Nguyen D Q, Boulat E, Troussier J, Reyt E I, Lavieille J P, Schmerber S I
CHU Grenoble, Service ORL, F-38043 Grenoble cedex 09, France.
Rev Laryngol Otol Rhinol (Bord). 2005;126(1):7-13.
The jugulotympanic paragangliomas (JTP) represents the most frequent tumour of the middle ear but also of the temporal bone, after the acoustic neurinoma. The management of these vascular tumours remains uncleared. The purpose of this study was to report our experience about JTP in the CHU of Grenoble.
Retrospective study of 41 patients, between 1973 and 1996. Six stages A, 8 stages B and 27 stages C are reported in whom 20 cases (49%) presented an intracranial extension (classification of Fisch). There were 2 familial cases with multiple localisations, in particular carotid. All the patients were divided in 3 groups: surgery or radiation therapy in first intention, surgery followed by radiation therapy.
A total tumor removal without recurrence was achieved by surgery in more than 95% of the cases with 6 years follow-up but was associated with significant morbidity (major cranial nerve injury). We noticed one death by laryngospasme (C2Di2 tumour operated by infratemporal A approach). A stabilization of the tumour was obtained with radiotherapy in first intention in 75% of the cases (5 years follow-up) but with a risk of radionecrosis. A revision surgery was necessary in 3 cases.
The comparaison of our different therapeutic management, surgery (23), radiation therapy (16) or combined (2), encourage us to perform a radical surgery whenever possible. Because of the slow rate of growth, the radiotherapy is indicated for older patients, at risk for surgery or extensive tumors. The objectives of the radiation therapy are to obtain a tumoral stabilization with improvement of the symptoms and low morbidity. The management of this rare pathology must be multidisciplinary. The recent discoveries on genes encoding three succinate dehydrogenase subunits (SDHD, SDHB et SDHC) will allow a genetic detection of asymptomatic case and will define the procedures for their management, coordinated by a national network PGL.NET. A retrospective study could also study the real incidence of familial paragangliomas.
颈静脉鼓室副神经节瘤（JTP）是中耳最常见的肿瘤，也是继听神经瘤之后颞骨最常见的肿瘤。这些血管性肿瘤的治疗方法仍不明确。本研究的目的是报告我们在格勒诺布尔大学医院（CHU）治疗JTP的经验。
对1973年至1996年间的41例患者进行回顾性研究。报告了6例A期、8例B期和27例C期患者，其中20例（49%）出现颅内扩展（Fisch分类）。有2例家族性病例出现多处病变，尤其是颈动脉处。所有患者分为3组：首选手术或放疗、手术后继以放疗。
超过95%的病例通过手术实现了肿瘤完全切除且6年随访无复发，但伴有显著的并发症（主要为颅神经损伤）。我们注意到1例因喉痉挛死亡（经颞下A入路手术的C2Di2肿瘤）。75%的病例首选放疗可使肿瘤稳定（5年随访），但有放射性坏死风险。3例患者需要进行翻修手术。
我们对不同治疗方法（手术23例、放疗16例或联合治疗2例）的比较，鼓励我们尽可能进行根治性手术。由于肿瘤生长缓慢，放疗适用于老年患者、有手术风险或肿瘤广泛的患者。放疗的目标是实现肿瘤稳定，改善症状并降低并发症发生率。这种罕见疾病的治疗必须是多学科的。最近关于编码三种琥珀酸脱氢酶亚基（SDHD、SDHB和SDHC）的基因的发现，将有助于对无症状病例进行基因检测，并确定其管理程序，由国家PGL.NET网络协调。一项回顾性研究也可以研究家族性副神经节瘤的实际发病率。
