Desmoplastic small round cell tumor: a case report and review of the literature

World J Surg Oncol. 2014 Jan 10:12:9. doi: 10.1186/1477-7819-12-9.

Abstract

Desmoplastic small round cell tumor is a rare malignant tumor that has a poor prognosis. It affects predominantly young males. In the current report, a 14-year-old male patient was admitted to the hospital for evaluation of abdominal distension, and abdominal pain. Imaging examination revealed a high prevalence of multiple intraperitoneal and liver parenchymal cystic and solid tumors. After an explorative surgery, the pathological findings confirmed the presentation of desmoplastic small round cell tumor. Diagnosis of desmoplastic small round cell tumor could easily have been overlooked since there was no specific evidence for this condition available in the clinical and imaging examinations. In the present study, ultrasound examination detected solid cystic masses, which suggested the presence of necrosis and hemorrhage. Immunohistochemistry and cytogenetic studies confirmed the diagnosis of desmoplastic small round cell tumor in this patient.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Abdominal Neoplasms / metabolism
  • Abdominal Neoplasms / pathology*
  • Adolescent
  • Biomarkers, Tumor / metabolism*
  • Desmoplastic Small Round Cell Tumor / metabolism
  • Desmoplastic Small Round Cell Tumor / pathology*
  • Humans
  • Immunoenzyme Techniques
  • Male
  • Prognosis

Substances

  • Biomarkers, Tumor