Objective: To describe the histopathologic features of two cases of epithel
ioid hemangioma occurring in the orbit and to distinguish this condition fr
om Kimura's disease and from other vascular lesions of proliferated endothe
lium.
Design: Two interventional case reports.
Intervention: Treatment consisted of orbitotomy with excision of the tumor.
Main Outcome Measures: Histopathologic examination including light microsco
py, immunohistochemistry, and electron microscopy and clinical follow-up.
Results: In one case, there was no local recurrence after 2 years of follow
-up, In the other case, local recurrence required re-excision 2 years after
surgery with no apparent recurrence 16 years later. Histopathologic examin
ation of both tumors disclosed an epithelioid hemangioma. A characteristic
finding was the presence of peculiar plump vacuolated endothelial cells lin
ing the vascular lumina.
Conclusions: Epithelioid hemangioma is an uncommon benign vascular tumor th
at can occur in the orbit, and surgical excision is usually required. it is
the same condition as angiolymphoid hyperplasia with eosinophilia. There a
re distinct clinical and histopathologic characteristics to distinguish epi
thelioid hemangioma from Kimura's disease and from other vascular tumors. (
C) 2000 by the American Academy of Ophthalmology.