Glomangiopericytoma of the Nasal Cavity with CTNNB1 p.S37C Mutation

A Case Report and Literature Review

Michihisa Kono, Nobuyuki Bandoh, Ryosuke Matsuoka, Takashi Goto, Toshiaki Akahane, Yasutaka Kato, Hiroshi Nakano, Tomomi Yamaguchi, Yasuaki Harabuchi, Hiroshi Nishihara

Research output: Contribution to journalArticle

Abstract

Glomangiopericytoma (GPC) is a rare mesenchymal tumor arising from the nasal cavity or paranasal sinuses. GPC was categorized as a borderline and low-malignant-potential tumor by the World Health Organization in 2005 and accounts for less than 0.5% of all sinonasal tumors. We report a case of GPC in a 74-year-old woman with a history of recurrent epistaxis and nasal obstruction. A reddish tumor was seen in the right nasal cavity. Enhanced computed tomography showed a mass lesion occupying the right nasal cavity. The tumor, which originated from the nasal septum in the olfactory fissure area, was resected with 5-mm mucosal margins by endoscopic sinus surgery. Histologic examination revealed a uniform proliferation of oval-to-short spindle-shaped cells beneath the epithelium. Immunohistologic analysis demonstrated the tumor cells were positive for α-smooth muscle actin, β-catenin and Vimentin, and negative for AE1/AE3, Bcl-2, CD34, CD117, Factor VIIIR Ag, S-100 protein, or STAT6. The percentage of Ki-67-positive cells was approximately 5%. Genetic analysis using next-generation sequencing revealed a missense mutation in the CTNNB1 gene (c.110C > G, p.S37C). While other CTNNB1 mutations have been described in GPC; this is the first report of this specific mutation. The mutation was confirmed using Sanger sequencing.

Original languageEnglish
JournalHead and Neck Pathology
DOIs
Publication statusAccepted/In press - 2018 Jan 1

Fingerprint

Nasal Cavity
Mutation
Neoplasms
Paranasal Sinuses
Nasal Septum
Catenins
Nasal Obstruction
Epistaxis
S100 Proteins
von Willebrand Factor
Vimentin
Missense Mutation
Smooth Muscle
Actins
Epithelium
Tomography
Genes

Keywords

  • CTNNB1
  • Endoscopic sinus surgery (ESS)
  • Glomangiopericytoma (GPC)
  • Next-generation sequencing (NGS)

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Otorhinolaryngology
  • Oncology

Cite this

Glomangiopericytoma of the Nasal Cavity with CTNNB1 p.S37C Mutation : A Case Report and Literature Review. / Kono, Michihisa; Bandoh, Nobuyuki; Matsuoka, Ryosuke; Goto, Takashi; Akahane, Toshiaki; Kato, Yasutaka; Nakano, Hiroshi; Yamaguchi, Tomomi; Harabuchi, Yasuaki; Nishihara, Hiroshi.

In: Head and Neck Pathology, 01.01.2018.

Research output: Contribution to journalArticle

Kono, Michihisa ; Bandoh, Nobuyuki ; Matsuoka, Ryosuke ; Goto, Takashi ; Akahane, Toshiaki ; Kato, Yasutaka ; Nakano, Hiroshi ; Yamaguchi, Tomomi ; Harabuchi, Yasuaki ; Nishihara, Hiroshi. / Glomangiopericytoma of the Nasal Cavity with CTNNB1 p.S37C Mutation : A Case Report and Literature Review. In: Head and Neck Pathology. 2018.
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