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

被引:3
作者
Hirose, Katsutoshi [1 ]
Naniwa, Kohei [1 ]
Usami, Yu [1 ]
Kohara, Masaharu [2 ]
Uchihashi, Toshihiro [3 ,4 ]
Tanaka, Susumu [3 ]
Yamada, Saori [3 ]
Iwamoto, Yuri [5 ]
Murakami, Shumei [5 ]
Oya, Kaori [6 ]
Fukuda, Yasuo [6 ]
Hori, Yumiko [2 ]
Morii, Eiichi [2 ]
Toyosawa, Satoru [1 ]
机构
[1] Osaka Univ, Dept Oral Pathol, Grad Sch Dent, 1-8 Yamadaoka, Suita, Osaka 5650871, Japan
[2] Osaka Univ, Dept Pathol, Grad Sch Med, 2-2 Yamadaoka, Suita, Osaka 5650871, Japan
[3] Osaka Univ, Dept Oral & Maxillofacial Surg 1, Grad Sch Dent, 1-8 Yamadaoka, Suita, Osaka 5650871, Japan
[4] Osaka Univ Hosp, Unit Dent, 2-15 Yamadaoka, Suita, Osaka 5650871, Japan
[5] Osaka Univ, Dept Oral & Maxillofacial Radiol, Grad Sch Dent, 1-8 Yamadaoka, Suita, Osaka 5650871, Japan
[6] Osaka Univ, Clin Lab, Dent Hosp, 1-8 Yamadaoka, Suita, Osaka 5650871, Japan
关键词
Alveolar soft part sarcoma; ASPS; Cheek tumour; ASPSCR1; TFE3;
D O I
10.1007/s12105-021-01324-6
中图分类号
R36 [病理学];
学科分类号
100104 ;
摘要
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.
引用
收藏
页码:1426 / 1431
页数:6
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