Practica Oto-Rhino-Laryngologica
Online ISSN : 1884-4545
Print ISSN : 0032-6313
ISSN-L : 0032-6313
Original articles
A Case of Glomangiopericytoma with a CTNNB1 Mutation
Shinpei AraiShin ItoTakashi AnzaiHiroko OnagiSho TsuyamaYuki FukumuraTsuyoshi SaitoKatsuhisa Ikeda
Author information
JOURNAL RESTRICTED ACCESS

2021 Volume 114 Issue 3 Pages 195-201

Details
Abstract

Glomangiopericytoma (GPC) is a rare sinonasal tumor. GPC is categorized as a low-grade neoplasm with borderline malignancy and shows a tendency for local recurrence. GPC is histologically characterized by perivascular proliferation of tumor cells and needs to be distinguished from other perivascular neoplasms, such as solitary fibrous tumor and glomus tumor. In addition to general pathological examinations, we performed genetic testing in this case.

We report a case of GPC in a 73-year-old female patient, that was accidentally diagnosed by MRI of the temple region. A reddish smooth tumor was observed in the right nasal cavity. Biopsy revealed a possible perivascular tumor, such as GPC. Thus, we performed complete resection by endoscopic surgery after embolization. The tumor cells showed positive immunohistochemical staining for smooth muscle actin and vimentin, but negative staining for STAT6, CD34 and bcl-2. The tumor cells also showed nuclear accumulation of beta-catenin. In addition, genetic testing revealed a CTNNB1 mutation (p.S37C).

There was no cytologic atypia or mitoses, and no necrosis was observed. A good prognosis can be expected in this case, because the patient could be successfully treated by endoscopic resection and no markers of aggressive behavior were observed. We propose to conduct genetic testing in cases of GPC in the future.

Content from these authors
© 2021 The Society of Practical Otolaryngology
Previous article Next article
feedback
Top