines and alpha adrenergic blocking drugs such as Dibenzyline are sometimes used to prevent Carcinoid Syndrome attacks. Certain very severe and prolonged carcinoid crises associated with bronchial (lung) carcinoids or some carcinoids of the stomach are responsive to treatment with corticosteroids (prednisone, Decadron) and Thorazine or Compazine.
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Signs, symptoms & indicators of Carcinoid Cancer:
Conditions that suggest Carcinoid Cancer:
Risk factors for Carcinoid Cancer:
Carcinoid Cancer suggests the following may be present:
Carcinoid Cancer can lead to: | | | | | Digestion |  Diarrhea | Diarrhea can occur along with facial flushing or by itself. Stools are watery and the diarrhea can be mild to severe. Episodes can occur several times each day and can interfere with daily life. |
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Nutrients |
 General Mineral Requirement |
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Recommendations and treatments for Carcinoid Cancer: | | | | | Diet |  Alcohol Avoidance| | All carcinoid patients should avoid alcoholic beverages since these can precipitate carcinoid crisis attacks. |
High/Increased Protein Diet| | A high protein diet is often recommended. An adequate intake of the amino acid tryptophan, along with niacin, can prevent the tendency towards pellagra, which may otherwise occur from the excessive use of tryptophan by carcinoid tumors. |
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Drug |
 LDN - Low Dose Naltrexone
Conventional Drug Avoidance| | Adrenaline-like drugs should be avoided for carcinoid tumors as they can cause carcinoid crisis attacks. Examples include various asthma inhalers, nasal decongestants and adrenaline itself. |
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Lab Tests/Rule-Outs |
Chromogranin A (CgA) Test| | The measurement of CgA is considered "the gold standard" of chemical tests for confirming the diagnosis of carcinoid and neuroendocrine tumors and following their course. |
5-HIAA Urine test| | In the presence of Carcinoid Syndrome the amount of 5-HIAA is almost always distinctly increased above normal. |
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Physical Medicine |
Rest| | Physical stress should be avoided since this can precipitate carcinoid crisis attacks. |
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Psychological |
Stress Management| | Emotional stress can precipitate a carcinoid attack and should be avoided where possible. |
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KEY | | Weak or unproven link | | | Strong or generally accepted link | | | Proven definite or direct link |  | | Weakly counter-indicative |  | | Strongly counter-indicative | | | Likely to help | | | Highly recommended |
GLOSSARY
Abdomen (Abdominal)
That part of the body between the chest and the hips that contains the stomach, intestines, liver, bladder, pancreas and other organs.
Adrenaline (Epinephrin, Epinephrine)
A hormone secreted by the adrenal medulla that is released into the bloodstream in response to physical or mental stress, as from fear or injury. It initiates many bodily responses, including the stimulation of heart action and an increase in blood pressure, metabolic rate, and blood glucose concentration.
Amino Acid (Amino Acids)
An organic acid containing nitrogen chemical building blocks that aid in the production of protein in the body. Eight of the twenty-two known amino acids are considered "essential," and must be obtained from dietary sources because the body can not synthesize them.
Asthma (Asthmatic)
A lung disorder marked by attacks of breathing difficulty, wheezing, coughing, and thick mucus coming from the lungs. The episodes may be triggered by breathing foreign substances (allergens) or pollutants, infection, vigorous exercise, or emotional stress. Extrinsic Asthma is triggered by pollen, chemicals or some other external agent; Intrinsic Asthma is triggered by boggy membranes, congested tissues, or other native causes… even adrenalin stress or exertion.
Benign
Literally: innocent; not malignant. Often used to refer to cells that are not cancerous; they tend to grow slowly and don't spread (metastasize) like cancer tumors do.
Bile
A bitter, yellow-green secretion of the liver. Bile is stored in the gallbladder and is released when fat enters the first part of the small intestine (duodenum) in order to aid digestion.
