Maier W, Marangos N, Laszig R
Universitäts-Hals-Nasen-Ohren-Klinik, Freiburg, Germany.
J Laryngol Otol. 1999 Nov;113(11):978-82. doi: 10.1017/s0022215100145761.
Tumours of the neuroendocrine system in the head and neck region are mostly paragangliomas of the glomus tympanicum or jugulare, or of the carotid body. The majority of these tumours are benign, and the coexistence of multiple paragangliomas seems to be rare. Pre-operative embolization and surgery are regarded as primary therapy for these tumours. The treatment regimen in any patient depends on age, general health, hearing status and the function of the lower cranial nerves. Several presentations are possible in which paragangliomas occur as systemic disease. 1. Paragangliomas may occur bilaterally, or, in rare cases, in multiple areas. Pre-operative bilateral angiography is of utmost importance. In case of multicentricity, it might be necessary to proceed without, or just with, unilateral surgery for preservation of adjacent structures. In surgery of jugular vein paraganglioma, we usually perform a modified transmastoidal and transcervical approach with preservation of middle-ear structures and the ossicles. As an alternative or supplement to surgery, radiotherapy or definitive embolization may be used in the treatment of paragangliomas. 2. Paragangliomas may occur as multiple endocrine neoplasia (MEN) syndrome combined with medullary thyroid gland carcinoma, and, facultatively, pheochromocytoma. In these cases, endocrinological examination and magnetic resonance imaging (MRI) of the adrenal region, the thorax and the neck are required for an adequate therapeutic strategy. As MEN may be inherited, family history should be evaluated. 3. Paragangliomas can became malignant and metastasize. Thus, cervical lymph node metastases or distant metastases may occur. We recommend the removal of all ipsilateral lymph nodes and their histological examination.
头颈部神经内分泌系统肿瘤大多为鼓室球瘤、颈静脉球瘤或颈动脉体瘤。这些肿瘤大多为良性，多发副神经节瘤共存似乎较为罕见。术前栓塞和手术被视为这些肿瘤的主要治疗方法。任何患者的治疗方案都取决于年龄、总体健康状况、听力状况以及下颅神经功能。副神经节瘤作为全身性疾病可能有几种表现形式。1. 副神经节瘤可能双侧发生，或在罕见情况下，发生于多个部位。术前双侧血管造影至关重要。如果是多中心性病变，可能有必要仅进行单侧手术或不进行手术以保留相邻结构。在颈静脉球瘤手术中，我们通常采用改良经乳突和经颈入路，保留中耳结构和听小骨。作为手术的替代或补充治疗，放射治疗或确定性栓塞可用于副神经节瘤的治疗。2. 副神经节瘤可能作为多发性内分泌肿瘤（MEN）综合征与甲状腺髓样癌合并，并且可能合并嗜铬细胞瘤。在这些情况下，需要进行肾上腺区域、胸部和颈部的内分泌检查及磁共振成像（MRI）以制定适当的治疗策略。由于MEN可能是遗传性的，应评估家族史。3. 副神经节瘤可发生恶变并转移。因此，可能会出现颈部淋巴结转移或远处转移。我们建议切除所有同侧淋巴结并进行组织学检查。
