A brief description of the disease, it's symptoms, diagnosis, treatment and prognosis. PLEASE do not rely solely on the information that I have presented. It represents my understanding as a layperson and is slanted towards my particular case. A good place to start looking at medical professional information about this disease is The Carcinoid Cancer Foundation's Website.

---

`

Overview: Carcinoid Cancer tumors produce enzymes and other chemicals that usually cause a variety of symptoms. The primary or beginning tumor can be located in almost any organ; however, they usually are found in the appendix and small bowel. Secondary tumors or Metastases are most often found in the liver, lymph nodes, mesentery or lung. Carcinoids grow very slowly compared to other cancers and it usually takes many years before they become sizable. The tumor pictured here is about 1 cm and was located on the small bowel. Most of the symptoms and deaths as well are related to chemicals produced by the tumor rather than the tumors themselves. A key thing to remember is that the tumors usually grow slowly and there is time to explore various options. For more detailed overview please read Diagnosis, Treatment and Management of Carcinoid Cancer presented by Richard R. Warner, M.D. of the Mt Sinai Medical Center in New York. Dr Warner is also the Medical Director of the Carcinoid Cancer Foundation.

Cause:

The cause of Carcinoid Cancer is unknown. No link has been found to family history, smoking, chemicals in the workplace or foods. A slight statistical (10%) increase in the number of cases in families with the MEN1 syndrome has been observed. As to gender the incidence between Caucasian men and women is equal. Statistically African-American men seem to have a slightly higher risk than women but the reason is not known. Persons with certain diseases that damage the stomach and reduce acid production have a higher risk of developing stomach carcinoids but their risk for other organs is not affected.

Symptoms:

Carcinoids are likely to grow for many years without producing any symptoms. Because of this most patients are diagnosed in advanced stages. The symptoms depend on the locationof the tumor and the individuals own body chemistry:

• Intermittant abdominal pain (not necessarily severe) and fluctuations in bowel function This is the most common sign of carcinoid cancer and often preceeds flushing by years.
• Flushing (Red face and bloodshot eyes) This is the second most common symptom of Carcinoid Cancer. This can be triggered by eating a large meal, alcohol consumption, tomatoes, fatty foods, coffee or other caffinated beverages, chocolate and spicy foods. Any kind of emotional distress and heavy exercise may also trigger flushing in excess of what is appropriate to the activity. It is due to enlargement of the vessels of the skin by chemical stimulation from chemicals released by the tumor.
• Diarrhea and other chronic digestive irregularities are also common symptoms.
• Shortness of breath or other asthma-like symptoms
• Weight loss

Any or all of these symptoms in patients with metastatic or widespread Carcinoid Cancer along with increased urine 5-HIAA and/or blood serotonin is referred to as Carcinoid Syndrome.

Diagnosis:

When suspected, a simple 5-HIAA urine test. blood chromogranin A and blood serotonin can usually quickly confirm the diagnosis. A CT scan or Ultrasound examination of the abdomen usually is used to further confirm the diagnosis and establish the site and extent of the disease. A Needle Biopsy of the Carcinoid may be used to confirm the existence of the cancer. More CT scans as well as an Octreoscan will probably be used to detect possible tumors in other parts of the body. Sometimes small bowel X-ray studies and endoscopy may also be used in the diagnostic process. The main impediment to making the diagnosis is not thinking of the Carcinoid Syndrome or even considering it because of it's rarity. Most Doctors have never seen or treated the disease and their only knowledge of it may be from medical school if at all.

Treatment:

Surgery: Surgery is the best treatment when it is possible to remove all of the tumor tissue and it can result in a complete cure. Unfortunately most cases of Carcinoid Cancer with the syndrome present are not diagnosed until distant spread has occurred and in these cases surgery will not usually cure the disease. Surgery may still need to be used to relieve mechanical obstructions such as bowel blockage or artery pinching. In some cases debulking surgery may also be used to reduce tumor size and relieve symptoms. This could involve simply excising the tumor or a large part of it or destroying it by Cryoablation (freezing) or by Radio Frequency Ablation (heat).

Chemoembolization: is also sometimes used to reduce the size of liver tumors. The artery that is supplying blood to the tumors is injected with a combination of embolic (plugging) material that is loaded with chemotherapy drugs. This reduces the blood flow to the tumors and also loads them with tumor destroying and growth inhibiting chemotherapy. Since the liver has a dual blood supply it is normally able to survive this treatment. Toxic effects of the chemotherapy on the rest of the body are reduced since most of the drugs go to the tumors rather than elsewhere. There is some risk in that the dying flesh can become infected and very painful but this is not usually the case.

Chemotherapy: is often used when other methods have failed. Results with single chemotherapy drugs have been disappointing with this disease and usually it is necessary to use them in combination which can have moderate to severe toxic effects. Numerous combinations have been tried with good effects in only 20-30% of cases. Fortunately though failure to respond to one combination does not necessarily mean that another combination won't work.

Radiation: therapy has not been useful in treating most of the usual tumor locations such as the liver. In general, it is used only to control the symptoms of the advanced disease or pain when tumors have spread to bone.