Biopsy
Removal of a sample of tissue from a living being for diagnosis. A pathologist later uses a microscope to look for certain features, such as cancer cells, in the sample. A fine-needle aspiration biopsy involves inserting a thin needle to remove a small amount of tissue, sometimes using CT or ultrasound to guide the needle. A core biopsy involves obtaining a sample of tissue with a thick needle or by inserting a thin, lighted tube (laparoscope) into a small incision in the abdomen. Another biopsy method is to remove tissue during an operation.
Calcium
The body's most abundant mineral. Its primary function is to help build and maintain bones and teeth. The body also needs calcium to carry nerve signals, keep the heart functioning, contract muscles, clot blood and maintain healthy skin. Calcium helps control blood acid-alkaline balance, plays a role in cell division, muscle growth and iron utilization, activates certain enzymes, and helps transport nutrients through cell membranes. Calcium also forms a cellular cement called ground substance that helps hold cells and tissues together.
Chemotherapy
A treatment of disease by any chemicals. Used most often to refer to the chemical treatments used to combat cancer cells. Chemotherapy is usually given in cycles: a treatment period followed by a recovery period, then another treatment period, and so on. Most anticancer drugs are given by injection into a blood vessel (IV); some are given by mouth. Chemotherapy is a systemic therapy, meaning that the drugs enter the bloodstream and travel throughout the body. Usually, a patient has chemotherapy as an outpatient (at the hospital, at the doctor's office, or at home). However, depending on which drugs are given and the patient's general health, a short hospital stay may be needed.
Colon (Colonic)
The part of the large intestine that extends to the rectum. The colon takes the contents of the small intestine, moving them to the rectum by contracting.
Congestive
Pertaining to accumulation of blood or fluid within a vessel or organ.
Corticosteroid (Corticosteroids)
Steroid hormone produced by the adrenal cortex.
Diarrhea
Excessive discharge of contents of bowel.
Endoscopy
A procedure that uses an Endoscope: Examination with a flexible viewing tube.
Gastric
Of, relating to, or associated with the stomach.
Gastrointestinal (GI, GI Tract)
Pertaining to the stomach, small and large intestines, colon, rectum, liver, pancreas, and gallbladder.
Glandular (Glandulars, Raw Glandulars)
Either referring to a gland, OR glands and organs from animals specially processed at temperatures not exceeding body heat and put into supplement form.
Hormones (Hormone)
Chemical substances secreted by a variety of body organs that are carried by the bloodstream and usually influence cells some distance from the source of production. Hormones signal certain enzymes to perform their functions and, in this way, regulate such body functions as blood sugar levels, insulin levels, the menstrual cycle, and growth. These can be prescription, over-the-counter, synthetic or natural agents. Examples include adrenal hormones such as corticosteroids and aldosterone; glucagon, growth hormone, insulin, testosterone, estrogens, progestins, progesterone, DHEA, melatonin, and thyroid hormones such as thyroxine and calcitonin.
Hypotension
Low blood pressure.
Iron
An essential mineral. Prevents anemia: as a constituent of hemoglobin, transports oxygen throughout the body. Virtually all of the oxygen used by cells in the life process are brought to the cells by the hemoglobin of red blood cells. Iron is a small but most vital, component of the hemoglobin in 20,000 billion red blood cells, of which 115 million are formed every minute. Heme iron (from meat) is absorbed 10 times more readily than the ferrous or ferric form.
Lesion (Lesions)
Any damage to tissue structure or function; an abnormal change in body tissue caused by disease or injury. A scar is a lesion, as is cancer, a stomach ulcer or a pimple.
Liver (Hepatic)
The largest and one of the most complex organs of the body, the liver is responsible for much of the metabolism of fats, proteins and carbohydrates. It is the site of much of the body's detoxification. It is connected very closely with digestion and the regulation of blood sugar, among many other functions. Found behind the ribs on the right side of the abdomen, it has many important functions such as removing harmful material from the blood, making enzymes and bile that help digest food, and converting food into substances needed for life and growth. Hepatic: Pertaining to the liver.
Lung (Lungs, Pulmonary)
Organ of the body, located in the chest cavity which is designed to bring oxygen from the air into the blood stream, while also expelling carbon dioxide and other waste gases out of the body. Pulmonary: Related to the lungs.
