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Related Experiment Videos

Intra-abdominal desmoplastic small round cell tumor.

J D Nathan1, C Gingalewski, R R Salem

  • 1Department of Surgery, Yale University School of Medicine, New Haven, Connecticut, USA. natha002@mc.duke.edu

The Yale Journal of Biology and Medicine
|March 16, 2001
PubMed
Summary

Desmoplastic small round cell tumors are aggressive abdominal cancers in young males. Despite chemotherapy and surgery, complete tumor removal and cure remain challenging, highlighting the poor prognosis.

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Area of Science:

  • Oncology
  • Pathology

Background:

  • Intra-abdominal desmoplastic small round cell tumor (DSRCT) is a rare, aggressive malignancy primarily affecting young males.
  • DSRCT presents unique histological and immunocytochemical features, differentiating it from other small round cell tumors.
  • The tumor's aggressive spread, resistance to chemotherapy, and difficulty in complete surgical resection contribute to a poor prognosis.

Observation:

  • A 39-year-old male presented with diffuse abdominal and pelvic involvement of DSRCT.
  • CT-guided biopsy revealed characteristic DSRCT histology with positive epithelial, mesenchymal, and neural markers.
  • The patient received neoadjuvant chemotherapy followed by surgery.

Findings:

  • Chemotherapy resulted in a 10-15% tumor mass regression.

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  • Surgical exploration identified extensive omental, colonic, pelvic, diaphragmatic, peritoneal, and mesenteric involvement.
  • Complete surgical resection was not achievable.

    • Implications:

    • DSRCT remains a highly aggressive cancer with a dismal prognosis.
    • While chemotherapy may induce partial tumor response, complete surgical resection is rarely possible.
    • Surgical interventions are often palliative in managing DSRCT.