Metastatic esthesioneuroblastoma with widespread recurrence to the central nervous system

Esthesioneuroblastomas (ENBs) are rare malignancies of the upper digestive tract, often demonstrating local metastasis to the intracranial space through the cribriform plate. These tumors show high rates of recurrence locally following treatment. Here, we report a patient with advanced recurrent ENB 2 years following initial treatment, affecting both the spine and intracranial space without evidence of local recurrence or contiguous extension from the initial tumor site.

A 32-year-old male presents with a 2 month history of neurological symptoms 2 years following treatment of Kadish C/AJCC stage IVB (T4a, N3, M0) ENB. No evidence of locoregional recurrent disease was observed prior with intermittent imaging. Imaging revealed a large ventral epidural tumor invading multiple levels of the thoracic spine as well as a ring enhancing lesion in the right parietal lobe. The patient was treated surgically with debridement, decompression, and posterior stabilization of the thoracic spine followed by radiotherapy to the spinal and parietal lesions. Chemotherapy was also initiated. Despite treatment, the patient passed away 6 months after surgery.

We report a case of delayed recurrent ENB with widespread metastases to the central nervous system without evidence of local disease or contiguous extension from initial tumor site. This represents a highly aggressive form of this tumor as recurrences are primarily locoregional. In follow-up of ENB treatment, clinicians must be cognizant of these tumors demonstrated ability to spread to distal regions. All new onset neurological symptoms should be investigated fully even if no local recurrence is observed.