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Glomangiopericytoma with CTNNB1 mutation
  1. Shinji Iwata1,
  2. Riko Kitazawa2,
  3. Sohei Kitazawa2 and
  4. Naohito Hato3
  1. 1Otolaryngology, Ehime Prefectural Niihama Hospital, Niihama, Japan
  2. 2Molecular Pathology, Ehime University Hospital, Toon, Japan
  3. 3Otolaryngology, Ehime University Hospital, Toon, Japan
  1. Correspondence to Dr Shinji Iwata; h447033x{at}


Glomangiopericytoma (GPC) is a rare tumour, accounting <0.5% of all nasal cavity tumours. It is classified as borderline malignant to indolent and is associated with perivascular myeloid cells. We report a case of GPC in a woman in her 80s who presented with nasal obstruction. The tumour originated from the nasal septum and was resected via endoscopic sinus surgery. Histopathological examination revealed dense spindle-shaped to oval-shaped mesenchymal cells forming tumour nodules with staghorn-like vessel formation. Immunohistochemical analysis revealed that the tumour cells were positive for α-smooth muscle actin, CD34, β-catenin and cyclin D1, whereas they were negative for STAT6. The percentage of Ki-67-positive cells was approximately 2%. Recent studies have demonstrated a high frequency of β-catenin (CTNNB1) mutations in GPC. We report a case of GPC where identifying CTNNB1 mutations (c.94G>C, p.D32H) was crucial for establishing a definitive diagnosis.

  • ear, nose and throat/otolaryngology
  • pathology
  • head and neck cancer

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  • Contributors The following authors were responsible for drafting of the text, sourcing and editing of clinical images, investigation results, drawing original diagrams and algorithms, and critical revision for important intellectual content: SI, RK, SK and NH. The following authors gave final approval of the manuscript: SI, RK, SK and NH.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.