Mediouni A, Ammari S, Wassef M, Gimenez-Roqueplo A-P, Laredo J-D, Duet M, Tran Ba Huy P, Oker N
Service de médecine nucléaire, hôpital Lariboisière (Assistance Publique-Hôpitaux de Paris), 2, rue Ambroise-Paré, 75010 Paris, France.
Service de cytologie et d'anatomie pathologique, hôpital Lariboisière (Assistance Publique-Hôpitaux de Paris), 2, rue Ambroise-Paré, 75010 Paris, France; Université Paris-Diderot Paris-7, 75205 Paris cedex 13, France.
Eur Ann Otorhinolaryngol Head Neck Dis. 2014 Jun;131(3):159-66. doi: 10.1016/j.anorl.2013.05.003. Epub 2013 Nov 14.
The objective of this study was to report 11 cases of malignant head and neck paraganglioma and to compare their epidemiological, clinical, and genetic characteristics, their natural history and their treatment with those of a series of 131 benign paragangliomas.
Retrospective analysis of 142 patients with head and neck paraganglioma managed between 2001 and 2008. Age at the time of diagnosis, gender, primary tumour site, presence of other non-head/neck paragangliomas and/or metastases diagnosed by imaging (CT, MRI, Octreoscan or (18)F-FDG PET), histology, urinary catecholamine and metanephrine levels, family history, and genetic test results were recorded.
This series comprised 131 benign head and neck paragangliomas, mostly observed in women with a mean age at diagnosis of 45 years and a predominance of tympanojugular sites (followed by carotid and vagal sites) with 5% of secreting tumours and 20% of multifocal tumours. Eleven patients (7.7%) with a 1:1 sex ratio presented criteria of malignancy. These patients, with a lower mean age (38 years), predominantly presented carotid lesions with a higher rate of secreting and multifocal tumours, 27% and 46% respectively. The main sites of metastases were bone and lymph nodes. No tympanic paragangliomas were observed.
Malignant paragangliomas are mainly observed in young patients with multifocal tumours, particularly carotid tumours, and are predominantly related to subunit SDH-B mutation. The work-up in these high-risk patients must include whole body scintigraphy and spine MRI. Malignancy is not necessarily associated with a poor short-term prognosis due to the slow course of the disease.
本研究的目的是报告11例恶性头颈部副神经节瘤，并将其流行病学、临床和遗传学特征、自然病史及治疗情况与131例良性副神经节瘤进行比较。
对2001年至2008年间诊治的142例头颈部副神经节瘤患者进行回顾性分析。记录诊断时的年龄、性别、原发肿瘤部位、通过影像学检查（CT、MRI、奥曲肽扫描或（18）F-FDG PET）诊断的其他非头颈部副神经节瘤和/或转移灶、组织学、尿儿茶酚胺和甲氧基肾上腺素水平、家族史及基因检测结果。
该系列包括131例良性头颈部副神经节瘤，多见于女性，诊断时平均年龄为45岁，主要位于鼓室颈静脉区（其次为颈动脉和迷走神经区），5%为分泌型肿瘤，20%为多灶性肿瘤。11例患者（7.7%）符合恶性标准，男女比例为1:1。这些患者平均年龄较低（38岁），主要表现为颈动脉病变，分泌型和多灶性肿瘤发生率较高，分别为27%和46%。主要转移部位为骨和淋巴结。未观察到鼓室副神经节瘤。
恶性副神经节瘤主要见于患有多灶性肿瘤的年轻患者，尤其是颈动脉肿瘤，主要与亚基SDH-B突变有关。这些高危患者的检查必须包括全身闪烁扫描和脊柱MRI。由于疾病进展缓慢，恶性并不一定与短期预后不良相关。
