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 |
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Rok vydání: | 2021 |
Předmět: |
0301 basic medicine
Pathology medicine.medical_specialty Contrast Media TFE3 Case Reports Pathology and Forensic Medicine Metastasis Diagnosis Differential Young Adult 03 medical and health sciences Exon 0302 clinical medicine Alveolar soft part sarcoma medicine Humans business.industry Soft tissue sarcoma Cheek medicine.disease Magnetic Resonance Imaging Primary Alveolar Soft Part Sarcoma Sarcoma Alveolar Soft Part 030104 developmental biology medicine.anatomical_structure Oncology Otorhinolaryngology Lymphatic Metastasis 030220 oncology & carcinogenesis Immunohistochemistry Female business |
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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