ATNT-09BEVACIZUMAB FOR METASTATIC DURAL AND ORBITAL ESTHESIONEUROBLASTOMA

Abstract

Esthesioneuroblastoma is a malignant neuroectodermal tumor originating from the olfactory neuroepithelium. Bevacizumab is a specific inhibitor of the vascular endothelial growth factor ligand, preventing its interaction with receptors on endothelial cells. We report two cases of metastatic dural esthesioneuroblastoma that responded to bevacizumab. Patient 1, a 45 year-old man, had been treated initially for local disease with resection and radiation therapy (RT); for submandibular recurrence with radical neck dissection, RT, and cis-platinum plus etoposide; and subsequently for leptomeningeal disease with temozolomide plus sunitinib. He had an enlarging right orbital mass with progressive dural and leptomeningeal involvement. Patient 2, a 66 year-old woman, had been treated initially for local disease with craniotomy, RT, and cis-platinum; then for local recurrence with both regional and stereotactic RT. She developed enlarging orbital, dural and scalp masses. Both patients were treated with bevacizumab, 5 mg/kg every 2 weeks. Patient 1 achieved a partial response, with reduction in dural contrast enhancement, tumor cyst volumes, and perilesional edema, along with improvement in left leg weakness and right ptosis. His tumors progressed on imaging after 260 days of therapy. He died after 602 days of bevacizumab despite the addition of lomustine. Patient 2 achieved stable disease for 457 days but died of respiratory complications after developing a cerebrospinal fluid leak, without tumor progression. These patients with metastatic orbital and dural esthesioneuroblastoma achieved durable responses with bevacizumab therapy. Anti-angiogenic strategies may play a role in salvage therapy for this tumor.