RESUMO
A patient was referred to the department for an incidentally discovered right orbital mass on MRI. After surgical excision, histopathologic examination led to the diagnosis of epithelioid hemangioma, a tumor rarely located in the orbit, whose main histopathologic feature is an exuberant proliferation of small vascular channels lined by enlarged endothelial cells. The clinical characteristics and histopathology of epithelioid hemangioma allow differentiation with Kimura disease, angiosarcoma, intravascular papillary endothelial hyperplasia, and other vascular tumors that share the common feature of an abnormal proliferation of endothelial cells. Although epithelioid hemangioma is extremely rare, it should be considered in the differential diagnosis of orbital tumors.
Assuntos
Hiperplasia Angiolinfoide com Eosinofilia/diagnóstico , Doenças Orbitárias/diagnóstico , Idoso , Hiperplasia Angiolinfoide com Eosinofilia/cirurgia , Feminino , Humanos , Imageamento por Ressonância Magnética , Doenças Orbitárias/cirurgia , Acuidade VisualRESUMO
INTRODUCTION: To report a case of idiopathic orbital sclerosing inflammation (ISOI) with intranasal extension. MATERIAL AND METHODS: The patient presented with a 6-month history of epiphora, upper eyelid swelling, ptosis and mild orbital pain. Ophthalmologic examination, CT, MRI and biopsy with surgical debulking were performed. RESULTS: MRI revealed a homogeneously enhancing diffuse right orbital mass in the inferonasal quadrant of the orbit, which extended to the nasal cavity up to inferior nasal concha, maxillary and ethmoid sinuses. Histological analysis showed dense collagenous tissue with sparse infiltration of mixed inflammatory cells. Inmunohistochemical analysis confirmed polyclonality. The diagnosis of idiopathic sclerosing orbital inflammation was made and 80 mg/day of oral prednisolone was prescribed. At last follow up, one year later, there was no clinical evidence of recurrent orbital disease. CONCLUSION: ISOI can present with extraorbital extension. Corticosteroids are a reasonable first-line treatment, until the pathogenesis is better understood.