A Rare Presentation of Sinonasal Hemangiopericytoma-like Tumour in Frontoethmoidal Sinus: A Diagnostic Challenge

Abstract

Sinonasal hemangiopericytoma-like tumour (SHPCL) are rare vascular neoplasms derived from Zimmerman’s capillary pericytes. They originate in a paranasal sinus and extend into the nasal cavity secondarily. Hemangiopericytomas of soft tissue usually occur in the retroperitoneum or the thigh and are an uncommon finding in the nasal and paranasal sinuses. They occur most commonly in adults in the sixth and seventh decades of life and clinically mimic allergic polyps. These patients most commonly present with symptoms of epistaxis and nasal obstruction. Microscopically, these tumours demonstrate a vascular architecture, are composed predominantly of spindle cells, and lack nuclear or cytoplasmic pleomorphism, mitotic activity, haemorrhage or necrosis. These criteria include the presence or absence of mitotic figures, necrosis, anaplasia, and haemorrhage. The present case with early onset in fourth decade with predominantly orbital symptoms, minimal findings on nasal endoscopy and biopsy gives a diagnostic challenge. Treated with complete surgical excision and diagnosis was confirmed by immunohistochemistry reports as a rare SHPCL. Being a locally invasive tumour with very less propensity for metastasis or recurrence, two year of recurrence free endoscopic follow up is sufficiently justified it to be cured.