Sinha Parul, Sharma Suresh C, Agarwal Shipra, Gupta Siddhartha Datta
Department of Otorhinolaryngology-Head and Neck Surgery, All India Institute of Medical Sciences, New Delhi, India.
Ann Otol Rhinol Laryngol. 2011 Dec;120(12):769-74. doi: 10.1177/000348941112001201.
Ganglioneuroma is a rare benign tumor of the sympathetic nervous system that is seldom localized to the head and neck. Rarer still are cases of functional ganglioneuroma with catecholamine production. We report an unusual case of a large parapharyngeal space ganglioneuroma in a patient with neurofibromatosis that caused an intraoperative hypertensive crisis in the absence of a neuroblastomatous component - hitherto an undocumented complication.
We present a case of functional parapharyngeal ganglioneuroma and review the clinicopathologic and biochemical features of these rare tumors.
Fewer than 40 anecdotal cases of parapharyngeal ganglioneuromas have been reported, and there is a lack of adequate literature on the biology and appropriate treatment approach of these tumors.
Our case illustrates the importance of vigilant preoperative precautions in the management of neurogenic tumors, particularly ganglioneuromas. These usually nonfunctional tumors can result in sudden intraoperative and postoperative hypertensive crises even in the absence of any preoperative autonomic symptoms.
神经节神经瘤是一种罕见的交感神经系统良性肿瘤，很少局限于头颈部。更罕见的是具有儿茶酚胺分泌功能的神经节神经瘤病例。我们报告了一例不寻常的病例，一名患有神经纤维瘤病的患者发生了巨大的咽旁间隙神经节神经瘤，该肿瘤在没有神经母细胞瘤成分的情况下引发了术中高血压危象——这是迄今为止未记录的并发症。
我们展示了一例功能性咽旁神经节神经瘤病例，并回顾了这些罕见肿瘤的临床病理和生化特征。
已报道的咽旁神经节神经瘤病例不足40例，且缺乏关于这些肿瘤生物学特性和适当治疗方法的充分文献。
我们的病例说明了在神经源性肿瘤，特别是神经节神经瘤的管理中，术前采取警惕性预防措施的重要性。这些通常无功能的肿瘤即使在没有任何术前自主神经症状的情况下，也可能导致术中及术后突然出现高血压危象。
