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Alveolar soft part sarcoma in a child - a case report
Summary
Alveolar soft part sarcoma (ASPS) is a rare cancer. Complete surgical removal offers the best chance for a cure, especially in children, but long-term monitoring is crucial due to metastasis risk.
Area of Science:
- Oncology
- Pediatric Oncology
- Genetics
Background:
- Alveolar soft part sarcoma (ASPS) is a rare mesenchymal malignancy with uncertain origins.
- ASPS predominantly affects young individuals, with approximately 25% of diagnoses occurring in children.
Observation:
- An 11-year-old girl presented with a painless subcutaneous mass in her left elbow.
- Imaging revealed a suspicious intramuscular soft-tissue mass, confirmed by biopsy as ASPS.
- Histology showed an epithelioid-cell population with a pseudoalveolar pattern, TFE3 expression, and the ASPSCR1::TFE3 gene fusion.
Findings:
- Surgical excision of the ASPS lesion was performed.
- A subsequent wide re-excision of the scar achieved complete tumor removal without microscopic residual disease.
- The patient remains disease-free with no evidence of local recurrence or metastasis.
Implications:
- ASPS is an aggressive, chemoresistant neoplasm with a better prognosis in children than adults.
- Early detection and complete surgical resection are critical for favorable outcomes.
- Long-term follow-up is essential due to the potential for late metastases.
