Nuffield Department of Clinical Neurosciences, John Radcliffe Hospital, University of Oxford, Level 6 West Wing, Headley Way, Oxford, OX3 9DU, UK.
Department of Neurosurgery, John Radcliffe Hospital, Oxford, UK.
Head Neck Pathol. 2020 Sep;14(3):792-798. doi: 10.1007/s12105-019-01062-w. Epub 2019 Aug 6.
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.
嗅神经母细胞瘤（ONB）是一种罕见的恶性肿瘤，起源于鼻腔穹窿的上部。尽管进行了手术切除和辅助放疗，这些病例的临床特征仍为隐匿性，且复发率很高。有少数报告显示 ONB 具有不同的上皮或神经节分化，并且 ONB 也与腺癌同时发生。我们报告了一例混合性嗅神经母细胞瘤伴腺癌。临床表现不典型，有强直阵挛性发作，此前有慢性头痛和嗅觉丧失。影像学显示从左侧鼻腔嗅沟延伸穿过筛板至前颅窝的肿块。病理学显示左侧嗅沟上皮内神经内分泌细胞增生。这一发现为这种罕见肿瘤的细胞起源提供了独特的见解，并似乎证实了 ONB 起源于嗅神经上皮中的神经干细胞这一理论。尽管进行了根治性治疗，但患者在治疗后 1 年内出现远处复发，这突出了该肿瘤的侵袭性。
