ArticleInternational medical case reports journal2025
Alveolar Soft Part Sarcoma in a 44-year-Old Female: A Case from Uganda.
Article in International medical case reports journal, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
Background: Alveolar Soft Part Sarcoma (ASPS) is a rare, highly vascular soft tissue sarcoma characterized by the specific ASPSCR1-TFE3 fusion, typically affecting adolescents and young adults. Despite its often-indolent growth, it has a high propensity for late hematogenous metastasis. Management for localized disease is centered on wide surgical excision. Case Presentation: We report a case of ASPS in a 44-year-old female from the Ankole tribe, presenting with a slow-growing 8 cm mass in her right upper arm over two years. This age of presentation is outside the typical peak incidence. The diagnosis was established solely on classic histopathological features-a pseudoalveolar pattern, large polygonal cells with abundant eosinophilic cytoplasm, and PAS-positive, diastase-resistant intracytoplasmic granules-due to resource limitations precluding molecular confirmation (TFE3 IHC/FISH). She underwent wide local excision with negative margins and remains free of recurrence at 12 months post-surgery. Conclusion: This case highlights the occurrence of ASPS in an older-than-typical patient and underscores the enduring reliability of classic histopathology for definitive diagnosis in resource-limited settings like Uganda. Although surgically cured locally, the patient's large tumor size and age confer a high risk for future metastasis, mandating rigorous, long-term surveillance.
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