Hemangiopericytoma of the maxilla in a pediatric patient: A case report

Department of Pedodontics, Sardar Patel Post Graduate Institute of Dental Sciences, Lucknow (UP), India.
Journal of dentistry for children (Chicago, Ill.) 09/2010; 77(3):180-2.
Source: PubMed


The Hemangiopericytoma is a malignant vascular tumor arising from mesenchymal cells with pericytic differentiation. Hemangiopericytoma is most commonly seen in adults, and only 5% to 10% of cases occur in children. The tumor is extremely rare in the head and neck region (16%)1. Cytogenic abnormalities have been present in some hemangiopericytoma cases. Surgical resection remains the mainstay treatment. Adjuvant chemotherapy and radiotherapy is appropriate for cases of incomplete resections and life-threatening tumors particularly in children. Late relapses may occur and require long-term follow-up. A 4-year-old child patient with hemangiopericytoma of the maxilla presented with firm, recurrent, but painless jaw mass. Radiographic investigations revealed a poorly circumscribed radiolucency. The lesion biopsy showed well-circumscribed multiple lobules of tumor mass consisting of tightly packed, spindle-shaped cells. Chemotherapy and radiotherapy of the lesion was conducted. The role of the pediatric dental team is extensive in children with hemangiopericytoma, who require a regular dental review. The patient's oncologist should be immediately contacted if there is any suspicion of recurrence.

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    ABSTRACT: Haemangiopericytoma (HPC) is a vascular tumour which originates in the pericytes of vessels and therefore it may occur at any site, but it is very uncommon in the jaw. From January 2000 to December 2011, a retrospective analysis of nine consecutive patients with HPCJ was performed. There were five patients with a primary tumour and four patients with a recurrent tumour. Of the nine patients, eight were male and one female. Their ages ranged from 23 years to 51 years, with a median age of 38 years. The tumours were located in the mandible in six patients and in maxilla in three cases. The median course of disease was 7.6 months (range 2-12 months). All patients underwent surgery. Two patients had postoperative adjuvant radiotherapy, and two cases were given postoperative adjuvant chemotherapy. The median follow-up period was 49 months (10-101 months). One patient suffered from lumbar metastasis, while another case had metastasis at local and multiple distant sites, and eventually died. There was no local recurrence or metastasis in other seven cases. HPCJ are rare and the clinical characteristics are not specific. The first choice of treatment is radical surgery. Adjuvant radiotherapy may be effective to improve the prognosis of HPCJ.
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    ABSTRACT: Hemangiopericytoma (HPC) is a rare tumor of uncertain malignant potential. Stout and Murray described HPC as "vascular tumor arising from Zimmerman's pericyte" in 1942. The World Health Organization (WHO) reclassified HPC as a fibroblastic/myofibroblastic tumor, after further characterization. HPC is found mostly wherever there is increased vascularity seen. The incidence of the tumor in head and neck area is only 15%, mostly seen in adults. We report here a case of HPC of a 22-year-old female, who presented to our department with a tender swelling in maxillary anterior region and the mass was well-circumscribed, sessile and soft on palpation. The skin over the tumor was intact and normal. The tumor was completely removed with wide surgical resection. The histopathological staining supported the diagnosis of HPC, this was further confirmed by immunohistochemistry (IHC) in which CD99 showed strong positivity.
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