Advances in treatment of alveolar soft part sarcoma: an updated review.
alveolar soft part sarcoma
chemotherapy
immunotherapy
surgery
targeted therapy
Journal
Japanese journal of clinical oncology
ISSN: 1465-3621
Titre abrégé: Jpn J Clin Oncol
Pays: England
ID NLM: 0313225
Informations de publication
Date de publication:
05 Nov 2023
05 Nov 2023
Historique:
received:
02
06
2023
accepted:
31
07
2023
medline:
10
11
2023
pubmed:
26
8
2023
entrez:
26
8
2023
Statut:
ppublish
Résumé
Alveolar soft part sarcoma is a rare neoplasm of uncertain histogenesis that belongs to a newly defined category of ultra-rare sarcomas. The neoplasm is characterized by a specific chromosomal translocation, der (17) t(X; 17)(p11.2;q25), that results in ASPSCR1-TFE3 gene fusion. The natural history of alveolar soft part sarcoma describes indolent behaviour with slow progression in deep soft tissues of the extremities, trunk and head/neck in adolescents and young adults. A high rate of detection of distant metastasis at presentation has been reported, and the most common metastatic sites in decreasing order of frequency are the lung, bone and brain. Complete surgical resection remains the standard treatment strategy, whereas radiotherapy is indicated for patients with inadequate surgical margins or unresectable tumours. Although alveolar soft part sarcoma is refractory to conventional doxorubicin-based chemotherapy, monotherapy or combination therapy using tyrosine kinase inhibitors and immune checkpoint inhibitors have provided antitumor activity and emerged as new treatment strategies. This article provides an overview of the current understanding of this ultra-rare sarcoma and recent advancements in treatments according to the clinical stage of alveolar soft part sarcoma.
Identifiants
pubmed: 37626447
pii: 7251339
doi: 10.1093/jjco/hyad102
pmc: PMC10632598
doi:
Substances chimiques
Oncogene Proteins, Fusion
0
Types de publication
Review
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
1009-1018Subventions
Organisme : Japan Society for the Promotion of Science
ID : 21K16709
Informations de copyright
© The Author(s) 2023. Published by Oxford University Press.
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