Sandostatin: was originally designed to control the symptoms of Carcinoid Cancer. It has now been found to also inhibit and sometimes retard the growth of tumors and is rapidly becoming the mainstay of treatment for advanced disease regardless of symptoms. This occurs because Sandostatin interferes with the chemical message that the tumors send to blood vessels to get them to grow and increase the tumor's blood supply. The tumor can only grow if it has a sufficient blood supply, therefore at the very least, growth is slowed temporarily. To learn more please click the following link to a paper presented May 11, 2000 by Eugene Woltering, M.D. of LA State U.Medical Center entitled The Role of Sandostatin Analogue in the Reduction of Tumor Growth.

Liver Transplant: Only a few patients have been subjected to this extremely expensive and debilitating treatment. Their survival has been about equal to those patients treated by more conventional means. This is probably not a good option except in the most extraordinary cases.

Treatment of symptoms:

Sandostatin (Octreotide Acetate) is the most effective drug for management of symptoms in most people. In some cases Interferon is also used, however it usefulness is often limited because of some unpleasant side effects (fatigue and flu-like symptoms which can be severe). Diet and lifestyle can also play a role in the control of symptoms. Almost all carcinoid patients who had or have Carcinoid Syndrome will tell you that prior to treatment a large meal or emotional distress would trigger the syndrome. Statistical and other studies have shown that other foods and beverages such as alcohol, caffeine and some seemingly unlikely ones such as raw tomatoes also may trigger the syndrome in many people. This writer has recently eliminated caffeine intake with very good results. To learn more about the dietary aspect please click the following link to a May 11, 2000 presentation by Monica E. Warner, M.S., R.D., C.D.N. of the Carcinoid Cancer Foundation that is entitled Nutritional Concerns for the Carcinoid Patient.

Pattern of Spread:

Regional spread can occur within the organ that contains the primary tumor. It is not uncommon to find more than one small tumor in the small bowel. Distant Spread most often occurs to the liver (65%), followed by lymph glands in the abdomen (34%), mesentery (19%) and lung (14%). Other less common locations include the small intestine, ovary, colon, pancreas and medastium. In a few cases spread to the bone, breast, thymus and kidneys has also occurred.

Prognosis:

In patients where the tumor is still localized and removable by surgery and who have not experienced Carcinoid Syndrome, surgery usually results in a complete cure.

In those tumors that are somewhat larger and have spread to local tissues and lymph nodes but are still completely removable by surgery, average survival is 8 years with a range up to 23 years and more.

In patients who have distant spread and functioning Carcinoid Syndrome the prognosis is not as good. Prior to the development of Sandostatin in 1988, the average survival of a patient from the onset of flushing was 3 years. From the time of diagnosis survival averaged about 2 years although the range extended to over 10 years. Fully 75% of patients died not from the tumors themselves but rather from the harmful effects of the chemicals released into the bloodstream by the tumors. The 5 year survival rate was only 18%.

In the last eleven years the survival rate has changed dramatically as has the quality of life with the disease - the 5 year survival rate now exceeds 67%. In most cases the top end of studies has not been reached which makes it difficult to give accurate predictions. Suffice it to say that there is good reason for hope.

Follow up

Regardless of the outcome of any treatment that has taken place, it is important that the patient remain under surveillance for any possibile recurrence of cancer. Patients whose tumors were completely removed by surgery in the early stages do not usually need an intense follow-up. Patients with advanced disease should be examined regularly for several years. The frequency of this follow-up will depend on the extent of the disease and the general physical health of the patient. During follow-ups, patients are examined, blood tests performed, if necessary, and a CT scan of the location of the tumors is done. In some cases it may be advisable to do an Otreoscan to find any widespread recurrance of the disease.

Future Treatments

The most "exciting" treatment being developed that this writer has heard about involves using the same sort of procedure that is used with an Octreoscan. Instead of a diagnostic dose of radiation being bonded to the octreotide that is used to carry the radiation into the tumors, a tumor killing dose of very short range beta particle radioactive material is used. The octreotide carries the radiation into the tumors themselves where the tumors are destroyed from the inside out without all of the harmful radiation effects to other parts of the body that occur with conventional radiation therapy. It sounds simple but I would imagine that things such as making certain that the medication goes only to the tumors and not to some other vital spot would be very difficult and obviously very important. This sort of treatment is especially important to someone like myself who has distant spread with multiple inoperable tumors. It also would be very important to a patient with micro-tumors. I am told that human trials are underway and this treatment may be available in a year or so.

Genetic research is also being done for all forms of Cancer and may eventually yield a real cure involving DNA/gene modifications that will restore the defective genes that cause cancer to their normal condition thereby causing cancer cells to revert back to being normal cells.

Finally, improvements on existing techniques are being developed daily; new chemotherapy drugs and combinations of drugs; Less invasive and debilitating forms of surgery such as Laproscopic surgery; Cryoablation (freezing); Radio Frequency Ablation (heat); and many others.