Szymańska Anna, Szymański Marcin, Czekajska-Chehab Elżbieta, Gołąbek Wiesław, Szczerbo-Trojanowska Małgorzata
Department of Interventional Radiology and Neuroradiology, Medical University of Lublin, Jaczewskiego 8, 20-154, Lublin, Poland.
Eur Arch Otorhinolaryngol. 2015 Aug;272(8):1991-9. doi: 10.1007/s00405-014-3126-z. Epub 2014 Jun 12.
Paragangliomas (PGs) are slowly growing, usually benign neoplasms. The aim of the study was to analyze the incidence, diagnostic and therapeutic management of patients with multiple paragangliomas of the head and neck. A retrospective review of the records of 84 patients with head and neck PGs, diagnosed and treated in our institution was performed for the years 1983-2013 to identify patients with multiple tumors. Fourteen (16.6 %) patients developed multiple PGs, synchronous or metachronous, within 4-21 years of follow-up. Clinical data of these patients were reviewed to evaluate the diagnosis, location, stage and management strategy. There was a total number of 37 tumors in 14 patients. There were 20/37 (54.0 %) carotid PGs, 9/37 (24.3 %) jugular PGs and 8/37 (21.7 %) vagal PGs. Carotid PGs were observed in 12/14 (86 %) patients and in 8/14 (57 %) cases bilateral tumors occurred. Vagal PGs developed in 7/14 (50 %) patients and bilateral tumors were found in 1/14 (7 %) case. Jugular PGs occurred in 9/14 (64 %) patients. There were 30 synchronous tumors and seven metachronous PGs diagnosed 2-18 years after removal of the first tumor. Single metachronous mediastinal PG occurred. All patients had at least one tumor removed, with histopathological confirmation of the diagnosis. One patient had positive history of familial PGs. Carotid PGs are most common multiple paragangliomas. Radiological survey of the head and neck is required to detect multicentric tumors. Metachronous mediastinal and abdominal tumors may occur. Regular, prolonged follow-up is essential to identify metachronous PGs and possible postoperative gradual ICA occlusion.
副神经节瘤（PGs）是生长缓慢的肿瘤，通常为良性。本研究旨在分析头颈部多发性副神经节瘤患者的发病率、诊断及治疗情况。对1983年至2013年期间在我院诊断并治疗的84例头颈部PGs患者的记录进行回顾性分析，以确定患有多发性肿瘤的患者。14例（16.6%）患者在4至21年的随访期内出现了同步或异时性的多发性PGs。对这些患者的临床资料进行回顾，以评估诊断、肿瘤位置、分期及治疗策略。14例患者共有37个肿瘤。其中颈动脉体瘤20/37（54.0%），颈静脉球瘤9/37（24.3%），迷走神经副神经节瘤8/37（21.7%）。12/14（86%）例患者有颈动脉体瘤，其中8/14（57%）例为双侧肿瘤。7/14（50%）例患者有迷走神经副神经节瘤，其中1/14（7%）例为双侧肿瘤。9/14（64%）例患者有颈静脉球瘤。共诊断出30个同步肿瘤和7个异时性PGs，后者在切除首个肿瘤后2至18年被诊断出。出现1例孤立的异时性纵隔PG。所有患者至少切除了1个肿瘤，且经组织病理学确诊。1例患者有家族性PGs阳性病史。颈动脉体瘤是最常见的多发性副神经节瘤。需要对头颈部进行影像学检查以发现多中心肿瘤。可能会出现异时性纵隔和腹部肿瘤。定期、长期随访对于发现异时性PGs及术后可能逐渐发生的颈内动脉闭塞至关重要。
