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ASPS — SCE Medical Oncology MCQ

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HardSarcomaASPSSCE Medical Oncology

A 40-year-old woman with alveolar soft part sarcoma (ASPS) of the thigh has metastatic lung disease that has been stable for 3 years. She asks about systemic therapy. What is unique about ASPS biology and treatment?

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Correct answer: A — ASPS is characterised by ASPSCR1-TFE3 fusion, is highly vascular, has an indolent course with late metastases, and is relatively chemoresistant — anti-angiogenic TKIs (sunitinib, pazopanib, cediranib) and immunotherapy (atezolizumab) have shown activity

Alveolar soft part sarcoma is a rare sarcoma with distinctive features: ASPSCR1-TFE3 fusion (diagnostic), high vascularity, indolent but persistent course, late metastases (lung, brain — years-decades after diagnosis), and resistance to conventional chemotherapy (doxorubicin, ifosfamide). Anti-angiogenic TKIs exploit its hypervascularity (cediranib — CASPS trial; sunitinib; pazopanib). Atezolizumab showed promising activity in the phase II trial by Daimon et al. Brain surveillance is important as ASPS has a propensity for CNS metastases.

Reference: ESMO 2024 STS; CASPS trial; Daimon et al JAMA Oncol 2022