Neuroendocrine carcinomas (carcinoid tumor) of the thymus. A clinicopathologic analysis of 80 cases.

Abstract

We studied 80 cases of primary thymic neuroendocrine carcinomas. Most patients had symptoms; approximately one third were asymptomatic. All cases were treated by surgical excision. The tumors were divided according to histopathologic features into low- (n = 29), intermediate- (n = 36), and high-grade (n = 15) types. The tumors displayed a variegated histologic appearance and unusual cytologic features. Some cases showed transition from low to high grade within the same tumor mass. Mitotic activity ranged from fewer than 3 to more than 10 mitotic figures per 10 high-power fields, and most tumors displayed marked cellular atypia and areas of necrosis. In 73 patients, the tumor was confined to the anterior mediastinum. Positive immunohistochemical reaction was observed using antibodies for CAM 5.2 low-molecular-weight cytokeratins, broad-spectrum keratin, chromogranin, synaptophysin, and Leu-7. The clinical follow-up obtained in 50 patients correlated well with tumor differentiation. Therefore, the behavior of these tumors seems to correlate with histologic grade, which seems directly proportional to degree of differentiation. We propose replacing the term thymic carcinoid with thymic neuroendocrine carcinoma, which better reflects the aggressive biologic behavior of these tumors in the mediastinal location.