Orbital intraosseous hemangioma; report of three cases and systematic review

PURPOSE: To present three new cases of primary intraosseous orbital hemangioma (PIOH), including rare lacrimal bone involvement, and to provide an updated systematic review of previously reported cases. METHODS: We retrospectively reviewed three patients with orbital PIOH from our institution. Clinical, radiographic, histopathologic, and surgical findings were analyzed. A comprehensive systematic review of 84 previously published PIOH cases was performed to assess demographics, anatomic location, imaging, and treatment outcomes. RESULTS: All three patients had firm, progressively enlarging orbital masses. Imaging showed well-circumscribed, expansile lesions confirmed as PIOH histologically. Case 1 involved the zygomatic and sphenoid bones; Case 2, the maxilla and lacrimal bone; and Case 3, the frontal bone. All underwent orbitotomy with subtotal or complete excision and reconstruction. No recurrence was observed at 1-34 month follow-up. In the systematic review, the zygoma (50%) and frontal bone (44%) were most frequently involved. Common symptoms included swelling, proptosis, pain, and globe displacement. CT was the most used imaging modality. Surgical excision, often with preoperative embolization, remains the preferred treatment. Only one recurrence (1.8%) was reported out of the 56 cases with follow-up data. CONCLUSIONS: Orbital PIOH is a rare vascular tumor best managed by tailored surgical excision. Our findings and updated systematic review emphasize individualized planning, particularly in rare sites like the lacrimal bone.