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[副神经节瘤的多系统表现。诊断、鉴别诊断及治疗指征]

[Polytopic manifestations of paragangliomas. Diagnosis, differential diagnosis and indications for therapy].

作者信息

Hoffmann J, Kröber S M, Hahn U, Ernemann U, Reinert S

机构信息

Klinik und Poliklinik für Mund-, Kiefer- und Gesichtschirurgie Tübingen, Deutschland.

出版信息

Mund Kiefer Gesichtschir. 2000 Jan;4(1):53-6. doi: 10.1007/s100060050014.

Abstract

Paragangliomas of the glomus caroticum are relatively rare, but highly vascularized neoplasmas, which develop from chemoreceptors. They can be develop at any age, but most often in the third or fourth decade of life. Paragangliomas grow very slowly and are most always of benign origin. There is a familial predisposition, and an autosomal-dominant transmission is presumed. They are commonly located in the jugular region; in rare cases a polytopic manifestation is found. We describe the case of a 47-year-old male patient who was referred to our department because of a progressive swelling of the neck on both sides. Contrast-enhanced computed tomography had displayed soft tissue tumors in the jugular regions. We performed an operative exploration, which showed a highly vascularized tumor. Histopathologic analysis revealed the diagnosis of a paraganglioma. An angiography of the neck and thoracic region, furthermore, revealed an additional paraganglioma in the anterior mediastinum. Using a surgical approach via lateral cervicotomies and thoracotomy the paragangliomas were extirpated. Our case report demonstrates the rare polytopic manifestation of paragangliomas. This perivascular neoplasms have to be removed before haemodynamic complications develop. The extent of this tumors is clearly illustrated by use of an angiography. Because of the familial predisposition, clinical and radiological examinations of relatives are mandatory.

摘要

颈动脉体副神经节瘤相对罕见,但血管高度丰富,由化学感受器发展而来。它可在任何年龄发病,但最常见于生命的第三个或第四个十年。副神经节瘤生长非常缓慢,大多起源于良性。存在家族易感性,推测为常染色体显性遗传。它们通常位于颈部区域;在罕见情况下会发现多部位表现。我们描述了一例47岁男性患者,因双侧颈部进行性肿胀被转诊至我科。增强计算机断层扫描显示颈部区域有软组织肿瘤。我们进行了手术探查,发现了一个血管高度丰富的肿瘤。组织病理学分析确诊为副神经节瘤。此外,颈部和胸部区域的血管造影显示前纵隔还有一个副神经节瘤。通过侧颈切开术和开胸手术的方法将副神经节瘤切除。我们的病例报告展示了副神经节瘤罕见的多部位表现。这种血管周围肿瘤必须在血流动力学并发症发生之前切除。血管造影清楚地显示了这种肿瘤的范围。由于存在家族易感性,必须对亲属进行临床和放射学检查。

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