Inoperable Orbital Epithelioid Hemangioendothelioma Treated With Radiation Therapy

Abstract

Epithelioid hemangioendotheliomas (EHs) are rare vascular tumors originating from bone or soft tissue. An 81-year-old woman with inoperable EH of the right orbit presented with right-sided decreased vision, proptosis, and facial numbness secondary to a frontotemporal soft tissue mass. Imaging revealed a heterogeneous enhancing mass surrounding the right sphenoid bone extending into the right orbit and right temporal intracranial fossa. The biopsy of the lesion was consistent with EH. The patient received orbital radiation with improvement in her proptosis, vision, and tumor size. However, 20 months later, she developed metastatic spread and her brief improvement rapidly worsened to require eventual enucleation. Orbital EH is rare, with few case reports in the literature. Treatment typically involves complete excision, although adjuvant radiation has been rarely reported. This case highlights rare orbital EH treated initially with radiotherapy with good effect, potentially indicating a role for this palliative therapy in extensive disease.