Neuroendocrine Tumours

Article about neuroendocrine tumours (NETS).

Definition

Neuroendocrine tumour refers to the type of cell that a tumour grows from rather than where that tumour is located. Neuroendocrine cells produce hormones or regulatory proteins, and so tumours of these cells usually have symptoms that are related to the specific hormones that they produce.

Neuroendocrine tumours - detailed technical article

Description

Neuroendocrine cells have roles both in the endocrine system and the nervous system. They produce and secrete a variety of regulatory hormones, or neuropeptides, which include neurotransmitters and growth factors. When these cells become cancerous, they grow and overproduce their specific neuropeptide.

Neuroendocrine tumours are generally rare. One type of neuroendocrine tumour is a carcinoid tumour. This type of tumour can occur in the intestinal tract, appendix, rectum, bronchial tubes, or ovary. Most carcinoid tumours secrete serotonin. When the blood concentration of this hormone is high enough, it causes carcinoid syndrome. This syndrome refers to a variety of symptoms that are caused by the excessive amount of hormone secreted rather than the tumour itself.

The total incidence of neuroendocrine tumours is thought to be between five and nine million people in the United States. It is possible that these tumours are underreported because they grow slowly and do not always produce dramatic symptoms.

Causes and symptoms

Many of the symptoms of carcinoid tumour are due to the hormones that the tumour secretes. These hormones can affect the whole body and cause what is referred to as carcinoid syndrome. The most common symptom of carcinoid syndrome is flushing, a sudden appearance of redness and warmth in the face and neck that can last from minutes to hours. Other symptoms of carcinoid syndrome are diarrhea, asthma-like symptoms and heart problems. Since most carcinoid tumours are found in the appendix, the symptoms are often similar to appendicitis, primarily pain in the abdomen. When these tumours are found in the small intestine, they can cause abdominal pain that is often initially diagnosed as bowel obstruction. Many patients have no symptoms and the carcinoids are found during routine endoscopy of the intestines.

Diagnosis

The diagnosis of carcinoid syndrome is made by the measurement of 5–hydroxy indole acetic acid (5–HIAA) in the urine. 5–HIAA is a breakdown (waste) product of serotonin. If the syndrome is diagnosed, the presence of carcinoid tumour is a given. When the syndrome is not present, diagnosis may be delayed, due to the vague symptoms present.

Diagnosis can sometimes take up to two years. It is made by performing a number of tests, and the specific test used depends on the tumour’s suspected location. The tests that may be performed include gastrointestinal endoscopy, chest x-ray, computed tomography scan (CT scan), magnetic resonance imaging, or ultrasound. A biopsy of the tumour is performed for diagnosis. A variety of hormones can be measured in the blood as well to indicate the presence of a carcinoid.

Treatment

The only effective treatment for carcinoid tumour is surgical removal of the tumour. Although chemotherapy is sometimes used when metastasis has occurred, it is rarely effective. The treatment for carcinoid syndrome is typically meant to decrease the severity of symptoms. Patients should avoid stress as well as foods that bring on the syndrome. Some medications can be given for symptomatic relief; for example, tumours of the gastrointestinal tract may be treated with octreotide (Sandostatin) or lanreotide (Somatuline) to relieve such symptoms as diarrhea and flushing. These drugs are known as somatostatin analogs. Liver transplantation is a treatment option for patients with neuroendocrine tumours that have metastasized only to the liver. This approach is reported to offer patients long, disease-free periods and relief of symptoms.

Prognosis

The prognosis of carcinoid tumours is related to the specific growth patterns of that tumour, as well as its location. For example, a group of researchers at the University of Wisconsin reported in 2004 that patients with gastrointestinal tumours in the hindgut had longer periods of disease-free survival than those with foregut or midgut cancers. For localized disease the five-year survival rate can be 94%, whereas for patients where metastasis has occurred, the average five-year survival rate is 18%. It is not unusual for patients with carcinoid tumours to live 10–15 years after the initial diagnosis.

Prevention

Neuroendocrine tumours such as carcinoid tumours are rare, and no information consequently is yet available on cause or prevention.

Neuroendocrine tumours - detailed technical article

Resources 

Periodicals 

Ahlman, H., S. Friman, C. Cahli, et al. ‘‘Liver Transplantation for Treatment of Metastatic Neuroendocrine tumours.’’ Annals of the New York Academy of Sciences 1014 (April 2004): 265–269.

Oberg, K., L. Kvols, M. Caplin, et al. ‘‘Consensus Report on theUse of Somatostatin Analogs for the Management of Neuroendocrine tumours of the Gastroenteropancreatic System.’’ Annals of Oncology 15 (June 2004): 966–973.

Van Gompel, J. J., R. S. Sippel, T. F. Warner, and H. Chen. ‘‘Gastrointestinal Carcinoid tumours: Factors That Predict Outcome.’’ World Journal of Surgery 28 (April 2004): 387–392. 

Organizations 

Carcinoid Cancer Foundation, 333 Mamaroneck Ave. #492, New York, NY, 10605, (888) 722-3132, http://www.carcinoid.org