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Focus: Rare Disease: Sinonasal Glomangiopericytoma: Review of Imaging Appearance and Clinical Management Update for a Rare Sinonasal Neoplasm
Authors:Yaser M. Al-Jobory  Zenggang Pan  R. Peter Manes  Sacit B. Omay  Ichiro Ikuta
Affiliation:aRadiology & Biomedical Imaging, Yale University School of Medicine, New Haven, CT, USA;bDepartment of Pathology, Yale University School of Medicine, New Haven, CT, USA;cDepartment of Surgery, Division of Otolaryngology, Yale University School of Medicine, New Haven, CT, USA;dNeurosurgery, Yale University School of Medicine, New Haven, CT, USA
Abstract:Introduction: Glomangiopericytoma (GPC) is a rare tumor in the nasal cavity or paranasal sinuses with low malignant potential. Initially deemed a hemangiopericytoma, in 2005 it was classified as a distinct entity by the World Health Organization (WHO). Case Presentation: A male patient in his early 60s presented with new-onset right arm and leg weakness/numbness, who was incidentally found to have a left ethmoid sinus mass with extension in the olfactory fossa. On CT and MRI, the mass enhanced with well-defined borders and eroded the bone, but without dural enhancement. The mass was surgically excised, and pathology confirmed the diagnosis of glomangiopericytoma by microscopic appearance and staining. Discussion: Glomangiopericytoma has less than 0.5% incidence of all neoplasms of the sinonasal cavity, making it rare. Most diagnosed patients are in their 6th or 7th decade of age, with a slight female predominance. Treatment is complete surgical excision, with excellent prognosis, although there is up to 17% local recurrence. Despite the non-specific appearance on CT and MRI, imaging can help provide differential diagnosis, tumor extent, size, and reassuring non-aggressive characteristics of the tumor prior to surgery. GPC tumors are relatively resistant to radiation and chemotherapy. Conclusion: It is important to recognize glomangiopericytoma in the differential of masses of the nasal cavities or paranasal sinuses, as they rarely warrant aggressive treatment beyond local excision. Each reported case of glomangiopericytoma helps to build guidance for imaging and treatment since GPC is rare and not well-represented in the medical literature.
Keywords:sinonasal   glomangiopericytoma   neoplasm   skull base   rare
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