Abstract
BACKGROUND: Malignant triton tumor (MTT) is a rare and histological complexity characterized by a mixture of peripheral nerve sheath tumors and with rhabdomyoblastic differentiation. It follows a particularly malignant course. CASE PRESENTATION: In the present study, we report the first MTT of epididymis. The patient is a 22-year-old male presented with swelling in the left scrotum over a 2-month period. He did not have the history or symptoms of neurofibromatosis type 1. A mass measured about 3 cm × 4 cm was found in the left epididymis by ultrasound and CT scan. It was diagnosed as epididymis tumor and underwent exploration; intraoperative frozen section was diagnosed malignant tumor and treated with radical orchidoepididymectomy. The pathological report was malignant triton tumor. Despite taken high-dose radiation therapy and followed by chemotherapy for four cycles, he was died of progressive disease with multiple metastases 26 months after surgery. The clinic pathologic characteristics and optimal treatment strategy are reviewed.