Hemangiopericitoma maligno de cavidad oral

  1. Enrique Coscarón Blanco
  2. Inmaculada Ursúa Sarmiento
Journal:
Revista ORL

ISSN: 2444-7986,  2444-7986

Year of publication: 2015

Volume: 6

Pages: 125-131

Type: Article

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Abstract

Hemangyopericytoma are unfrequently malignant vascular tumours arising from Zimmerman´s pericytes (cells with mechanical support and vascular regulation rol). They account for about 1% of the tumours as a whole. Only about 20% arise in head and neck areas, mainly in the neck, sinonasal tract and skin. They are uncommonly encountered in the oral cavity or the pharynx. Their etiology reamains unclear. Case description: An unusual case report of an extending-to-canine-fossa malignant oral cavity hemangiopericytoma arising in the gingivolabial groove is provided. Neither its behaviour nor its anatomical origin are aknowledged to be the normal pattern of this disease. Discussion: Due to their unfrequency their differential diagnosis, mainly with other cystic tumours or conditions proper to this site, is not always available prior to surgical excision, which is the key for treatment. The presence of peritumoral tissue adherence and a bloody tumoral bedsite should lead to suspect this tumour. Their histological pattern of dendritic or staghorn appearance is very characteristic, although diagnosis relies in immunochemistry nowadays, with its characteristic intense positivity for vimentin and mild positivity for CD34.Surgery is key for treatment but should be complemented with chemotheraphy and/or radiotheraphy in high-grade tumours or when excision is not feasible or completely curative. Conclusions: The paucity of case reports by particular site and proliferative degree makes difficult to draw definitive conclusions on the management and treatment of this rare tumour difficult to suspect prior to surgery.

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