Subscribe to RSS
Please copy the URL and add it into your RSS Feed Reader.
https://www.thieme-connect.de/rss/thieme/en/10.1055-s-00050348.xml
Download PDF
CC BY 4.0 · Indian J Med Paediatr Oncol 2024; 45(01): 068-073
DOI: 10.1055/s-0042-1758540
DOI: 10.1055/s-0042-1758540
How I Treat
How I Treat Alveolar Soft Part Sarcoma? The Therapeutic Journey from Nihilism to Cautious Optimism…
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/)
Thieme Medical and Scientific Publishers Pvt. Ltd.
A-12, 2nd Floor, Sector 2, Noida-201301 UP, India
-
References
- 1 Christopherson WM, Foote Jr FW, Stewart FW. Alveolar soft-part sarcomas; structurally characteristic tumors of uncertain histogenesis. Cancer 1952; 5 (01) 100-111
- 2 Reichardt P, Lindner T, Pink D, Thuss-Patience PC, Kretzschmar A, Dörken B. Chemotherapy in alveolar soft part sarcomas. What do we know?. Eur J Cancer 2003; 39 (11) 1511-1516
- 3 McCarville MB, Muzzafar S, Kao SC. et al. Imaging features of alveolar soft-part sarcoma: a report from Children's Oncology Group Study ARST0332. AJR Am J Roentgenol 2014; 203 (06) 1345-1352
- 4 Li X, Ye Z. Magnetic resonance imaging features of alveolar soft part sarcoma: report of 14 cases. World J Surg Oncol 2014; 12: 36
- 5 Cui JF, Chen HS, Hao DP, Liu JH, Hou F, Xu WJ. Magnetic resonance features and characteristic vascular pattern of alveolar soft part sarcoma. Oncol Res Treat 2017; 40 (10) 580-585
- 6 Ladanyi M, Lui MY, Antonescu CR. et al. The der(17)t(X;17)(p11;q25) of human alveolar soft part sarcoma fuses the TFE3 transcription factor gene to ASPL, a novel gene at 17q25. Oncogene 2001; 20 (01) 48-57
- 7 Williams A, Bartle G, Sumathi VP. et al. Detection of ASPL/TFE3 fusion transcripts and the TFE3 antigen in formalin-fixed, paraffin-embedded tissue in a series of 18 cases of alveolar soft part sarcoma: useful diagnostic tools in cases with unusual histological features. Virchows Arch 2011; 458 (03) 291-300
- 8 Wang Y, Cui J, Yan X, Jin R, Hong X. Alveolar soft part sarcoma with multiple brain and lung metastases in pregnancy: a case report and literature review. Medicine (Baltimore) 2017; 96 (46) e8790
- 9 Wang H, Jacobson A, Harmon DC. et al. Prognostic factors in alveolar soft part sarcoma: A SEER analysis. J Surg Oncol 2016; 113 (05) 581-586
- 10 Ogura K, Beppu Y, Chuman H. et al. Alveolar soft part sarcoma: a single-center 26-patient case series and review of the literature. Sarcoma 2012; 2012: 907179
- 11 Stacchiotti S, Negri T, Zaffaroni N. et al. Sunitinib in advanced alveolar soft part sarcoma: evidence of a direct antitumor effect. Ann Oncol 2011; 22 (07) 1682-1690
- 12 Li T, Wang L, Wang H. et al. A retrospective analysis of 14 consecutive Chinese patients with unresectable or metastatic alveolar soft part sarcoma treated with sunitinib. Invest New Drugs 2016; 34 (06) 701-706
- 13 Jagodzińska-Mucha P, Świtaj T, Kozak K. et al. Long-term results of therapy with sunitinib in metastatic alveolar soft part sarcoma. Tumori 2017; 103 (03) 231-235
- 14 Stacchiotti S, Mir O, Le Cesne A. et al. Activity of pazopanib and trabectedin in advanced alveolar soft part sarcoma. Oncologist 2018; 23 (01) 62-70
- 15 Kim M, Kim TM, Keam B. et al. A Phase II trial of pazopanib in patients with metastatic alveolar soft part sarcoma. Oncologist 2019; 24 (01) 20-e29
- 16 Kummar S, Allen D, Monks A. et al. Cediranib for metastatic alveolar soft part sarcoma. J Clin Oncol 2013; 31 (18) 2296-2302
- 17 Judson I, Scurr M, Gardner K. et al. Phase II study of cediranib in patients with advanced gastrointestinal stromal tumors or soft-tissue sarcoma. Clin Cancer Res 2014; 20 (13) 3603-3612
- 18 Judson I, Morden JP, Leahy MG. et al. Activity of cediranib in alveolar soft part sarcoma (ASPS) confirmed by CASPS (cediranib in ASPS), an international, randomized phase II trial [abstract 11004]. Proc ASCO. 2017;35(15).
- 19 Schuetze SM, Bolejack V, Choy E. et al. Phase 2 study of dasatinib in patients with alveolar soft part sarcoma, chondrosarcoma, chordoma, epithelioid sarcoma, or solitary fibrous tumor. Cancer 2017; 123 (01) 90-97
- 20 Schöffski P, Wozniak A, Kasper B. et al. Activity and safety of crizotinib in patients with alveolar soft part sarcoma with rearrangement of TFE3: European Organization for Research and Treatment of Cancer (EORTC) phase II trial 90101 ‘CREATE’. Ann Oncol 2018; 29 (03) 758-765
- 21 Wagner AJ, Goldberg JM, Dubois SG. et al. Tivantinib (ARQ 197), a selective inhibitor of MET, in patients with microphthalmia transcription factor-associated tumors: results of a multicenter phase 2 trial. Cancer 2012; 118 (23) 5894-5902
- 22 Flores RJ, Harrison DJ, Federman NC. et al. Alveolar soft part sarcoma in children and young adults: a report of 69 cases. Pediatr Blood Cancer 2018; 65 (05) e26953
- 23 Simha V, Bajpai J. Alveolar Soft Part Sarcomas(ASPS): A tertiary cancer care center experience from India. Journal of Clinical Oncology 2019; 37 (15) e22553
- 24 Groisberg R, Hong DS, Behrang A. et al. Characteristics and outcomes of patients with advanced sarcoma enrolled in early phase immunotherapy trials. J Immunother Cancer 2017; 5 (01) 100
- 25 Naqash AR, Coyne GHO, Moore N. et al. Phase II study of atezolizumab in advanced alveolar soft part sarcoma (ASPS). Journal of Clinical Oncology 2021; 39 (15) ,suppl. 11519
- 26 Wilky BA, Trucco MM, Subhawong TK. et al. Axitinib plus pembrolizumab in patients with advanced sarcomas including alveolar soft-part sarcoma: a single-centre, single-arm, phase 2 trial. Lancet Oncol 2019; 20 (06) 837-848
- 27 Saerens M, Brusselaers N, Rottey S, Decruyenaere A, Creytens D, Lapeire L. Immune checkpoint inhibitors in treatment of soft-tissue sarcoma: A systematic review and meta-analysis. Eur J Cancer 2021; 152: 165-182
- 28 Lieberman PH, Brennan MF, Kimmel M, Erlandson RA, Garin-Chesa P, Flehinger BY. Alveolar soft-part sarcoma. A clinico-pathologic study of half a century. Cancer 1989; 63 (01) 1-13