ArticleFrontiers in pediatrics2025
Metastatic pediatric alveolar soft part sarcoma: a rare case report highlighting multidisciplinary treatment, molecular diagnostics, and novel therapeutic approaches.
Article in Frontiers in pediatrics, 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 an extremely rare soft tissue sarcoma, accounting for less than 1% of all soft tissue sarcomas. Approximately 5%-10% of ASPS cases occur in children and adolescents. Despite its indolent local course, its high metastatic potential necessitates comprehensive, multidisciplinary management. Case presentation: We present a pediatric patient diagnosed with metastatic ASPS originating from the left thigh. Initial management included wide local excision and adjuvant radiotherapy. Immunohistochemistry demonstrated strong nuclear TFE3 positivity, supporting the diagnosis. Although FISH analysis for ASPSCR1-TFE3 fusion was planned, technical limitations prevented archival imaging. Despite initial local control, the patient developed pulmonary, hepatic, brain, and intraabdominal metastases during follow-up. Serial imaging, including Conclusion: This case highlights the critical role of histopathological and molecular confirmation, multidisciplinary care, and emerging targeted and immunotherapy approaches in pediatric ASPS. Collaborative multicenter trials are urgently needed to establish evidence-based treatment strategies for this challenging disease.
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