Cranial and spinal leptomeningeal dissemination in esthesioneuroblastoma: Two reports of distant central nervous system metastasis and rationale for treatment.

BACKGROUND: Esthesioneuroblastoma is a locally aggressive cancer of the nasal cavity. While systemic metastasis can occur in 10-30% of patients, there are only six reported cases of distal metastasis from leptomeningeal dissemination.

CASE DESCRIPTION: The authors report two cases of esthesioneuroblastoma treated previously with multimodal therapy in which distal metastatic recurrence was found and describe their treatment protocol, which has resulted in long-term success.

CONCLUSION: Understanding the drivers of leptomeningeal dissemination in more prevalent primary neuroectodermal tumors may hold the key to developing successful treatment algorithms for this disease.