Clinical Presentation and Management of Carcinoid Tumors

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The generally indolent nature of neuroendocrine tumors is an advantage in the management of patients who have localized disease, and surgery alone is often curative. This same property presents a challenge in the treatment of patients who have metastatic disease, in whom standard cytotoxic chemotherapy has a limited benefit. In such patients, the use of somatostatin analogs, interferon, and the treatment of hepatic metastases may provide effective palliation. The highly vascular nature of carcinoid tumors has led to the investigation of antiangiogenic agents in this setting. Preliminary reports of activity associated with agents targeting the vascular endothelial growth factor pathway suggest that such strategies may play a role in the future treatment of patients who have this disease.

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Incidence

The incidence of carcinoid tumors has been estimated to be 1 to 2 per 100,000 population [2]. Because carcinoids often pursue an indolent clinical course, their true incidence is likely much higher. A Swedish series, in which the incidence of carcinoid tumors was evaluated in surgical specimens and autopsies in a single geographic location, estimated the true incidence of carcinoids to be 8.4 cases per 100,000 population, suggesting that a large proportion of patients who have carcinoid tumors

Clinical Presentation and Management of Localized Disease

A commonly used classification scheme groups carcinoid tumors according to their presumed derivation from the embryonic gut: foregut (bronchial and gastric), midgut (small intestine and appendiceal), and hindgut (rectal). The clinical presentation and management of these tumors varies, depending on their site of origin (Table 2).

Imaging Techniques

Patients in whom metastatic disease is suspected are generally first evaluated with an abdominal CT scan to rule out liver metastases. Liver function tests are an unreliable indicator of tumor involvement, and the serum alkaline phosphatase is frequently normal despite extensive liver involvement by carcinoid tumor. Carcinoid liver metastases are often hypervascular, and may become isodense relative to the liver with the administration of intravenous contrast. CT scans should thus be performed

Summary

The generally indolent nature of neuroendocrine tumors is an advantage in the management of patients who have localized disease, and surgery alone is often curative in such cases. This same property presents a challenge in the treatment of patients who have metastatic disease, in whom standard cytotoxic chemotherapy has only limited benefit. In such patients, the use of somatostatin analogs, interferon, and the treatment of hepatic metastases may provide effective palliation. The highly

Acknowledgments

The author would like to thank Taylor S. Spear for invaluable assistance in the preparation and editing of the manuscript.

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    M. H. Kulke is supported in part by NIH grants K23 CA 093401, K30 HL04095, and gifts from Raymond and Beverly Sackler, the Caring for Carcinoid Foundation, and the Caroline Kaufer Fund for Neuroendocrine Tumor Research.

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