Extraprimary Local Recurrence of Esthesioneuroblastoma: A Case Series

Background Esthesioneuroblastoma (ENB) is a rare sinonasal malignancy arising from olfactory neuroepithelium. Recurrence most often occur locoregionally at the primary site or in the form of cervical metastasis. However, delayed local recurrence away from the initial primary occurrence is exceedingly rare.

Methods Retrospective review of three patients with histologically confirmed extraprimary local recurrence of ENB treated at two tertiary academic centers with review of the literature.

Case Series All three cases initially presented with ENB isolated to the unilateral or bilateral cribriform plates treated with primary surgical resection and adjuvant radiotherapy. In the first case, the patient was noted to have recurrence of ENB, 8 years after initial presentation, involving the right orbit. She was subsequently found to have metastases to the spine, neck, and mandible requiring composite resection and ultimately four courses of radiotherapy throughout the course of her disease. In the second case, a patient presented 8 years after initial presentation with ENB recurrence of the left dorsal septum bordering the nasofrontal beak with metastases to the right neck requiring septectomy, bilateral neck dissection, and repeat radiotherapy. In the third case, the patient remained free of disease until 7 years after initial treatment when he was found to have recurrent disease in the posterior nasopharynx requiring endonasal nasopharyngectomy. All three patients demonstrated no evidence of disease respectively at 2, 3 years, and 3 months posttreatment.

Discussion ENB is a rare sinonasal malignancy with a tendency for local and delayed recurrence. This is the first case series to describe delayed extraprimary local recurrence of histologically confirmed ENB. Treatment of extraprimary recurrences is similar to other forms of ENB and should include primary surgical resection with adjuvant radiotherapy, with favorable survival outcomes. Long-term close follow-up is essential due to the risk of extreme delays in recurrence.