Braun J, Schuldes H, Berkefeld J, Zanella F, Jonas D, Usadel K H, Badenhoop K
Medizinische Klinik I, Schwerpunkt Endokrinologie, Klinikum der J.W. Goethe-Universität, Frankfurt am Main, Germany.
Clin Endocrinol (Oxf). 2001 Feb;54(2):273-6. doi: 10.1046/j.1365-2265.2001.01023.x.
A 43-year-old man, with a history of central diabetes insipidus diagnosed 3 years previously, complained about reduced libido. An MRI scan showed a suprasellar lesion just below the supraoptic recess of the third ventricle. A stereotactically guided biopsy revealed fibrous glia, but no other specific tissue and no inflammatory cells. Two months later the patient presented with fatigue and muscular weakness. Tertiary adrenal failure and hypothyroidism were diagnosed by endocrine function tests and therapy with levothyroxine and hydrocortisone was started. Another 2 months later the patient was admitted with giddiness, nausea, peripheral oedema and oliguria. Radiological imaging and an open transperitoneal kidney exploration showed severe fibrosis around both ureters. Histological examination confirmed the diagnosis of idiopathic retroperitoneal fibrosis. Presumably the suprasellar tumour was the first manifestation of retroperitoneal fibrosis. Once the diagnosis 'idiopathic retroperitoneal fibrosis' is confirmed, fibrotic manifestations and complications involving extra-retroperitoneal tissues including the endocrine system, should be sought.
一名43岁男性，3年前被诊断为中枢性尿崩症，现主诉性欲减退。磁共振成像（MRI）扫描显示第三脑室视交叉上隐窝下方有一个鞍上病变。立体定向引导活检显示为纤维性胶质，但无其他特定组织且无炎症细胞。两个月后，患者出现疲劳和肌肉无力。通过内分泌功能检查诊断为三发性肾上腺功能减退和甲状腺功能减退，并开始使用左甲状腺素和氢化可的松治疗。又过了两个月，患者因头晕、恶心、外周水肿和少尿入院。影像学检查和开放性经腹肾脏探查显示双侧输尿管周围严重纤维化。组织学检查确诊为特发性腹膜后纤维化。推测鞍上肿瘤是腹膜后纤维化的首发表现。一旦确诊为“特发性腹膜后纤维化”，就应寻找涉及包括内分泌系统在内的腹膜外组织的纤维化表现和并发症。
