Carcinoid Tumors

Carcinoid tumors arise from Kulchitsky's or enterochromaf-fin cells and have been described in almost every organ in the body. Carcinoid tumors occur most commonly in the intestine. Carcinoids of the pancreas are rare and make up less than 5% of all carcinoid tumors.9,38 In contrast with midgut carcinoids, they often secrete the serotonin analog hydroxytryptophan. Episodic flushing and diarrhea are the most common symptoms of the carcinoid syndrome and are usually associated with metastases to the liver from a small bowel carcinoid. Metastatic disease may require no treatment for months or even years if the patient is asymptomatic or the symptoms are mild.39

Numerous therapeutic options are available for the symptomatic patient, and it is important to consider the severity of symptoms and select the appropriate therapy for the clinical situation. Surgical resection is often curative for early-stage disease, and it can prolong survival and provide significant improvement in symptoms, even in patients with metastatic disease.9 Potentially curative surgery should be considered in all patients who can be rendered disease free with resection, and palliative surgery should be offered to selected patients because it improves symptoms and may delay or reduce the need for chemotherapy.40 Selected patients not suitable for hepatic resection for metastatic disease may benefit from hepatic artery embolization.41 However, this may be associated with significant side effects such as fever, nausea, and pain.

Chemotherapy can be considered for patients with progressive, symptomatic, unresectable disease. STZ-based regimens are helpful in the treatment of patients with carcinoid tumors of the pancreas.5-9 Etoposide and cisplatin have been given to 27 patients with carcinoid syndrome; unfortunately, only two patients showed partial tumor regression.39 In patients with aggressive variants of carcinoid tumor, the same combination was administered and a 67% response rate was noted. Patients with typical carcinoids did not respond in this study.42 Octreotide is effective for treating symptoms in patients with metastatic carcinoid and may result in disease stabilization or even objective tumor regression.39

Cyproheptadine (Periactin) is a serotonin and histamine antagonist and is indicated for diarrhea caused by carcinoid syndrome. Although it does not decrease circulating hormone levels, it blocks the effects of these hormones in the bowel. Most patients have significant relief of diarrhea. Interferon has been used in patients with metastatic carcinoid with the malignant carcinoid syndrome. Twenty percent of patients had a measurable regression of tumor, 40% had a decrease in hormone excretion, 65% had relief of flushing, and 33% were relieved of diarrhea. Unfortunately, these results were transient.43 The addition of interferon to somatostatin analogs has been helpful in patients resistant to octreotide alone.44

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