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2026 Proffered Presentations

2026 Proffered Presentations

 

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S158: BEYOND THE SKULL BASE: INTRADURAL SPINAL METASTASES IN ESTHESIONEUROBLASTOMA
Danielle D Dang, MD, MA; Jordan Jones, MD, PhD; Anna Davide, BA; Ruth Agwaze, BS; Alexander Pais, MD; Derrick Obiri-Yeboah, MD; Colin Driscoll, MD; William Krauss, MD; Michael Link, MD; Mayo Clinic Rochester

Introduction: Esthesioneuroblastoma (ENB) is a rare malignant neoplasm of the olfactory epithelium with highly variable clinical behavior. Local invasion, intracranial extension, and hematogenous spread to cervical lymph nodes and bone are well described, but distant intradural spinal metastases through cerebrospinal fluid dissemination are uncommon and poorly understood.

Objective: To systematically review all reported cases of intradural spinal metastases from ENB and evaluate potential risk factors and predictors for survival

Methods: We present a case of thoracic intramedullary spinal metastasis 17 years after ENB resection, with clinical course and outcomes. A systematic review was conducted using a MEDLINE search of English literature through September 2025 per PRISMA guidelines to identify all cases of intradural spinal metastases from ENB. Exclusion criteria included extradural or vertebral-contiguous metastases and meta-analyses without extractable patient-level data. Univariate analyses used t-tests and Chi square/Fisher’s exact tests. Kaplan–Meier curves with log-rank testing assessed risk factors for metastases and predictors for overall survival (OS). Type 1 error was defined as p<0.05.

Results

Case Report: A 57-year-old female with Kadish C, Hyams grade 2 ENB underwent combined anterior craniofacial resection. Seventeen years later, with interval treatment for regional recurrences, she developed a 9 x 11 x 15mm intramedullary T3 metastasis and a spheno-orbital mass. She underwent resection and fractionated spinal radiotherapy (3,500 cGy) followed by frontotemporal craniotomy with anterior clinoidectomy. Pathology confirmed ENB at both sites. She remains alive without disease 16 months postoperatively, 20 years since initial diagnosis (Figure 1).

Systematic Review: Twenty-two studies met inclusion, yielding 24 intradural spinal metastases including our case. Demographics, primary ENB characteristics, surgical course, and adjuvant therapies were abstracted (Figure 2). Regional recurrence and postoperative CSF leaks correlated with shorter latency to spinal metastases (p=0.0012; p= 0.0026) and preferential thoracolumbar and cervicothoracic involvement, respectively. Females trended toward leptomeningeal disease (p=0.074), and older age toward decreased OS (p=0.083).

Mean latency was 59.4 months (range 3.5–240). Sites included thoracic (52.2%) and lumbar (39.1%) spine. 79.2% harbored discrete intradural lesions, 54.2% incurred leptomeningeal dissemination, and 33.3% had both. Within the intradural compartment, 77.8% were extramedullary, 16.7% involved the proximal nerve root, and 11.1% were intramedullary. Solitary lesions were present in 61.1%, while 38.9% had multiple tumors.

Management varied by disease burden, targetable lesions, and symptom severity: radiotherapy alone (30.4%) was most common, followed by surgery plus radiotherapy (21.7%), chemoradiation (17.4%), and surgery plus chemotherapy (13%). Median OS after spinal metastasis was 11 months (95% CI: 4–41) (Figure 3). Treatment modality was the only factor associated with survival where combined modalities (14-18 months) were superior to radiotherapy or chemotherapy alone (4-5 months) (Figure 4).

Conclusion: Intradural spinal metastases from ENB are extremely rare with poor prognosis, with median survival under one year. Regional recurrence and postoperative CSF leak may increase risk. Our case represents the second longest latency, one of three intramedullary presentations, and the only survival beyond eight months from discovery. Findings highlight the importance of prolonged surveillance and multimodal treatment strategies for survival benefit.

 

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