Thymic neuroendocrine carcinoma (carcinoid): a clinicopathologic study of fourteen cases.
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.
de Montpréville
V T
VT
Department of Pathology, Marie-Lannelongue Surgical Center, (Université Paris-Sud), Le Plessis-Robinson, France.
Macchiarini
P
P
Dulmet
E
E
D000328:Adult; D000368:Aged; D002276:Carcinoid Tumor / Q000175:diagnosis / Q000401:mortality / Q000473:pathology* / Q000601:surgery; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D016019:Survival Analysis; D015996:Survival Rate; D013950:Thymus Gland / Q000737:chemistry / Q000473:pathology*; D013953:Thymus Neoplasms / Q000175:diagnosis / Q000401:mortality / Q000473:pathology* / Q000601:surgery; D013997:Time Factors