A Case of Glomangiopericytoma at the Nasal Septum

被引:11
作者
Anzai T. [1 ]
Saito T. [2 ]
Tsuyama S. [2 ]
Toh M. [1 ]
Ikeda K. [1 ]
Ito S. [1 ]
机构
[1] Department of Otorhinolaryngology Head and Neck Surgery, Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo-ku, Tokyo
[2] Department of Human Pathology, Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo-ku, Tokyo
基金
日本学术振兴会;
关键词
Endoscopic sinus surgery; Glomangiopericytoma; Hemangiopericytoma; Nasal septum; β-Catenin;
D O I
10.1007/s12105-017-0870-6
中图分类号
学科分类号
摘要
Glomangiopericytoma (GPC) is a rare sinonasal perivascular tumor that accounts for < 0.5–1% of all sinonasal tumors. GPC is categorized as a low-grade neoplasm with borderline malignancy and a tendency of local recurrence. GPC is a rare mesenchymal neoplasm characterized by the perivascular proliferation of tumor cells, and it requires being distinguished from solitary fibrous tumors. Here, we report a case of GPC in a 68-year-old male patient who presented at the emergency room of our hospital with a complaint of sudden epistaxis. A small, reddish, protruding tumor was observed on the right nasal septum. A biopsy revealed a possible perivascular tumor such as a GPC or solitary fibrous tumor. Thus, we performed complete resection with endoscopic surgery. The size of the resected tumor was 12 × 5 mm, and it showed a uniform proliferation of oval-to-short spindle-shaped cells with slightly branching vascular structures. The tumor cells showed minimal cytologic atypia and there were an average of 3 mitoses in 10 high power fields. Necrosis was not observed. The tumor cells showed strong and diffuse nuclear immunostaining with beta catenin and were negative with STAT6, CD34 and bcl-2. The MIB-1 labeling index was approximately 5%. Genetic testing revealed CTNNB1 mutation (p.S33C). Thus, a diagnosis of low grade GPC was made on the biopsy and the patient could be successfully treated with endoscopic resection. © 2017, The Author(s).
引用
收藏
页码:572 / 575
页数:3
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