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June 11, 2026F1000Research0 citationsOpen Access

Case Report: Advanced Olfactory Neuroblastoma with Orbital and Skull Base Involvement: A Rare Case Presentation

JAJailene AyalaBLBianca LupiaAPAmalia Pontes

Key Points

  • To present a unique case of advanced olfactory neuroblastoma involving the orbit and skull base and discuss treatment outcomes.
  • Conducted imaging revealing a 6.7 cm sinonasal mass with orbital and skull base involvement.
  • Administered neoadjuvant chemotherapy with cisplatin and etoposide.
  • Performed surgical resection of the anterior skull base and surrounding structures.
  • Significant tumor reduction observed after chemotherapy.
  • Residual viable tumor detected in left infratemporal fossa and related tissues.
  • Patient scheduled for follow-up chemoradiation due to positive margins.

Abstract

Olfactory neuroblastoma (ONB) is an uncommon malignant cancer that invades the upper nasal cavity. Our patient presented with progressive left-sided vision loss and proptosis. The initial symptoms were misattributed to cataract-related changes, resulting in a delayed diagnosis. Further imaging revealed a 6.7 cm locally invasive sinonasal mass involving the ethmoid, sphenoid, and maxillary sinuses with orbital and skull base extension. Imaging confirmed extensive locoregional spread without intracranial invasion or distant metastasis. Histopathologic analysis of an endoscopic biopsy confirmed Hyams grade III olfactory neuroblastoma (ONB). The patient underwent neoadjuvant chemotherapy with cisplatin and etoposide, resulting in significant tumor reduction. Chemotherapy was followed up by resection of the anterior skull base, including the cribriform plate, left infratemporal fossa, left medial orbital wall, and left ethmoid mass, with a high-flow cerebrospinal fluid (CSF) leak repair. The final pathology displayed residual viable tumor in the left infratemporal fossa, lateral dura, and intracranial specimens, revealing positive margins; thus, our patient was scheduled to follow up for chemoradiation.

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Cite This Study

Ayala et al. (2026) studied this question.

synapsesocial.com/papers/6a2a517c80c8f91e7f39dc9bhttps://doi.org/10.12688/f1000research.178216.1
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