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Related Experiment Videos

Intra-abdominal desmoplastic small round cell tumor

G Yeoh1, P Russell, E J Wills

  • 1Department of Anatomical Pathology, Royal Prince Alfred Hospital, Sydney.

Pathology
|April 1, 1993
PubMed
Summary

A rare aggressive tumor in children and adolescents, primarily found in the abdomen, has been identified. This distinct small round cell tumor (SRCT) presents unique growth patterns and cellular characteristics.

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Area of Science:

  • Pediatric Oncology
  • Surgical Pathology
  • Tumor Biology

Background:

  • Small round cell tumors (SRCT) represent a heterogeneous group of aggressive neoplasms in pediatric patients.
  • Intra-abdominal tumors in children can be challenging to diagnose due to overlapping features.
  • Recent literature describes a distinctive aggressive malignant tumor predominantly occurring intrabdominally in young individuals.

Observation:

  • This case report details a mass in the greater omentum of a 15-year-old girl.
  • The tumor exhibited a diffuse growth pattern of small cells with hyperchromatic nuclei and scant cytoplasm.
  • Key features included patchy epithelial differentiation, co-expression of keratin and desmin, and a desmoplastic stromal component.

Findings:

  • The observed tumor aligns with the recently described aggressive intra-abdominal malignant tumor in childhood and adolescence.

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  • Immunohistochemical analysis revealed co-expression of keratin and desmin intermediate filaments.
  • A focal, pronounced desmoplastic stromal reaction was a notable characteristic.

    • Implications:

    • This case adds to the limited number of reported cases (33) of this rare SRCT variant.
    • Accurate diagnosis is crucial for appropriate management and understanding the prognosis of this aggressive tumor.
    • Further research into the pathogenesis and optimal treatment strategies for this distinct entity is warranted.