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SURPRISING HEMANGIOMAS

Posted on: Junho 10, 2019 Posted by: admin Comments: 0

SURPRISING HEMANGIOMAS

Joana Roque

Introduction:
Cavernous hemangiomas are the most common orbital neoplams in adults. The diagnosis of these vascular tumors is suspected clinically and usually confirmed with neuroimaging, preferably Magnetic Resonance Imaging (MRI).

Materials and methods:
Report of two clinical cases of suspected orbital hemangiomas and literature review regarding the differencial diagnosis with other entities.

Results:
Case 1: A 29-year-old male with irrelevant clinical history presented with slowly progressive painless proptosis of the left eye. Besides the proptosis, ophthalmological examination was unremarkable, without evidence of visual compromise. Computed Tomography (CT) suggested a cavernous hemangioma of the left orbit. Contrast-enhaced MRI revealed a large, well-delineated intra and extraconical lesion, also suggestive of hemangioma but not excluding a neurofibroma. The mass was ressected and pathology confirmed the neurofibroma hypothesis. In contrast to the plexiform subtype, localized neurofibromas are relatively rare orbital tumors. These well-circunscribed benign masses are associated only in a minority of cases with neurofibromatosis type 1, which was excluded in the patient.
Case 2: A 35 -year-old previously healthy woman presented with an extremely similar condition. Neuroimaging studies showed an extraconical homogenous mass in the of lateral portion of the left orbit, highly suggestive of cavernous hemangioma. Surprisingly, in this case the pathology of the excised lesion revealed an adenoid cystic carcinoma (ACC). These malignant tumors represent a rare subtype of adenocarcinoma. They can affect the orbit, usually in the form of lacrimal gland ACC. This case was remarkable because of the imaging feautures confounding it with a hemangioma and because of the absence of obvious lacrimal gland involvement.

Conclusions:
Despite the typical clinical and imagiological diagnosis of hemangiomas, there must be some suspicion for other orbital lesions. Symptomatic or atypical masses should be surgically excised, whereas small asymptomatic suspected hemangiomas can be followed periodically with ophthalmological evaluation and CT or MRI studies.