Up to 5% of all small cell carcinomas develop at extrapulmonary sites. Primary small cell carcinomas originating in the kidney are extremely rare neoplasms. Here we report a case of primary small cell carcinoma of the kidney. A nephrectomy was performed on a 52-year-old female patient to remove a large tumor located in the right kidney. The histology and immunohistochemistry of the resected tumor revealed a pure small cell carcinoma with invasion into the renal capsule. The patient received postoperative adjuvant chemotherapy with etoposide and cisplatin. The patient has been monitored with regular check ups and remains stable with no recurrence at 28 months after the initial diagnosis.
*Nephrectomy ; Middle Aged ; Kidney Neoplasms/*diagnosis/drug therapy/surgery ; Humans ; Female ; Carcinoma, Small Cell/*diagnosis/drug therapy/surgery

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.

Adult ; Carcinoma, Small Cell ; diagnosis ; drug therapy ; surgery ; Combined Modality Therapy ; Fibroma, Desmoplastic ; diagnosis ; drug therapy ; surgery ; Humans ; Male ; Testicular Neoplasms ; diagnosis ; drug therapy ; surgery ; Treatment Outcome