Lyon D B, Tang T T, Kidder T M
Department of Ophthalmology, University of Wisconsin, Madison.
Ophthalmology. 1992 Dec;99(12):1773-8. doi: 10.1016/s0161-6420(92)31723-3.
The authors report a case of an epithelioid hemangioendothelioma arising in the orbital bones. A review of the literature related to this rare orbital neoplasm identified eight well-documented cases, one of which occurred in a patient younger than that reported here, but none of which originated in bone.
A 3 1/2-month-old boy had a left inferior orbital mass that had grown rapidly over a 3-day period. An emergency computed tomography scan showed a large neoplasm with significant bone destruction of the zygoma and maxilla. Initial examination suggested a rhabdomyosarcoma, and a transconjunctival biopsy was performed, which was complicated by significant blood loss. The final pathologic diagnosis was an epithelioid hemangioendothelioma, or grade 2 hemangioendothelioma, of bone origin. No other sites of disease were found on metastatic survey. Subsequent treatment consisted of an en bloc tumor resection sparing the orbital soft tissues and globe.
The patient is free of disease and has normal visual fixation and ocular motility 20 months after surgery.
Epithelioid hemangioendothelioma, a vascular malignancy of endothelial cell origin, very rarely involves the orbit. This case is notable for its early development, rapid growth, bony origin, and epithelioid histology.
作者报告一例发生于眼眶骨的上皮样血管内皮瘤病例。对与这种罕见眼眶肿瘤相关的文献进行回顾后发现了8例记录完整的病例，其中1例患者的年龄比本文所报告的患者小，但均非起源于骨。
一名3个半月大的男童左侧眶下有一肿物，在3天内迅速增大。急诊计算机断层扫描显示一个大的肿瘤，伴有颧骨和上颌骨的明显骨质破坏。初步检查提示为横纹肌肉瘤，遂进行经结膜活检，但术中出现大量出血。最终病理诊断为骨源性上皮样血管内皮瘤，即2级血管内皮瘤。转移检查未发现其他疾病部位。后续治疗包括整块切除肿瘤，保留眼眶软组织和眼球。
患者术后20个月无疾病复发，视力固定和眼球运动正常。
上皮样血管内皮瘤是一种起源于内皮细胞的血管恶性肿瘤，极少累及眼眶。该病例因其早期发病、生长迅速、骨源性及上皮样组织学特征而值得关注。