Lymph Nodes (Axillary Nodes, Inguinal Nodes, Lymph Gland, Lymph Glands, Lymph Node, Lymphatic)
Small, bean-shaped nodes at various points throughout the body that function to filter the lymph fluid and attempt to destroy the microorganisms and abnormal cells which collect there. The most common locations are the neck (both sides and front), armpit (axillary) and groin (inguinal), but also under the jaw and behind the ears. Swollen or painful lymph nodes generally result from localized or systemic infection, abscess formation, or malignancy. Other causes of enlarged lymph nodes are extremely rare. Physical examination for lymph nodes includes pressing on them to check for size, texture, warmth, tenderness and mobility. Most lymph nodes can not be felt until they become swollen, and then will only be tender when pressed or massaged. A lymph node that is painful even without touching indicates greater swelling. Lymph nodes can usually be distinguished from other growths because they generally feel small, smooth, round or oval-shaped and somewhat mobile when attempts are made to push them sideways. Because less fat covers the lymph nodes in children, they are easier to feel, even when they are not busy filtering germs or making antibodies. Childrens' nodes enlarge faster, get bigger in response to an infection and stay swollen longer than an adult's. Also known (incorrectly) as lymph glands.
Magnesium
An essential mineral. The chief function of magnesium is to activate certain enzymes, especially those related to carbohydrate metabolism. Another role is to maintain the electrical potential across nerve and muscle membranes. It is essential for proper heartbeat and nerve transmission. Magnesium controls many cellular functions. It is involved in protein formation, DNA production and function and in the storage and release of energy in ATP. Magnesium is closely related to calcium and phosphorus in body function. The average adult body contains approximately one ounce of magnesium. It is the fifth mineral in abundance within the body--behind calcium, phosphorus, potassium and sodium. Although about 70 percent of the body's magnesium is contained in the teeth and bones, its most important functions are carried out by the remainder which is present in the cells of the soft tissues and in the fluid surrounding those cells.
Magnetic Resonance Imaging (MRI)
A technique used in diagnosis that combines radio waves and magnetic forces to produce detailed images of the internal structures of the body.
Malignant (Cancerous)
Dangerous. Mainly used to describe a cancerous growth -- when used this way, it means the growth is cancerous and predisposed to spreading.
Metabolism (Metabolic, Metabolize, Metabolizes, Metabolizing)
The chemical processes of living cells in which energy is produced in order to replace and repair tissues and maintain a healthy body. Responsible for the production of energy, biosynthesis of important substances, and degradation of various compounds. Also defined as the sum total of changes in an organism in order to achieve a balance (homeostasis): Catabolic burns up, anabolic stores and builds up; the sum of their work is metabolism.
Metabolite (Metabolites)
Any product (foodstuff, intermediate, waste product) of metabolism.
Metastasize (Metastases, Metastasized, Metastasizes, Metastasizing, Metastatic)
Usually in reference to cancer: to spread, especially destructively.
Mineral (Minerals)
Plays a vital role in regulating many body functions. They act as catalysts in nerve response, muscle contraction and the metabolism of nutrients in foods. They regulate electrolyte balance and hormonal production, and they strengthen skeletal structures.
Niacin (B3, B-3, Niacinamide, Vitamin B3)
A coenzyme B-complex vitamin that assists in the breakdown of carbohydrates, fats and proteins. Essential for the health of the skin, nerves, tongue and digestive system. It is found in every cell of the body and is necessary for energy production. Niacin is also needed for DNA formation.
Ovary (Ovaries)
Ovaries are about the size of an almond and lie on either side of the lower abdomen beside the uterus. They usually produce one egg each month and whether the egg is fertilized or not, the reproductive process follows a monthly cycle, with constant changes in various hormone levels, preparing another opportunity for conception. The ovary is responsible for most of the estrogen production in women. At menopause (sometime after middle-age), egg production ceases and hormone levels drop significantly.
