Shamsi Zaid-Ahmed, Shaikh Fareed-Ahmed, Wasif Muhammad, Chaudhry Mustafa-Belal-Hafeez, Siddiqui Nadeem-Ahmed, Sophie Ziad
Department of Surgery, Aga Khan University Hospital, Karachi, Pakistan .
Department of Surgery, Ziauddin University and Hospital.
Iran J Otorhinolaryngol. 2021 Mar;33(115):113-117. doi: 10.22038/ijorl.2020.43602.2448.
Paraganglioma are infrequent neuroendocrine tumors that are most commonly found in the carotid body, ganglia of the vagus, jugular and tympanic nerve. Very rarely they can involve other cranial nerves outside the cranial cavity, we present one such case of hypoglossal nerve paraganglioma in neck.
A 48 years old male presented with 1-month history of right sided stroke and aphasia. Ultrasonography of neck revealed a highly vascular mass on the right side of the neck. CT angiogram confirmed a highly vascular mass arising above the carotid bifurcation. With the working diagnosis of Glomus tumor, he underwent right sided neck exploration, however, intra-operatively tumor was found to be arising from the hypoglossal nerve instead. Surgery was abandoned on basis of the available literature, with only 6 reported cases in the past 54 years. Patient had no immediate post op complications and was sent for cyber knife treatment. After completion of 5 cycles of cyber knife there was a total of 45% reduction in the size of the paraganglioma with the resolution of the patient's symptoms after a follow up of 6 months.
Hypoglossal nerve paraganglioma is an uncommon tumor of the neck and can be misdiagnosed with the other tumors in this region especially chemodectoma and glomus tumor. The diagnostic criteria and appropriate treatment modalities have not been established due to the rare presentation hence hypoglossal paraganliomas should be kept in mind when Highly vascular neck mass is encountered.
副神经节瘤是一种罕见的神经内分泌肿瘤,最常见于颈动脉体、迷走神经节、颈静脉神经节和鼓室神经节。它们极少会累及颅腔外的其他颅神经,我们在此报告一例颈部舌下神经副神经节瘤病例。
一名48岁男性,有1个月右侧中风和失语病史。颈部超声检查发现颈部右侧有一个高血管性肿块。CT血管造影证实一个高血管性肿块起源于颈动脉分叉上方。初步诊断为球瘤,患者接受了右侧颈部探查手术,但术中发现肿瘤实际起源于舌下神经。根据现有文献,过去54年仅有6例报告,因此手术终止。患者术后无即刻并发症,随后接受了射波刀治疗。完成5个周期的射波刀治疗后,副神经节瘤大小总共缩小了45%,随访6个月后患者症状消失。
舌下神经副神经节瘤是一种罕见的颈部肿瘤,可能会被误诊为该区域的其他肿瘤,尤其是化学感受器瘤和球瘤。由于其罕见性,尚未确立诊断标准和合适的治疗方式,因此当遇到颈部高血管性肿块时应考虑舌下神经副神经节瘤。