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

Autor: 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
Rok vydání: 2021
Předmět:
Zdroj: Head Neck Pathol
ISSN: 1936-0568
DOI: 10.1007/s12105-021-01324-6
Popis: 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.
Databáze: OpenAIRE
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