Primary Intraosseous Hemangioma of the Zygomatic Bone

1. Primary Intraosseous Hemangioma of the Zygomatic Bone Igal Leibovitch MD, Jean-Paul Dray MD

2. Introduction • Intraosseous hemangiomas are uncommon • Constitutingless than 1% of all osseous tumors • Primary involvement of the facial bones is rare and zygomatic bone involvement is even more rare

3. Case Report • A 47-year-old woman presented with a two-year history of progressive painless swelling in the temporal part of the right orbital rim • The ophthalmologic examination was otherwise unremarkable and the visual acuity was 20/20 in both eyes

4. Case Report – cont. • CT showed a well defined bony lesion located in the Rt zygoma with internal radiating trabeculations and a honeycomb pattern, without an associated soft tissue mass

5. Case Report- cont. • The patient had a history of surgically treated breast carcinoma 8 years ago and was concerned that the mass could be related

6. Case Report- cont. • Surgery was performed using a temporal skin incision and dissection until the zygomatic bone followed by orbitotomy and osteotomy with an en bloc resection of the tumor using an oscillating saw incising adjacent to the tumor in the normal bone • Hemostasis was achieved using compression, bone wax and diathermy

7. Histology • Large cavernous vascular spaces filled with erythrocytes and lined by a single layer of endothelial cells and surrounded by osseous trabeculae

8. Diagnosis • The diagnosis was primary intraosseous cavernous hemangioma of the zygomatic bone and the patient recovered uneventfully with no recurrence of the tumor after a two-year follow up

9. Discussion • Hemangiomasare hamartomas with an aberrant vascular proliferation • Intraosseous hemangiomas of the facial bones are rare and mostcommonly arise in the maxilla, mandible or nasal bones • Reported cases in the zygoma are even more rare and only about25 cases of zygomatic hemangiomas have been reported previously in the English written literature

10. Discussion – cont. • Most patients presentin about the fourth or fifth decade of life • CT and MR are useful diagnostic tools showing a characteristiccross-sectional configuration, with radiating trabeculae andintact, smooth cortices

11. Discussion – cont. • The orbital intraosseous hemangiomasare slow growing, and treatment is generally not indicated • There are several therapeutic options to facial bone hemangiomas, including the use of steroids, radiotherapy, curettage, and embolization of the main afferent vessels

12. Discussion – cont. • Possible indicationsfor surgery include pressure of the mass on the adjacent eye, control of hemorrhage and because of cosmetic reasons • Surgery includes removing the mass completely as well as rim of normalbone and immediate reconstruction of the created defect • Recurrence after removal is rare

13. Discussion – cont. • The patient we presented with a slowly growing bony mass in the right orbital rim had a previous history of breast carcinoma • Although she had no clinical symptoms and no visual disturbances, the history of breast cancer was crucial in the decision to perform the operation and not just continue observation

14. Conclusion • We believe that in patients with an orbital intraosseous mass and radiological findings suggestive of this rare hamartoma but with a history of malignancy, there is an indication for surgical removal of the tumor to rule out metastatic disease