Moyes V J, Alusi G, Sabin H I, Evanson J, Berney D M, Kovacs K, Monson J P, Plowman P N, Drake W M
Department of Endocrinology, St Bartholomew's Hospital, London, UK.
Eur J Endocrinol. 2009 Jan;160(1):115-9. doi: 10.1530/EJE-08-0557. Epub 2008 Nov 4.
A 64-year-old woman was previously treated for Cushing's disease with trans-sphenoidal surgery, external beam radiotherapy and bilateral adrenalectomy. Progression of an aggressive corticotroph adenoma was evident 3 years post-adrenalectomy; involvement of the clivus was treated with surgery and gamma knife radiosurgery. Tumour spread through the skull base, occiput and left ear with persistent facial pain and left ear discharge; progression continued despite second gamma knife treatment. ACTH levels peaked at 2472 and 2265 pmol/l pre- and post-hydrocortisone respectively. Treatment with temozolomide resulted in a significant improvement in symptoms, a reduction of plasma ACTH to 389 pmol/l and regression of tumour on magnetic resonance imaging scan after four cycles of treatment. We propose that temozolomide is an effective and well-tolerated therapeutic tool for the treatment of Nelson's syndrome and a useful addition to the range of therapies available to treat this condition.
一名64岁女性曾接受经蝶窦手术、外照射放疗和双侧肾上腺切除术治疗库欣病。肾上腺切除术后3年，侵袭性促肾上腺皮质激素腺瘤进展明显；斜坡受累通过手术和伽玛刀放射外科治疗。肿瘤扩散至颅底、枕骨和左耳，伴有持续性面部疼痛和左耳流脓；尽管进行了第二次伽玛刀治疗，病情仍继续进展。促肾上腺皮质激素水平在氢化可的松治疗前和治疗后分别达到峰值2472和2265 pmol/l。替莫唑胺治疗使症状显著改善，血浆促肾上腺皮质激素降至389 pmol/l，且在四个疗程治疗后磁共振成像扫描显示肿瘤消退。我们认为替莫唑胺是治疗纳尔逊综合征的一种有效且耐受性良好的治疗工具，是治疗该疾病现有治疗方法的有益补充。
