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Alveolar soft part sarcoma in a child - a case report

Publication at Faculty of Medicine in Pilsen |
2023

Abstract

Background: Alveolar soft part sarcoma (ASPS) is a very rare mesenchymal malignancy of un-certain origin. It mostly affects young people, with about a quarter of cases being diagnosed in children.

Case: An 11-year-old girl had a painless subcutaneous "lump" in the left elbow area. Imaging exams revealed a solid soft-tissue intramuscular mass of suspicious appear-ance.

A surgical excision of lesion was performed. The bio psy consisted of a lobular tumor measuring 35 x 20 x 12 mm.

Histology revealed an epithelioid-cell population arranged in organoid pseudoalveolar pattern. It immunohistochemically expressed TFE3 and harbored the ASPSCR1::TFE3 gene fusion.

A diagnosis of ASPS was established. Subsequently, a wide re-ex-cision of the scar was performed without microscopic residual tumor.

The patient is currently without evidence of local recurrence or metastasis. Conclusion: ASPS is considered an aggre-ssive and prognostically unfavorable chemoresistant neoplasm.

Children have a better prognosis compared to adults. Early detection of tumor in a localized stage with complete surgical removal remains a mainstay therapeutic option.

Due to its tendency to late metastases, a long--term thorough follow-up of the patient is necessary.