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J Thorac Cardiovasc Surg 1996;111:134-141
© 1996 Mosby, Inc.


GENERAL THORACIC SURGERY

THYMIC NEUROENDOCRINE CARCINOMA (CARCINOID): A CLINICOPATHOLOGIC STUDY OF FOURTEEN CASES

Vincent Thomas de Montpréville, MDa, Paolo Macchiarini, MDb, Elisabeth Dulmet, MDa


Le Plessis-Robinson, France

Received for publication Dec. 28, 1994. Accepted for publication May 2, 1995. Address for reprints: V. Thomas de Montpréville, Service d'Anatomie Pathologique, Centre Chirurgical Marie Lannelongue, 133 Avenue de la Résistance, 92350 Le Plessis-Robinson, France.

Abstract

The medical records and histologic documents of 14 patients treated at our institution for a thymic carcinoid tumor were reviewed. There were 3 women and 11 men with an age range from 35 to 71 years. One patient had a multiple endocrine neoplasia syndrome; another had a neurofibromatosis. Twelve tumors were revealed by local symptoms and two were asymptomatic. One patient had Cushing's syndrome that appeared secondarily and was related to metastases. Tumors ranged from 6 to 20 cm and had the characteristic histologic appearance of atypical carcinoid tumor. Immunohistochemical evaluations were done. Tumors were positive for cytokeratin (92%), neuroendocrine markers (100%), and p53 oncoprotein (29%). S-100 protein antibody revealed numerous sustentacular cells in one case. Overall survival was 46% and 31% at 3 and 5 years, respectively. However, all patients died of the disease within 109 months as a result of local progression (n = 5), local relapse (n = 3), distant metastases (n = 8), or a combination of these reasons. Median survival was 71, 30, and 5 months for patients who had total resection (n = 4), partial resection (n = 5), or simple biopsy (n = 4), respectively (p = 0.023). In conclusion, thymic carcinoid tumors can be considered thymic neuroendocrine carcinomas because of their malignant behavior and histologic appearance of atypical carcinoid tumors. Complete surgical resection offers the best hope for long-term survival. (J THORACCARDIOVASCSURG1996;111:134-41)




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