Pancreas (Pancreatic)
Opposite the liver and behind the stomach, the pancreas has two main functions - to manufacture various enzymes for digestion, and to release hormones to help control the body's use of carbohydrates. It releases insulin to help each cell absorb glucose to burn as energy. In this way, insulin controls the amount of sugar (glucose) in the blood. Proper pancreatic function is very important: too much, too little, or no insulin production can be life-threatening. Some of the chemicals released by the pancreas are not hormones, but stimulate other glands to make hormones. Once again, balance is necessary. Nutritional requirements for the pancreas are many. Research indicates that chromium vitamins C, E, B-complex, calcium, magnesium and potassium are especially important.
Pellagra
A disease caused by a deficiency of thiamine (B1), usually occurring in alcoholics or in persons with malabsorption disorders.
Potassium
A mineral that serves as an electrolyte and is involved in the balance of fluid within the body. Our bodies contain more than twice as much potassium as sodium (typically 9oz versus 4oz). About 98% of total body potassium is inside our cells. Potassium is the principal cation (positive ion) of the fluid within cells and is important in controlling the activity of the heart, muscles, nervous system and just about every cell in the body. Potassium regulates the water balance and acid-base balance in the blood and tissues. Evidence is showing that potassium is also involved in bone calcification. Potassium is a cofactor in many reactions, especially those involving energy production and muscle building.
Prognosis
A prediction (estimate) of the future course and outcome of a disease and an indication of the likelihood of recovery from that disease.
Protein (Proteins)
Compounds composed of hydrogen, oxygen, and nitrogen present in the body and in foods that form complex combinations of amino acids. Protein is essential for life and is used for growth and repair. Foods that supply the body with protein include animal products, grains, legumes, and vegetables. Proteins from animal sources contain the essential amino acids. Proteins are changed to amino acids in the body.
Serotonin
A phenolic amine neurotransmitter (C10H12N2O) that is a powerful vasoconstrictor and is found especially in the brain, blood serum and gastric membranes of mammals. Considered essential for relaxation, sleep, and concentration.
Small Intestine (Small Bowel)
The small intestine lies between the stomach and the large intestine. It is about 6 meters (20 feet) long and its primary function is to digest (break down) food and absorb nutrients (vitamins, minerals, proteins, carbohydrates, and fats). The small intestine makes up more than 70% of the length and 90% of the surface area of the gastrointestinal (GI) tract.
Stenosis
Usually referring to Esophageal or GI tract: Narrowing.
Stomach
A hollow, muscular, J-shaped pouch located in the upper part of the abdomen to the left of the midline. The upper end (fundus) is large and dome-shaped; the area just below the fundus is called the body of the stomach. The fundus and the body are often referred to as the cardiac portion of the stomach. The lower (pyloric) portion curves downward and to the right and includes the antrum and the pylorus. The function of the stomach is to begin digestion by physically breaking down food received from the esophagus. The tissues of the stomach wall are composed of three types of muscle fibers: circular, longitudinal and oblique. These fibers create structural elasticity and contractibility, both of which are needed for digestion. The stomach mucosa contains cells which secrete hydrochloric acid and this in turn activates the other gastric enzymes pepsin and rennin. To protect itself from being destroyed by its own enzymes, the stomach’s mucous lining must constantly regenerate itself.
Syndrome
A medical condition characterized by a collection of related symptoms (what the patient feels) and signs (what a doctor can observe or measure).
Tryptophan
Essential amino acid. Natural relaxant and sleep aid due to its precursor role in serotonin (a neurotransmitter) synthesis. Along with tyrosine, it is used in the treatment of addictions.
Tumor (Tumors, Tumour, Tumours)
An abnormal growth of tissue resulting from uncontrolled, progressive multiplication of cells and serving no physiological function; a neoplasm. Literally, a swelling; in the past the term has been used in reference to any swelling of the body, no matter what the cause. However, the word is now being used almost exclusively to refer to a neoplastic mass, and the more general usage is being discarded.
X-rays (X-ray)
High-energy radiation used to take pictures of areas inside the body.
Last updated: Sep 13, 2009
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