Della Casa S, Corsello S M, Satta M A, Rota C A, Putignano P, Vangeli V, Colosimo C, Anile C, Barbarino A
Institute of Endocrinology, Catholic University School of Medicine, Rome, Italy.
Ann Endocrinol (Paris). 1997;58(6):503-9.
We report a case of a 52-year-old woman presenting with a recurrence of a large pituitary adenoma with suprasellar extension and an overt Cushing's clinical picture, five years after successful transsphenoidal treatment. After transfrontal ablation of the tumour, followed by external radiotherapy, she was asymptomatic for six years before she exhibited epileptic seizures. A left frontal intracranial neoplasm was diagnosed and removed, and at histological examination it was found to be constituted by a localization of the pituitary ACTH secreting neoplasia. One month later she exhibited spinal dissemination of the ACTH secreting neoplasia which was only partially removed. After four months a Magnetic Resonance Image (MRI) revealed recurrence of the intracranial localization and further spinal dissemination. Because of compressive symptoms, spinal masses with the same histologic features, were partially removed again in three successive surgical operations. Several medical treatments for obtaining the control of corticoid excess, caused by the ACTH overproduction, were tried, but none were satisfactory. Finally a bilateral adrenal venous embolization was performed thus obtaining a critical transient fall of serum cortisol. Five months later the patient died. At necroscopy bilateral adrenal enlargement was found, spinal disseminations were confirmed, and no metastatic lesions were discovered.
我们报告一例52岁女性患者，在经蝶窦成功治疗五年后，出现鞍上扩展的大型垂体腺瘤复发，并伴有明显的库欣临床表现。经额部肿瘤切除并进行外照射放疗后，她在出现癫痫发作前六年一直无症状。诊断并切除了左侧额叶颅内肿瘤，组织学检查发现其由垂体促肾上腺皮质激素分泌肿瘤的定位构成。一个月后，她出现促肾上腺皮质激素分泌肿瘤的脊髓播散，肿瘤仅部分切除。四个月后，磁共振成像（MRI）显示颅内肿瘤复发并进一步脊髓播散。由于压迫症状，在连续三次手术中又部分切除了具有相同组织学特征的脊髓肿块。尝试了几种用于控制促肾上腺皮质激素过度分泌引起的皮质醇过多的药物治疗，但均不理想。最后进行了双侧肾上腺静脉栓塞，从而使血清皮质醇出现关键性短暂下降。五个月后患者死亡。尸检发现双侧肾上腺肿大，证实有脊髓播散，未发现转移病灶。
