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Primary Alveolar Soft Part Sarcoma of Cheek: Report of a Case and Review of the Literature

Authors :
Eiichi Morii
Satoru Toyosawa
Katsutoshi Hirose
Shumei Murakami
Toshihiro Uchihashi
Yuri Iwamoto
Kohei Naniwa
Yumiko Hori
Saori Yamada
Susumu Tanaka
Masaharu Kohara
Yu Usami
Yasuo Fukuda
Kaori Oya
Source :
Head Neck Pathol
Publication Year :
2021
Publisher :
Springer Science and Business Media LLC, 2021.

Abstract

Alveolar soft part sarcoma (ASPS) is a rare soft tissue sarcoma characterized by an alveolar or organoid arrangement of polygonal tumour cells separated by fibrovascular septa. A specific fusion gene [ASPS critical region 1 (ASPSCR1)—TFE3] was detected in ASPS. Despite being a slow-growing tumour without pain and dysfunction, ASPS is characterized by early metastasis, which leads to poor prognosis. Herein, we report a rare case of primary ASPS of the cheek harbouring ASPSCR1 (exon 7)—TFE3 (exon 5) fusion gene in a 21 year-old woman. This tumour was a well-circumscribed, smooth, round mass that was clinically suspected as a benign tumour. However, histologically, it was observed that the polygonal tumour cells were arranged in solid and alveolar growth patterns. Post-operative examination of the whole body excluded the possibility of metastasis at other sites. Thus, careful immunohistochemical and genetic analyses, as well as whole-body examination, demonstrated that the tumour was a primary ASPS of the cheek.

Details

ISSN :
19360568
Volume :
15
Database :
OpenAIRE
Journal :
Head and Neck Pathology
Accession number :
edsair.doi.dedup.....930349327256d6de2532e70ccb5bdc00
Full Text :
https://doi.org/10.1007/s12105-021-01324-6