OBJECTIVETo investigate the clinical and pathological features of paratesticular desmoplastic small round cell tumor (DSRCT), and to improve the diagnosis and treatment of the disease.

METHODSOne case of paratesticular DSRCT was studied retrospectively and a considerable amount of related literature from Medline and Chinese journals reviewed. The patient was a 27-year-old man presenting with a painless testicular mass in the left hemiscrotum. On physical examination, a cystic mass was palpable while the testis was not in the left hemiscrotum.

RESULTSDuring the operation the paratesticular area was found full of multiple nodular tumor masses of various sizes ranging from 0.5 cm to 1.5 cm in diameter. Pathological examination showed the characteristic histological pattern of nests of small undifferentiated cells embedded in a dense fibrous stroma. The tumor presented an immunohistochemical feature of epithelial, mesenchymal as well as neural multidirectional differentiation. Following testicular tumor orchiectomy, chemotherapy was performed with DDP, VP16, ifosfamide and EPI. Three years follow-up found no tumor recurrence.

CONCLUSIONDesmoplastic small round cell tumor has a specific clinicopathologic stigmata, usually occurring in young males, for which surgical resection with chemotherapy is the treatment of choice. DSRCT located in the paratesticular region may have a better prognosis than its more frequently abdominal counterpart.