Management of desmoplastic small round cell tumor. Review uri icon

Overview

abstract

  • 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.

publication date

  • September 14, 2016

Research

keywords

  • Desmoplastic Small Round Cell Tumor
  • Peritoneal Neoplasms

Identity

PubMed Central ID

  • PMC5614508

Scopus Document Identifier

  • 85003454663

Digital Object Identifier (DOI)

  • 10.1053/j.sempedsurg.2016.09.005

PubMed ID

  • 27955733

Additional Document Info

volume

  • 25

issue

  • 5