How I Treat Alveolar Soft Part Sarcoma? The Therapeutic Journey from Nihilism to Cautious Optimism…

Alveolar soft part sarcoma (ASPS) is a very rare subtype, constituting less than 0.5% of malignant Soft tissue sarcoma.1 It is an orphan disease affecting adolescents and young adults, predominantly females.2 The rarity of the disease, with its indolent but relentless natural history and enigmatic line of differentiation, makes its diagnosis a challenge. Despite being a chemoresistant disease, it is known for prolonged survival even in a few metastatic patients with spontaneous disease stabilization and indolent disease behavior. Targeted therapy with antiangiogenic agents and immunotherapy is the way forward for this raredisease. In this review,weaimtogiveanoverviewof the approach to diagnosis and management of this orphan disease in 2022 in the Indian setting, which iswidely applicable in other low-middle income countries (LMIC) as well.