Treatment of intracranial metastatic esthesioneuroblastoma

Background: Esthesioneuroblastoma (ENB) is an uncommon tumor that may metastasize to the central nervous system (CNS). To the authors' knowledge there is no current consensus regarding treatment. The current study was conducted to determine the toxicity and response rate of combined modality therapy in the treatment of patients with ENB metastatic to the brain.

Methods: Six patients (2 men and 4 women) ranging in age from 47-61 years (median age, 53.5 years) with ENB had clinical and neuroradiographic evidence of CNS metastases (brain parenchyma in 6 patients and leptomeningeal metastases in 3 patients). CNS-directed therapy included radiotherapy (to the brain in three patients and to the spine in two patients) and chemotherapy (systemic in six patients and regional in three patients). Systemic chemotherapy was comprised of carboplatin, lomustine, and vincristine administered every 2 months. Patients were evaluated by neuroradiographic and neurologic examination every other month.

Results: Between 1-6 cycles of systemic chemotherapy were administered (median, 4.5 cycles). and 6-19 cycles of regional chemotherapy were administered (median, 17 cycles). Toxicity included aseptic meningitis (three patients), radiation enteritis (one patient), and > or = Grade 3 (according to the National Cancer Institute Common toxicity Criteria) myelosuppression (four patients). Two patients required hospitalization for neutropenic fever and two patients required a transfusion (platelets in two patients and red blood cells in one patient). No treatment-related deaths were reported. A partial response was achieved in four patients, and two patients demonstrated progressive disease. The median duration of response was 9 months (range, 2-12 months). Overall survival ranged from 3-13 months (median, 10.5 months). The cause of death was progressive parenchymal brain disease in three patients, systemic disease in two patients, and leptomeningeal disease in one patient.

Conclusions: In the small cohort of patients in the current study, combined modality therapy was found to have modest toxicity and palliative efficacy.