Abstract
Desmoplastic small round cell tumor (DSRCT) is a rare and highly aggressive mesenchymal tumor that develops in the abdominal cavity of young men adults. Patients typically present with symptoms of abdominal sarcomatosis. Diagnosis is based on histological analysis of biopsies which typically show small round blue cells in nests separated by an abundant desmoplastic stroma. DSRCT is associated with a unique chromosomal translocation t(11:22) (p 13; q 12) that involves the EWSR1 and WT1 genes. The prognosis is particularly poor; median survival ranges from 17 to 25 months, largely due to the presentation of the majority of patients with metastatic disease. Management of DSRCT remains challenging and current schemes lack a significant cure rate despite the use of aggressive treatments such as polychemotherapy, debulking surgery and whole abdominal radiation. Several methods are being evaluated to improve survival: addition of chemotherapy and targeted therapies to standard neoadjuvant protocol, completion of surgical resection with HIPEC, postoperative IMRT, treatment of hepatic metastases with [90Y]Yttrium microsphere liver embolization.
Highlights
Desmoplastic small round cell tumor (DSRCT) is a rare and highly aggressive mesenchymal tumor that was first described as a separate identity in 1989 by Gerald and Rosai [1]; since that time, fewer than 200 cases have been reported in the literature
Data supporting the use of hyperthermic intraperitoneal chemotherapy (HIPEC) in patients with DSRCT is limited and this technique is not recommended for the management of patients with DSRCT outside clinical trials
Pinnix et al reported a series of 8 patients treated with whole abdominopelvic Intensity-Modulated Radiation Therapy (IMRT) after neoadjuvant chemotherapy and debulking surgery [20]
Summary
Desmoplastic small round cell tumor (DSRCT) is a rare and highly aggressive mesenchymal tumor that was first described as a separate identity in 1989 by Gerald and Rosai [1]; since that time, fewer than 200 cases have been reported in the literature. The tumor typically develops in the abdominal cavity, invading the omentum with multiple peritoneal implants involving the diaphragm, splenic hilum, mesentery of small and large bowel, and the pelvic peritoneum. Involvement of extraperitoneal organs, such as the testes, ovaries, and pleura has been described in literature. As for of others intraabdominal tumors, patients may be asymptomatic for long periods of time and diagnosis is made when tumor burden is large. The search for visceral metastasis (hepatic and/or pulmonary) with CT scan is typically used
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