Otolaryngology, Sir Charles Gairdner Hospital, Perth, Western Australia, Australia
Otorhinolaryngology, Tan Tock Seng Hospital, Singapore.
BMJ Case Rep. 2023 May 2;16(5):e254848. doi: 10.1136/bcr-2023-254848.
Our patient is a man in his mid-20s with an atypical presentation of a catecholamine-secreting sinonasal paraganglioma. He was referred to our tertiary otolaryngology unit for persistent right infraorbital numbness. A nasoendoscopic examination demonstrated a smooth mass arising from the posterior aspect of the right middle meatus. There was also right infraorbital paraesthesia. Imaging revealed a lesion in the right pterygopalatine fossa. Blood investigations revealed significantly elevated serum normetanephrine levels. The lesion was demonstrated to be octreotide-avid with no other lesions detected. The presumptive diagnosis of a catecholamine-secreting paraganglioma was made, and an endoscopic resection of the tumour was performed. Histopathology of the tumour demonstrated a 'zellballen' growth pattern consistent with a paraganglioma. Catecholamine-secreting sinonasal paragangliomas are exceedingly rare with multifaceted challenges. More studies are required to improve our knowledge of this condition.
我们的患者是一位 20 多岁的男性，表现出非典型的儿茶酚胺分泌性鼻旁副神经节瘤。他因持续性右眶下麻木被转诊至我们的三级耳鼻喉科单位。鼻内镜检查显示右侧中鼻道后缘有一光滑肿块。同时存在右眶下感觉异常。影像学显示右侧翼腭窝有病变。血液检查显示血清去甲变肾上腺素水平显著升高。该病变表现为奥曲肽阳性，未发现其他病变。诊断为分泌儿茶酚胺的副神经节瘤，并进行了肿瘤内镜切除术。肿瘤的组织病理学表现为“zellballen”生长模式，符合副神经节瘤。分泌儿茶酚胺的鼻旁副神经节瘤极为罕见，具有多方面的挑战。需要进一步研究以提高对这种疾病的认识。
