Ashwin Borade, Sandesh Runwal*, Sandeep Karmarkar, Murargi Ghadage**
Department of Pediatrics*, ENT.**, Grant Medical Foundation, Ruby Hall Clinic, Pune, Maharashtra.

Address for correspondence: Dr.Ashwin Borade, PICU. Ruby Hall Clinic, Pune, Maharashtra. E- mail- ashwinborade@yahoo.com

Abstract: Hemangiopericytoma (HP) is a rare vascular tumor that can be benign or malignant, derived from perivascular modified smooth muscle cells (pericytes) (1). It comprises only one percent of all vascular neoplasms and is a very rare tumor in children. This case illustrates the need to recognize this entity as well as manage it effectively.

Key words:hemangiopericytoma, paranasal sinuses

HPs are uncommon vascular tumors, rarely located in the nasal cavity and paranasal sinuses. Very few cases have been reported in children. We herewith report a case of endonasal hemangiopericytoma in a 12 years old male child who presented with proptosis and epistaxis.

A 12 years old male child previously asymptomatic presented with gradual left eye proptosis (Fig .1), nasal obstruction, bilaterally diminished vision and epistaxis over period of 4 months. There was no history of vomiting, seizure, skin lesion or any neurodeficit. On examination vitals were stable and proptosis was present. Other systems were normal. CT scan paranasal sinuses and MRI Brain (Fig 2a,2b,2c) revealed a vascular endonasal lesion involving ethmoid cell, sphenoid sinus, nasal cavity with extension into maxillary sinuses bilaterally and left orbit causing destruction of skull base with intracranial extension. Child underwent lateral rhinotomy with near total maxillectomy with frontosphenoethmoidectomy with excision of mass. Frozen section was suggestive of hemangiopericytoma confirmed by histopathology and immunohistochemistry.

Hemangiopericytoma (glomangiopericytoma) was first described by Stout and Murray in 1942 (2). Hemangiopericytoma is a soft-tissue neoplasm most commonly seen in adults; only 5-10% of cases occur in children. In children HP has very benign course, as compared to adults. Most often, HP arise in the musculoskeletal system, the skin of the limbs, trunk and from the retroperitoneal area. They usually develop in the soft tissues of scalp, face or neck (3). The tumor is twice as common in the nasal cavity as in the paranasal sinuses. The sphenoid and ethmoid sinuses are involved four times more often than the maxillary sinuses. Most common symptom is nasal obstruction and epistaxis along with a wide array of other nonspecific findings. They may occur as slowly growing enlarged painless mass (4). Histologically, these tumors are submucosal, consist of numerous vascular channels with plump endothelial nuclei and surrounding packed proliferation of oval and spindle cells with dark nuclei with moderate amount of cytoplasm. The vascular channels range from capillary size to large patulous spaces that may have a ramifying “staghorn” or “antler-like” configuration (1). The neoplastic cells form a closely packed syncytium of uniform, monotonus, oval to slightly spindle-shaped cells with indistinct cell borders that contain vesicular to hyperchromatic, round to oval to spindle shaped nuclei. The lesional cells are immunoreactive with vimentin, Factor 12 antigen, HLA-DR antigen but not with CD34, CD31, or FVIII-R Ag (5). The differential diagnoses are histiocytoma, plasmacytoma, vascular leiomyoma, angiofibroma.

The treatment of hemangiopericytoma is dependent on amount of cellular dysplasia and mitotic activity. The most bland lesion with minimal mitotic activity is treated by wide local excision, but more the active and the dysplastic lesion are treated by radical surgery with or without adjunctive radiotherapy.

HP is rare in children but we need to effectively diagnose this entity and treat it effectively.

Dr.K.B.Grant ,Founder of Grant Medical Foundation for guidance. Dr.Ashish Atre, Dr.Karnik for Involvement in patient management.