Paratesticular desmoplastic small round cell tumor: case report

The desmoplastic small round cell tumor has recently been separated from other small round cell tumors because of its characteristic pathological and clinical features. They are usually intra-abdominal tumors affecting young people and have classically been associated with a bad prognosis. However, in recent years there have reports on desmoplastic small round cell tumors affecting other body regions, including the paratesticular area.

We report the case of a 23-year-old male, that consulted on a progressive enlargement of the right hemiscrotum in the last year and a half. He referred no previous urological symptoms and had no systemic symptomatology. Physical examination revealed a round elastic firm 2 to 3 cm mass distal to the tail of the epididymis, which was excised with a preoperative diagnosis of adenomatoid tumor. However, histological and immunohistochemical diagnosis confirmed a desmoplastic small round cell tumor. The extension study included a computed tomography scan and a plain chest radiograph, that showed no metastasis. The patient received chemoradiation therapy with methotrexate, dacarbacin, cyclophosphamide, actinomycin D and vincristin, but had to be changed to a vincristin, actinomycin D, cyclophosphamide and adriamicin scheme on severe toxicity. He completed five cycles of the chemotherapy with moderate toxicity. Today, 6 years after diagnosis the patient remains well and free of disease.

Recent reviews on desmoplastic small round cell tumor affecting the paratesticular area have shown a better prognosis for tumors of this origin compared to abdominal ones. We should include this lesion among the differential diagnosis of paratesticular tumors, mainly in children and adolescents.