Primary orbital intraosseous hemangioma

Abstract

Purpose: Primary orbital intraosseous hemangioma represents a rare, histopathologically benign, vascular tumor of the bony orbit. Only 41 cases have been documented in the literature to date. The authors present 4 new cases of the disease and review the relevant literature. Methods: Retrospective, multicenter case note analysis of 4 patients with histopathologically confirmed primary orbital intraosseous hemangioma and a systematic review of the English-language literature. Results: Four new cases of cavernous haemangiomata are presented with varying clinical manifestations, radiologic appearances, and treatments. Literature review (including the present 4 cases) yielded 45 cases in total. Presentation is often in the fourth and fifth decades (42% cases), the frontal bone being most commonly affected, followed by the zygoma, sphenoid, and maxilla. Intracranial extension occurred in 4 cases. Median duration of symptoms before presentation was 12 months (range, 1 month to 15 years) and the most frequent presentation was a painless mass, often on the orbital rim. The radiologic findings are reviewed. Histopathologically, the lesions were cavernous in 80%, capillary in 17%, and mixed in 3%; the capillary subtype seemed to be associated with more aggressive disease. Treatment was mainly by surgical excision and occasionally complicated by significant blood loss; preoperative embolization of lesions may reduce bleeding. Conclusions: Primary orbital intraosseous hemangioma is a rare vascular tumor that typically presents with a mass effect in the orbits of patients in the fourth and fifth decades of life. Preoperatively, it is important to be cognizant of the possible diagnosis as surgery can be complicated by life-threatening hemorrhage. Methods: Retrospective, multicenter case note analysis of 4 patients with histopathologically confirmed primary orbital intraosseous hemangioma and a systematic review of the English-language literature. Results: Four new cases of cavernous haemangiomata are presented with varying clinical manifestations, radiologic appearances, and treatments. Literature review (including the present 4 cases) yielded 45 cases in total. Presentation is often in the fourth and fifth decades (42% cases), the frontal bone being most commonly affected, followed by the zygoma, sphenoid, and maxilla. Intracranial extension occurred in 4 cases. Median duration of symptoms before presentation was 12 months (range, 1 month to 15 years) and the most frequent presentation was a painless mass, often on the orbital rim. The radiologic findings are reviewed. Histopathologically, the lesions were cavernous in 80%, capillary in 17%, and mixed in 3%; the capillary subtype seemed to be associated with more aggressive disease. Treatment was mainly by surgical excision and occasionally complicated by significant blood loss; preoperative embolization of lesions may reduce bleeding. Conclusions: Primary orbital intraosseous hemangioma is a rare vascular tumor that typically presents with a mass effect in the orbits of patients in the fourth and fifth decades of life. Preoperatively, it is important to be cognizant of the possible diagnosis as surgery can be complicated by life-threatening hemorrhage.