Advanced olfactory neuroblastoma in a teenager: a clinical case and short review of literature

Background: Olfactory neuroblastoma, also called esthesioneuroblastoma (ONB), is a rare neuroectodermal neoplasm that originates from the olfactory epithelium of the nose-sinus tract. It generally occurs with epistaxis, nasal obstruction, diplopia, and anosmia. Methods: A 16-year-old female was admitted to our Unit with a complaint of nasal obstruction, recurrent epistaxis, anosmia, and intermittent headache of sixth month’s duration. After the ENT consultation, physical examination, endonasal endoscopy, and multiple biopsies were performed. Instrumental images (CT, MRI) have been requested to stage the aforementioned pathology. Results: Instrumental images (CT, MRI) showed a mass filling the right nasal cavity and the maxillary bone and involving the cribriform plate without evidence of dural invasion. Craniofacial resection by means of a bifrontal craniotomy combined with a modified lateral rhinotomic transfacial route was performed. The reconstruction of the inferior and medial orbital walls with employing split-thickness calvarial grafts, pedicled galea-pericranium flap rotated downwards was performed. The patient received 56 Gy of external beam radiotherapy over a 6-week period. Conclusion: Early diagnosis and treatment coordinated by a multidisciplinary team of ENTs, neurosurgeons, oncologists, pathologists, and radiologists are a prerequisite for a good prognosis. An excellent surgical debulking, negative margins, and subsequent locoregional control of the pathology through radiotherapy is fundamental.