Ovarian involvement of a desmoplastic small round cell tumor of unknown primary origin with lymph node and lung metastases: A case report.

Desmoplastic small round cell tumors (DSRCTs) were initially characterized as exhibiting divergent differentiation and were extremely aggressive, belonging to the family of 'small round blue cell tumors'. Due to a male predominance, to date, only 15 cases in women have been reported in the English literature. The present study describes a case of DSRCT in a young woman who initially presented with ovarian masses accompanied with lymph node and lung metastases. A correct diagnosis was reached by combining the hematoxylin and eosin, and immunohistochemical staining results. Following surgery, the patient underwent the chemotherapy and, 3 months later, is in a good condition. The study also provides an overview of this uncommon disease.