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Case Reports
. 2004 Sep 2;122(5):223-6.
doi: 10.1590/s1516-31802004000500010. Epub 2004 Nov 10.

Head and neck hemangiopericytoma in a child: case report

Jomar Rezende Carvalho  1 , Leonardo Haddad, Fernando Danelon Leonhardt, Marcílio Ferreira Marques Filho, Rodrigo de Oliveira Santos, Onivaldo Cervantes, Márcio Abrahão
Affiliations
Case Reports

Head and neck hemangiopericytoma in a child: case report

Jomar Rezende Carvalho et al. Sao Paulo Med J. .

Abstract
in English, Portuguese

Context: Hemangiopericytoma is a relatively rare tumor, first described in 1942, with approximately 300 cases described in the literature to date. In most cases, it affects the trunk and lower extremities. The head and neck incidence is less than 20%, mostly in adults. We describe a case of malignant head and neck hemangiopericytoma in a child.

Type of study: Case report.

Case report: A twelve-year-old male patient noted the presence of a firm painless right-side retroauricular lymph node of 1 cm in diameter, which at first remained unchanged for six months, but subsequently enlarged progressively. He denied having had previous trauma at that site. In November 2000, he presented nasal obstruction and voluminous epistaxis that required hospitalization and blood transfusion. During dental treatment one month later, a cranial x-ray revealed bone alterations. A subsequent computed tomography scan showed an extensive lesion of soft tissue density that had invaded the maxillary fossa, eroding the skull base and middle and nasal fossa. The child was then referred to our service, where biopsy was performed, giving a diagnosis of hemangiopericytoma. Shortly afterwards, magnetic resonance imaging revealed that this lesion had undergone significant growth, while maintaining the same invasion pattern. The patient was submitted to conservative surgery in April 2001, with only partial resection of the tumor because of its extent. Histopathological examination of the specimen confirmed the presence of malignant hemangiopericytoma. Following the surgery, the patient presented fast regrowth of the lesion, with partial response to chemotherapy and radiotherapy.

CONTEXTO:: O hemangiopericitoma é uma neoplasia relativamente, descrita pela primeira vez em 1942, e com aproximadamente 300 casos descritos até hoje na literatura. Na maioria dos pacientes, acomete o tronco e membros inferiores, sendo a incidência em cabeça e pescoço menor que 20%, e principalmente em adultos. Relatamos um caso de hemangiopericitoma de cabeça e pescoço em criança.

TIPO DE ESTUDO:: Relato de caso.

RELATO DO CASO:: Paciente do sexo masculino, 12 anos, que, em dezembro de 1999, notou nódulo retroauricular à direita, endurecido, indolor e de 1 cm de diâmetro, que evoluiu estável por seis meses e com posterior crescimento progressivo. Negava história de trauma. Em novembro de 2000, apresentou obstrução nasal e epistaxe volumosa, necessitando de internação e transfusão sangüínea. Um mês depois, durante tratamento dentário, uma radiografia craniana evidenciou alterações ósseas. Tomografia computadorizada subseqüente mostrou lesão extensa característica de partes moles, invadindo fossa maxilares, destruindo base do crânio, fossa média e fossas nasais. A criança foi encaminhada ao nosso serviço, onde biópsia foi realizada, com diagnóstico de hemangiopericitoma. Logo após exame de ressonância nuclear magnética evidenciou a mesma lesão com aumento importante, mas invadindo as mesmas estruturas. Submetido à cirurgia conservadora em abril de 2001 com ressecção parcial do tumor devido à sua extensão. O exame histopatológico revelou hemangiopericitoma maligno. Após a cirurgia, o paciente apresentou rápido crescimento da massa tumoral e resposta parcial a quimioterapia e radioterapia.

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Conflict of interest statement

Conflict of interest: Not declared

Figures

Figure 1
Figure 1. Computed tomography scan of a twelve-year-old patient, showing lesion of soft tissue density invading maxillary fossa, eroding the skull and middle and nasal fossa.
Figure 2
Figure 2. Magnectic resonance imaging of a twelve-year-old patient, showing solid mass with isodense contrast in T1.
Figure 3
Figure 3. Angiography of a twelve-year-old patient.
See this image and copyright information in PMC

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