Hemangiopericitoma de órbita

Abstract

Hemangiopericytomas are rare tumors arising from the proliferation of pericytes. They may be found in the lungs, bones, skull, deep soft tissue or limbs. The tumor has an unpredictable prognosis and when it affects the orbital region, may have an aggressive behavior, with high incidence of recurrence. We report a case of orbital hemangiopericytoma and highlight clinical, surgical, and histopathological features of these tumors. Orbital hemangiopericytomas usually are solid, slow-growing tumors. They should be considered in the differential diagnosis of well-defined orbital masses along with epidermoid cysts, schwannomas, neurofibromas, fibrous histiocytomas, lipomas, and vascular malformations. The diagnosis is confirmed by anatomopathologic examination and sometimes complemented by immunohistochemistry. Complete excision of the tumor with wide margins is usually curative; however, radiotherapy and chemotherapy may be required for recurrent lesions.Keywords: Hemangiopericytoma. Neoplasms, vascular tissue. Orbit/surgery. RESUMOHemangiopericitomas são tumores raros originados a partir da proliferação de pericitos, ou seja, células que envolvem os capilares. São encontrados em ossos, pulmões, crânio, partes moles profundas ou membros inferiores, principalmente na coxa. É considerado um tumor com potencial de malignidade incerto e quando afeta a região orbitária pode apresentar um comportamento biológico agressivo, com grande chance de recidiva. O ob jetivo deste trabalho é relatar um caso de hemangiopericitoma orbital e destacar suas características clínicas, cirúrgicas e histopatológicas. Usualmente, os hemangiopericitomas da órbita são tumores sólidos, únicos e de crescimento lento. Devem ser lembra dos no diagnóstico diferencial dos tumores orbitários bem delimitados, como cistos epidermoides, schwannomas, neurofibromas, fibro-histiocitomas, lipomas e malformações vas culares. A confirmação diagnóstica é realizada pelo exame anatomopatológico e, por vezes, complementada pelo estudo imuno-histoquímico. O tratamento deve ser realizado com exérese completa do tumor, com margens amplas, sendo a radioterapia e a quimioterapia reservadas para casos de lesões reincidentes.Descritores: Hemangiopericitoma. Neoplasias de tecido vascular. Órbita/cirurgia.

Hemangiopericytoma: Conducts and perioperative management of an extent sinonasal tumor in a Jehovah's Witnesses patient ‐ Case report

“…4 , 6 , 9 , 12 In the immunohistochemistry study, vimentin and CD34 are considered as markers reliably detected in SNHPC tumor cells. 6 , 9 , 14 …”

“…Chemoradiotherapy as an exclusive treatment has limited efficacy and should be reserved as a palliative treatment for cases whose surgical resection presents a very high risk of death or in cases of advanced metastases. 6 , 14 Although there are several articles that used adjuvant chemotherapy or chemoradiotherapy applied in the treatment of head and neck HPC, the limited number of cases and follow‐up prevents a direct comparison of these results with other forms of treatment instituted. 5 …”

“…Distant metastasis of head and neck HPC is infrequent, appears late and can, with some frequency, affect the brain, lung, mediastinum, chest wall, bones, abdomen and liver; a rare case of thyroid metastasis is reported in the literature. 1 , 5 , 6 , 14 …”

“…Before the routine use of such exams, the diagnosis was commonly confused with other types of tumors, due to the similar vascularization that these diseases present, which are the blood vessels in the shape of “deer's antler” 4,6,9,12 . In the immunohistochemistry study, vimentin and CD34 are considered as markers reliably detected in SNHPC tumor cells 6,9,14 …”