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.
Keywords: desmoplastic small round cell tumor; immunohistochemistry; ovary.