Henry J F, Audiffret J, Denizot A
Unité de Chirurgie Endocrinienne, CHU de la Timone, Marseille.
J Urol (Paris). 1989;95(5):301-4.
In a series of 82 pheochromocytomas operated on, the authors report 8 cases of Sipple's syndrome, currently classified by Steiner among the type II multiple endocrine neoplasia (MEN II). Six patients were operated on, the mean follow up being 4 years. These patients all underwent total thyroidectomy and 4 of them total bilateral adrenalectomy. Gluco and mineralocorticoid and thyroid hormone replacement therapy was instituted. No clinical or biological recurrence has been noted. The ideal therapeutic schema is difficult to define given the rarity of MEN II.
在一组82例接受手术治疗的嗜铬细胞瘤患者中,作者报告了8例西普尔综合征病例,目前施泰纳将其归类为II型多发性内分泌肿瘤(MEN II)。6例患者接受了手术,平均随访时间为4年。这些患者均接受了甲状腺全切术,其中4例接受了双侧肾上腺全切术。开始了糖皮质激素、盐皮质激素和甲状腺激素替代治疗。未发现临床或生物学复发。鉴于MEN II的罕见性,理想的治疗方案难以确定。