背景和目的:头颈部副神经节瘤罕见,生长缓慢的神经内分泌肿瘤,绝大多数是良性的,但有可能发生远处转移。他们表现出极大的继承性,他们的行为被证明是不可预测的;因此,他们被认为是恶性的。材料与方法:本文旨在更全面地介绍其发病机制,流行病学,诊断方法,成像发展,和治疗指南。我们试图汇集所有必要的数据,在我们看来,头颈部医生应该知道何时处理这种类型的肿瘤。我们的主要重点是最近的研究,目的是对该病理学的所有当前指南和方法进行同质介绍。结果:头颈部副神经节瘤仍然是一个有争议的话题。其主要原因之一是每年每100,000人中有0.3至1人的低发病率。最常见的位置是颈动脉体,颞骨,颈静脉和乳突孔,还有迷走神经.他们的临床表现通常涉及无痛侧块与症状相关,如声音嘶哑,听力损失,耳鸣,和颅神经缺陷。其中高达40%是遗传的,主要与琥珀酸脱氢酶复合物的突变有关。影像学评估包括CT和MRI,以及18F-FDA和18F-FDGPET/CT等新的功能探索,18F-DOPAPET,123I-MIBG,和68Ga-DOTATEPET/CT。测量血浆和尿液中的儿茶酚胺水平是强制性的,尽管头颈部副神经节瘤很少表现出分泌行为。治疗主要包括手术,用不同的方法和技术,但是保守的管理方法,如等待和扫描,放射治疗,质子治疗,化疗已经证明了它们的效率。治疗性决定缺乏共识,目前的研究倾向于推荐一种个性化的方法。关于长期后续行动的准则仍然是一个辩论问题。
Background and objectives: Paragangliomas of the head and neck are rare, slow-growing neuroendocrine tumors, benign in their vast majority, but with a possibility of developing distant metastases. They show great inheritable character, and their behavior has proven to be unpredictable; therefore, they are considered malignant. Material and methods: This article aims to offer a more comprehensive presentation of the pathogenesis, epidemiology, diagnostic methods, imaging development, and treatment guidelines. We tried to bring together all the necessary data that, in our opinion, a head and neck practitioner should know when managing this type of tumor. Our main focus is on the most recent studies, with the purpose of a homogenous presentation of all current guidelines and approaches to this pathology. Results: Paragangliomas of the head and neck are still a disputed topic. One of the main reasons for that is their low incidence of 0.3 to 1 per 100,000 every year. The most frequent locations are the carotid body, the temporal bone, the jugular and mastoid foramen, and the vagal nerve. Their clinical presentation usually involves a painless lateral mass associated with symptoms such as hoarseness, hearing loss, tinnitus, and cranial nerve deficits. Up to 40% of them are inherited, mostly linked with mutations of succinate dehydrogenase complex. Imaging evaluation consists of CT and MRI, and new functional explorations such as 18F-FDA and 18F-FDG PET/CT, 18F-DOPA PET, 123I-MIBG, and 68Ga-DOTATE PET/CT. Measuring the catecholamine levels in the plasma and urine is mandatory, even though paragangliomas of the head and neck rarely display secretory behavior. Treatment mainly consists of surgery, with different approaches and techniques, but conservative management methods such as wait and scan, radiotherapy, proton therapy, and chemotherapy have proven their efficiency. The therapeutical decision lacks consensus, and current studies tend to recommend an individualized approach. Guidelines regarding long-term follow-up are still a matter of debate.