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Low-grade fibromyxoid sarcoma

M Fukunaga1, S Ushigome, N Fukunaga

  • 1Department of Pathology, Jikei University School of Medicine, Tokyo, Japan.

Virchows Archiv : an International Journal of Pathology
|November 1, 1996
PubMed
Summary
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This case report details a rare low-grade fibromyxoid sarcoma in a 16-year-old Japanese girl. The thigh tumor showed no signs of recurrence 11 months post-surgery.

Area of Science:

  • Orthopedic Oncology
  • Soft Tissue Pathology
  • Pediatric Sarcoma Research

Background:

  • Low-grade fibromyxoid sarcoma (LGFMS) is a rare soft tissue tumor.
  • This report focuses on a unique pediatric case presenting in the thigh.

Observation:

  • A 16-year-old female presented with a 3.5 cm thigh mass and pain.
  • Microscopic examination revealed a non-encapsulated, infiltrating tumor with spindle cells, myxoid areas, and fibrous components.
  • No significant atypia, mitotic activity, or necrosis was observed.

Findings:

  • Immunohistochemistry showed strong vimentin and desmin positivity, with some alpha smooth muscle actin expression.
  • Tumor cells were negative for markers including CAM5.2, EMA, HHF35, S-100, CD34, and CD31.
  • Flow cytometry confirmed a diploid DNA content with a low S-phase fraction (6.6%).

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Implications:

  • This case expands the understanding of LGFMS presentation in adolescents.
  • The findings support LGFMS as a distinct entity with specific immunohistochemical markers.
  • Successful surgical excision resulted in no evidence of disease, highlighting favorable prognosis for localized LGFMS.