Primary intraosseous alveolar soft part sarcoma: Report of two cases with radiologic-pathologic correlation

Pena‐Burgos, Eva Manuela; Pozo‐Kreilinger, José Juan; Tapia-Viñe, Mar; Redondo, Andrés; Mendiola-Sabio, M; Ortiz‐Cruz, Eduardo J.

doi:10.1016/j.anndiagpath.2022.152078

Cited by 2 publications

(1 citation statement)

References 31 publications

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“…ASPS presents asymptomatically as a slow-growing tumour ( 4 , 11 ) and commonly arises in deep soft tissues of the extremities (61%), trunk (20%), head and neck (9%), and internal organs (8%) ( 2 , 10 ). Uncommon sites include bone ( 12 , 13 ), brain ( 14 , 15 ), orbit ( 16 ), tongue ( 17 ), lung ( 18 ), mediastinum ( 19 , 20 ), bladder ( 21 ), prostate ( 22 ), uterus ( 23 ) and vagina ( 24 ). Because of the lack of associated symptoms or functional impairment, patients with ASPS often present with metastatic disease ( 4 ).…”

Section: Clinical Presentationmentioning

confidence: 99%

Advances in treatment of alveolar soft part sarcoma: an updated review

Fujiwara,

Kunisada,

Nakata

et al. 2023

Japanese Journal of Clinical Oncology

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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.

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