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CC BY 4.0 · Indian J Med Paediatr Oncol 2024; 45(01): 068-073
DOI: 10.1055/s-0042-1758540
How I Treat

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

Deepa Susan Joy Philip
1   Department of Medical Oncology, Regional Cancer Centre, Trivandrum, Kerala, India
,
Jyoti Bajpai
2   Department of Medical Oncology, Tata Memorial Centre, Mumbai, Maharashtra, India
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Introduction

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.



Publication History

Article published online:
03 December 2022

© 2022. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution License, permitting unrestricted use, distribution, and reproduction so long as the original work is properly cited. (https://creativecommons.org/licenses/by/4.0/)

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