Sinonasal Glomangiopericytoma with Skull Base Involvement: A Diagnostic Dilemma

Author:

Arnous Nouran Khalid1ORCID,Alkholaiwi Feras2ORCID,Alrasheed Abdulaziz S.3

Affiliation:

1. College of Medicine, King Saud University, Riyadh, Saudi Arabia

2. Department of Otorhinolaryngology-Head and Neck Surgery, College of Medicine, Imam Mohammad Ibn Saud Islamic University, Riyadh, Saudi Arabia

3. Department of Otolaryngology-Head and Neck Surgery, College of Medicine, King Saud University, Riyadh, Saudi Arabia

Abstract

Introduction: Glomangiopericytoma (GPC) is a rare type of neoplasm with hemangiopericytoma-like vasculature and perivascular hyalinization of capillary-sized veins. CD34 and S100 protein staining might be positive in a small percentage of GPC. Solitary fibrous tumors (SFTs) present clinically like GPC. However, challenges remain when differentiating GPC from SFT. Case Presentation: A 37-year-old male, smoker, presented with 3 years history of right-sided epistaxis and nasal congestion. He was also complaining of hyposmia but no headaches or visual complaints. On nasal endoscopy, he was found to have a right-sided nasal mass occupying the ethmoid cavity. Computed tomography showed a right sinonasal mass abutting the anterior skull base and magnetic resonance imaging demonstrated a hyperintense, enhancing mass within the right ethmoid cavity, superior septum, and anterior skull base. The patient underwent endoscopic resection with gross total resection and skull base reconstruction. Postoperative pathology initially was thought to be an SFT; however, subsequent STAT6 expression was negative, and therefore GPC diagnosis was confirmed. At the most recent follow-up (6 months), the patient remained free of local disease. Discussion: SFT, unlike GPC, stains strongly for CD34, in this case, due to strong CD34, the diagnosis of SFT was initially made. Nuclear STAT6 expression is highly specific and sensitive for SFT. This later returned as negative; therefore, GPC was confirmed. Conclusion: We present a case of sinonasal GPC with skull base involvement that was treated with endoscopic resection. At the most recent follow-up (6 months), the patient remained free of local disease.

Publisher

SAGE Publications

Subject

Otorhinolaryngology

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