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

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


JournalIndian Journal of Medical and Paediatric Oncology
Article typeOther
Publication date – Dec – 2022
Authors – Deepa Susan Joy Philip, Jyoti Bajpai
KeywordsAlveolar soft part sarcoma (ASPS), India, low-middle income countries (LMIC)
Open access – Yes
SpecialityMaxillofacial and oral surgery, Surgical oncology
World region Southern Asia
Country: India
Language – English
Submitted to the One Surgery Index on December 13, 2022 at 12:32 am
Abstract:

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 rare disease. In this review, we aim to give an overview of the approach to diagnosis and management of this orphan disease in 2022 in the Indian setting, which is widely applicable in other low-middle income countries (LMIC) as well.

OSI Number – 21837

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