Clinical and computed tomography features of adult abdominopelvic desmoplastic small round cell tumor

World J Gastroenterol. 2014 May 7;20(17):5157-64. doi: 10.3748/wjg.v20.i17.5157.

Abstract

To investigate the clinical and computed tomography (CT) features of desmoplastic small round cell tumor (DSRCT), we retrospectively analyzed the clinical presentations, treatment and outcome, as well as CT manifestations of four cases of DSRCT confirmed by surgery and pathology. The CT manifestations of DSRCT were as follows: (1) multiple soft-tissue masses or diffuse peritoneal thickening in the abdomen and pelvis, with the dominant mass usually located in the pelvic cavity; (2) masses without an apparent organ-based primary site; (3) mild to moderate homogeneous or heterogeneous enhancement in solid area on enhanced CT; and (4) secondary manifestations, such as ascites, hepatic metastases, lymphadenopathy, hydronephrosis and hydroureter. The prognosis and overall survival rates were generally poor. Commonly used treatment strategies including aggressive tumor resection, polychemotherapy, and radiotherapy, showed various therapeutic effects. CT of DSRCT shows characteristic features that are helpful in diagnosis. Early discovery and complete resection, coupled with postoperative adjuvant chemotherapy, are important for prognosis of DSRCT. Whole abdominopelvic rather than locoregional radiotherapy is more effective for unresectable DSRCT.

Keywords: Clinical features; Computed tomography; Desmoplastic small round cell tumor; Pathology; Peritoneum.

Publication types

  • Case Reports

MeSH terms

  • Abdominal Neoplasms / chemistry
  • Abdominal Neoplasms / diagnosis*
  • Abdominal Neoplasms / diagnostic imaging
  • Abdominal Neoplasms / pathology
  • Abdominal Neoplasms / therapy
  • Adult
  • Biomarkers, Tumor / analysis
  • Biopsy
  • Chemotherapy, Adjuvant
  • Desmoplastic Small Round Cell Tumor / chemistry
  • Desmoplastic Small Round Cell Tumor / diagnosis*
  • Desmoplastic Small Round Cell Tumor / diagnostic imaging
  • Desmoplastic Small Round Cell Tumor / secondary
  • Desmoplastic Small Round Cell Tumor / therapy
  • Fatal Outcome
  • Female
  • Humans
  • Immunohistochemistry
  • Male
  • Middle Aged
  • Pelvic Neoplasms / chemistry
  • Pelvic Neoplasms / diagnosis*
  • Pelvic Neoplasms / diagnostic imaging
  • Pelvic Neoplasms / pathology
  • Pelvic Neoplasms / therapy
  • Predictive Value of Tests
  • Radiotherapy Dosage
  • Retrospective Studies
  • Tomography, X-Ray Computed*
  • Treatment Outcome
  • Ultrasonography, Doppler, Color
  • Young Adult

Substances

  • Biomarkers, Tumor