Soft Tissue Sarcomas
A 22-year-old woman presents with a slowly growing, painless mass in her right thigh that has been present for 18 months. MRI shows a well-defined, highly vascular 6cm mass in the anterior compartment with prominent feeding vessels. Biopsy reveals nests of polygonal cells with abundant eosinophilic cytoplasm containing characteristic PAS-positive, diastase-resistant crystals. Molecular testing confirms ASPSCR1-TFE3 fusion. Regarding alveolar soft part sarcoma (ASPS):
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Alveolar soft part sarcoma is a rare soft tissue sarcoma (less than 1% of STS) that typically affect...
ASPS is characterized by a specific translocation t(X;17)(p11;q25) resulting in the ASPSCR1-TFE3 fus...
ASPS is a common sarcoma affecting elderly patients; the most common site is the retroperitoneum; it...
ASPS is highly vascular on imaging (MRI shows flow voids, prominent feeding vessels, intense enhance...
Treatment is surgical with wide resection; ASPS is relatively resistant to conventional chemotherapy...
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