Management of desmoplastic small round cell tumor

Semin Pediatr Surg. 2016 Oct;25(5):299-304. doi: 10.1053/j.sempedsurg.2016.09.005. Epub 2016 Sep 14.


Desmoplastic small round cell tumor (DSRCT) is a soft tissue sarcoma of mesenchymal cell origin that typically presents with multiple intra-abdominal tumors and exhibits a multi-phenotypic pattern of immunohistochemical staining. The specific organ or tissue type of origin has yet to be identified. DSRCT rarely arises as a singular tumor in the abdomen; in most cases, there are dozens to hundreds of abdominal peritoneal tumors that are detected on diagnosis. One very large dominant mass is usually present in the omentum, with an additional one or two large conglomerates of tumors in the pelvis and right peritoneum, respectively. Despite an often overwhelmingly large number of abdominal tumors, symptoms of bowel obstruction are rare. Ascites may be present. In late stages, pleural effusions, pleural implants, mediastinal adenopathy, supraclavicular adenopathy, or bone metastasis may be present. With this challenging disease, multidisciplinary therapy, including aggressive surgery, is warranted. This review will address DSRCT biology and treatment options and discuss outcomes.

Keywords: Childhood; HIPEC; Pediatric; Sarcoma; Sarcomatosis.

Publication types

  • Review

MeSH terms

  • Antineoplastic Agents / therapeutic use
  • Chemotherapy, Adjuvant
  • Cytoreduction Surgical Procedures
  • Desmoplastic Small Round Cell Tumor* / diagnosis
  • Desmoplastic Small Round Cell Tumor* / pathology
  • Desmoplastic Small Round Cell Tumor* / therapy
  • Humans
  • Neoplasm Staging
  • Peritoneal Neoplasms* / diagnosis
  • Peritoneal Neoplasms* / pathology
  • Peritoneal Neoplasms* / therapy
  • Peritoneum / surgery
  • Radiotherapy, Adjuvant


  • Antineoplastic Agents