Abstract
Background: Neuroendocrine neoplasms (NENs) of the nasal cavity, paranasal sinuses and nasopharynx account for a wide spectrum of histotypes. They can range from indolent form to highly aggressive tumors. Olfactory neuroblastoma, like most sino-nasal NENs, represents a rare neoplasm that prompts diagnostic pitfalls. From a morphological perspective, olfactory neuroblastoma can mimic many nasal neoplasms but a proper recognition is mandatory for its aggressive behaviour. Case presentation: A case of olfactory neuroblastoma in a 85 years old woman is reported. The neoplasm has been surgical removed from the nasal cavity with a clinical and radiographic diagnosis of nasal polyp. Correct diagnostic definition has needed a complete histological and immunohistochemical characterization of the tumoral tissue in surgical pathology laboratory. Conclusion: The correct diagnosis of olfactory neuroblastoma directs the clinical management that is unique for this neoplasm in comparison to other nasal ones. Bicranial-facial surgery or a trephination procedure represent the most common approach for the treatment of this malignancy. Their success determine disease prognosis.
Highlights
Neuroendocrine neoplasms (NENs) of the nasal cavity, paranasal sinuses and nasopharynx account for a wide spectrum of histotypes
The correct diagnosis of olfactory neuroblastoma directs the clinical management that is unique for this neoplasm in comparison to other nasal ones
Cases of sino-nasal neoplasms composed of neuroendocrine and non-neuroendocrine component have been documented in literature
Summary
The present manuscript represents a chance to evidence that ONBs have to be considered heterogeneous tumors from a carcinogenetic and clinical course perspective. The complexity of genomic aberrations and gene mutations in ONB are linked to tumorigenesis molecular mechanisms; genomic assessment of this kind of tumor could be related as to the clinical course, as its morphological aspects. Detection of new genetic hallmarks of ONBs could identify new potential therapeutic targets and predict response rate to standard therapies
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