Mixed Olfactory Neuroblastoma and Adenocarcinoma with In Situ Neuroendocrine Hyperplasia

Jonathan E Attwood,Deva Sanjeeva Jeyaretna,Fintan Sheerin,Ketan A Shah

doi:10.1007/s12105-019-01062-w

Jonathan E Attwood, Deva Sanjeeva Jeyaretna + Show 2 more

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https://doi.org/10.1007/s12105-019-01062-w

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Abstract

Olfactory neuroblastoma (ONB) is a rare malignant neoplasm arising from the superior aspect of the nasal vault. Cases are characterised by insidious clinical presentation and high rates of recurrence despite surgical resection and adjuvant radiotherapy. There are a small number of reports showing ONB with divergent epithelial or ganglionic differentiation, and ONB has also been found to coincide with adenocarcinoma. We present a case of mixed ONB with adenocarcinoma. The clinical presentation was unusual, with a tonic–clonic seizure preceded by chronic headache and anosmia. Imaging revealed a mass extending from the olfactory recess of the left nasal cavity through the cribriform plate to the anterior cranial fossa. The pathology demonstrated intraepithelial neuroendocrine cell hyperplasia in the left olfactory groove. This finding provides a unique insight into the cellular origin of this rare tumour, and appears to confirm the theory that ONB arises from neural stem cells in the olfactory neuroepithelium. Despite radical treatment, the patient suffered a distant recurrence within 1 year of treatment, which underlines the aggressive nature of this tumour.

Highlights

Olfactory neuroblastoma (ONB), or esthesioneuroblastoma, is a rare malignant neoplasm arising from the superior aspect of the nasal vault [1]
ONB was first described by Berger et al in 1924 [2], and is thought to originate from pluripotent neural stem cells which supply the olfactory neuroepithelium lining the underside of the cribriform plate of the ethmoid bone [1]
We present a case of mixed ONB with adenocarcinoma which demonstrates intraepithelial neuroendocrine cell hyperplasia in the left olfactory groove

Summary

Introduction

Olfactory neuroblastoma (ONB), or esthesioneuroblastoma, is a rare malignant neoplasm arising from the superior aspect of the nasal vault [1]. Head and Neck Pathology (2020) 14:792–798 response to chemotherapy [15] and radiotherapy [16] The diversity of this tumour is likely to be result of the high degree of pluripotency shown by the neural stem cells from which it is thought to arise. We present a case of mixed ONB with adenocarcinoma which demonstrates intraepithelial neuroendocrine cell hyperplasia in the left olfactory groove This finding provides a unique insight into the cellular origin of this rare tumour, and appears to confirm the theory that ONB arises from neural stem cells in the olfactory neuroepithelium. The specimens surgically resected from the left sinonasal cavity and from inside the anterior cranial fossa revealed a distinctly biphasic tumour, comprising an epithelial component of atypical cuboidal and ciliated columnar cells, and a neuroendocrine component of primitive-appearing small cells with scant cytoplasm and speckled nuclear chromatin (Fig. 3a–d). Six month post-treatment whole-body PET-CT has shown no evidence of recurrent disease

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