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[头颈部副神经节瘤]

[Paragangliomas of the head and neck].

作者信息

Strasser Verena, Steinbichler Teresa

机构信息

Department of Otorhinolaryngology, Head and Neck Surgery, Medical University of Innsbruck, 6020, Innsbruck, Austria.

出版信息

HNO. 2024 Aug;72(8):598-608. doi: 10.1007/s00106-024-01480-5. Epub 2024 Jun 4.

Abstract

Paragangliomas represent a heterogeneous group of rare neuroendocrine tumors with marked variability in symptoms and disease course. Due to the close proximity to neurovascular structures, paragangliomas of the head and neck region can cause a variety of symptoms. To this day, there are no reliable prognostic factors that can predict a potentially malignant course. All patients with newly diagnosed paragangliomas should undergo an early diagnostic workup and regular follow-up examinations in specialized centers. While radical resection was previously regarded as standard treatment for paragangliomas, radiotherapy and active surveillance (watch-and-scan strategy) have become equally important over the years. Low-threshold techniques for molecular pathology analysis of the mutation-specific behavior of paragangliomas are nowadays available.

摘要

副神经节瘤是一组异质性罕见的神经内分泌肿瘤,症状和病程差异很大。由于与神经血管结构相邻,头颈部副神经节瘤可引起多种症状。时至今日,尚无可靠的预后因素能够预测潜在的恶性病程。所有新诊断为副神经节瘤的患者均应在专业中心接受早期诊断检查和定期随访。虽然根治性切除曾被视为副神经节瘤的标准治疗方法,但近年来放疗和主动监测(观察与扫描策略)也变得同样重要。如今已有低门槛技术可用于副神经节瘤突变特异性行为的分子病理学分析。

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