Spindle-cell neuroendocrine carcinomas of the thymus (spindle-cell thymic carcinoid): a clinicopathologic and immunohistochemical study of seven cases

Abstract

Seven cases of spindle-cell neuroendocrine carcinomas (carcinoid tumors) of the thymus are presented. The patients were three women and four men between the ages of 26 and 74 years (median age, 50 yr). The lesions presented as large anterior mediastinal masses on radiographic examination and were treated by surgical excision. Grossly, the tumors were tan-brown and well circumscribed but encapsulated, and they measured from 2 to 15 cm in greatest diameter. Histologically, they were characterized by a dense proliferation of spindle cells that focally adopted a vaguely organoid pattern, with discrete nests of tumor cells separated by thin fibrovascular septa. Mitotic figures were present in all of our cases and ranged from 2 to 8 per 10 high power fields. Focal areas of necrosis were also present in all of the cases. Immunohistochemical studies performed in six cases showed positive staining for chromogranin in five cases, synaptophysin and keratin in four, and Leu 7 in three. Clinical follow-up showed that two patients died of their tumors 6 and 11 years after diagnosis; one was alive 8 years after diagnosis. Spindle-cell neuroendocrine carcinomas of the thymus (spindle-cell thymic carcinoids) should be considered in the differential diagnosis of spindle-cell neoplasms of the anterior mediastinum. Because of their aggressive clinical behavior, it is important to separate them from the other benign or low-grade spindle-cell tumors that are more common at this location.