Carcinoid Tumor

Carcinoid tumors belong to a larger group of tumors called neuroendocrine tumors (NET's). This broad group of tumors also includes the pancreatic islet cell tumors. Although both carcinoid and pancreatic islet cell tumors share similar morphological features, they secrete different classes of either bioactive amines or peptides, respectively. Carcinoid tumors are the most common form of NET's. The majority of carcinoids arise in the appendix and to a lesser extent in the small bowel, stomach, and duodenum whereas pancreatic islet cell tumors arise predominantly int the panreas.

Carcinoid tumors are generally quite rare. Although the majority of patients do not have symptoms associated with the tumor, when flushing and diarrhea occurs, the patient is said to have Carcinoid Syndrome. It is estimated that approximately 10% of patients with carcinoid tumors will develop the syndrome. The overall incidence of carcinoid tumors is approximately 2.0 to 2.5 per 100,000 population. The most common site for men is the small intestine whereas in women it is the appendix. A positive family history is an important risk factor of carcinoid tumors and there is an association with the development of Multiple Endocrine Neoplasia Type 1 (MEN-1). The majority of patients with NET's present in the fifth decade of life.

Depending on the clinical presentation, various imaging or endoscopic diagnostic studies may be performed. The Octreoscan is particularly useful for diagnosing the location of the tumor. Computerized tomography and/or magnetic resonance imaging are also useful to determine if liver metastases are present. Small bowel enteroscopy should be performed as well as colonoscopy for localizing the tumors. Recently, capsule endoscopy has been introduced and may permit evaluation for intestinal carcinoids.

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