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Endonasal Hemangiopericytoma in Child

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Endonasal Hemangiopericytoma in Child

Ashwin Borade1, Sandesh Runwal2, Sandeep Karmarkar1, Murargi Ghadage3.
1Grant Medical Foundation, Ruby Hall Clinic, Pune, Maharashtra, 2Department of Pediatrics, Grant Medical Foundation, Ruby Hall Clinic, Pune, Maharashtra, 3Department of ENT, Grant Medical Foundation, Ruby Hall Clinic, Pune, Maharashtra.
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Borade A, Runwal S, Karmarkar S, Ghadage M. Endonasal Hemangiopericytoma in Child. Pediatr Oncall J. 2007;4: 48-49.

Address for Correspondence
Dr.Ashwin Borade, PICU. Ruby Hall Clinic, Pune, Maharashtra.
 
Email
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.
 
Keywords
hemangiopericytoma, paranasal sinuses
 
Introduction
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.
 
Case Report
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.

Fig 1. Proptosis of left eye


Fig 2a,b,c. Endonasal lesion involving ethmoid cell, sphenoid sinus, nasal cavity with extension into maxillary sinuses bilaterally and left orbit.




 
Discussion
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, monotonous, 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.
 
Conclusion
HP is rare in children but we need to effectively diagnose this entity and treat it effectively.
 
Acknowledgement
Dr.K.B.Grant ,Founder of Grant Medical Foundation for guidance. Dr.Ashish Atre, Dr.Karnik for Involvement in patient management.
 
Funding
None
 
Conflict of Interest
None
 
References :
  1. Mathur N, Gupta AK, Gupta A, Kohli P, Bansal S, Kakkar N. Sinonasal Hemangiopericytoma in Adolescents: Histopathologic And Surgical Dilemma. The Internet J Otorhinolaryng. 2006.4:2.
  2. Stout AP, Murray MR. Hemangiopericytoma: vascular tumor featuring Zimmerman's pericytes. Ann of Surgery 1942; 116:26-33.  [CrossRef]
  3. Guerri er Y, Roustan J, Guerrier B, Merlier C. Hemangeopericytoma en ORL. Les Cahiers d'ORL 1978; 13:41-9.
  4. Schoen FJ, Ranzi SC. In Blood vessel. Robbins Pathology of Basic Diseases, Cotran, Kumar Collins. 6 th Ed. India: WB Saunders;2000:538.
  5. Thompson LD, Miettinen M, Wenig BM. Sinonasal- type hemangeopericytoma: A clinicopathologic and immunophenotypic analysis of 104 cases showing perivascular myoid differentiation. Am J Surg Pathol 2003; 27: 737-4.  [CrossRef]  [PubMed]

Last Updated : 01 September 2007 Vol 4 Issue 9 Art #37

Cite this article as: :
Borade A, Runwal S, Karmarkar S, Ghadage M. Endonasal Hemangiopericytoma in Child. Pediatr Oncall J. 2007;4: 48-49.
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