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Thymic neuroendocrine carcinoma (carcinoid): A clinicopathologic study of fourteen cases

Identifieur interne : 001359 ( France/Analysis ); précédent : 001358; suivant : 001360

Thymic neuroendocrine carcinoma (carcinoid): A clinicopathologic study of fourteen cases

Auteurs : Vincent Thomas De Montpréville [France] ; Paolo Macchiarini [France] ; Elisabeth Dulmet [France]

Source :

RBID : ISTEX:8D48E46A7CBA88AE68B33464A63F0DEDFD4E5B78

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 THORAC CARDIOVASC SURG 1996;111:134-41)

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DOI: 10.1016/S0022-5223(96)70409-9


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ISTEX:8D48E46A7CBA88AE68B33464A63F0DEDFD4E5B78

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<div type="abstract" xml:lang="en">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 THORAC CARDIOVASC SURG 1996;111:134-41)</div>
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