Retroperitoneal Desmoplastic Small Round Cell Tumor

Lee, Seung Hyun; Ahn, Byung Kwon; Baek, Sung Uhn; Kim, Young Ok

Abstract

A desmoplastic small round cell tumor (DSRCT) is a rare and aggressive neoplasm with an extremely poor prognosis. It primarily affects children and young males. The most common sites are the abdomen areas, particularly the pelvic region. Usually, it is a large tumor mass accompanied by extensive peritoneal seeding. Microscopically, the typical appearance is a well-defined nests or clusters of small round tumor cells with an undifferentiated type, surrounded by a prominent desmoplastic stroma. Immunohistochemically, the tumor cells show a primitive uncertain histogenesis that can simultaneously express the epithelial, mesenchymal, and neural phenotype. There is no definitive treatment modality. Although a surgical resection, chemotherapy and radiotherapy are to the main treatments, the prognosis is poor. We experienced a case of a desmoplastic small round cell tumor at the retroperitoneal space. A 42-year-old woman was admitted for a palpable mass in the left upper abdomen for 1 months. Compoterized tomography revealed a retroperitoneal tumor. A complete excision of the tumor was performed. The histological examination confirmed the diagnosis of a desmoplastic small round cell tumor. The tumor recurred 3 months later. She received the first chemotherapy regimin with high-dose cyclophosphamied, adriamycin and vincristine. Computed tomography showed that the recurred tumor disappeared after 6 cycles of chemotherapy, but it relapsed 6 months later. She died of sepsis during the second chemotherapy regimen. The survival time was 21 months.