Carcinoid Tumors

Carcinoid tumors (particularly foregut) are also over-represented in MEN 1 patients.18"22 They are all capable of behaving in a malignant fashion and as a group represent the second most common cause of tumor-related deaths in MEN 1 patients after pancreatic neuroendocrine tumors. Thymic carcinoids are highly aggressive. Management of such foregut tumors is by appropriate surgical resection. Transcervical thymectomy performed as part of HPT operations is not a guarantee against future development of thymic carcinoids.173"175

Gastroduodenal carcinoid tumors can be nonfunctioning or associated with gastrin or serotonin production.21176 Duodenal carcinoids are the principle cause of ZES in MEN 1 patients.22-50,140 They are often multiple and frequently metastasize to regional lymph nodes and subsequently to the liver. Gastric carcinoids can be gastrin producing (highly malignant) or can evolve secondary to the hypergastrinemia from gastrin-producing duodenal carcinoids (enterochro-maffin cell hyperplasia).21,176 Treatment requires excision and lymphadenectomy and, less often, gastrectomy or pancreatoduodenectomy, depending on the extent of foregut involvement.

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