Department of Endocrinology, Key Laboratory of Endocrinology of National Health and Family Planning Commission, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, China.
Department of Neurosurgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
J Clin Endocrinol Metab. 2019 Aug 1;104(8):3419-3427. doi: 10.1210/jc.2018-02297.
To summarize the etiologies of pituitary stalk thickening (PST) and the natural course of indistinguishable PST.
Clinical information, including the symptoms at onset and laboratory, imaging, operative, pathological, and follow-up data, of patients with MRI-confirmed PST at Peking Union Medical College Hospital from January 2014 to May 2017 was collected and reviewed.
Of 321 eligible patients with PST, 28.3% were ≤18 years old. Central diabetes insipidus was the initial symptom in 68.8% of patients. At least one anterior pituitary hormone deficit was found in 57.6% of patients. The adjusted OR of panhypopituitarism associated with hypothalamus involvement was 7.3 (95% CI, 3.0 to 17.8; P < 0.001). Confirmed diagnoses were established in 137 patients (42.7%), including neoplasms (75.2%), inflammation (13.1%), and congenital anomalies (11.7%). Intracranial germ cell tumors (66.7%) were the leading cause among children, whereas histiocytoses (20.0%) and malignant metastases (14.7%) were the most common causes in adults. Thirty-eight patients with indistinguishable PST underwent a second MRI at a median time of 4.4 months. Spontaneous remission was observed in 17 of these patients (44.7%) after a median 8.5 months, with complete remission in 14 (36.8%) and partial remission in three (7.0%); five (13.2%) patients exhibited progression, and the remaining 16 (42.1%) stabilized.
PST is highly heterogeneous, and most confirmed cases are attributed to neoplasms. The etiological spectrum varies with age. Physicians must be familiar with the major differential diagnoses, necessary investigations, and follow-up. Biopsy is indicated when radiological progression and/or worsening of pituitary function is detected.
总结垂体柄增厚（PST）的病因和无法明确病因的 PST 的自然病程。
收集并回顾了 2014 年 1 月至 2017 年 5 月在北京协和医院经 MRI 证实的 PST 患者的临床资料，包括发病时的症状以及实验室、影像学、手术、病理和随访数据。
在 321 例符合条件的 PST 患者中，28.3%的患者年龄≤18 岁。68.8%的患者首发症状为中枢性尿崩症。57.6%的患者至少存在一种垂体前叶激素缺乏。伴有下丘脑受累的全垂体功能减退症与下丘脑受累相关的调整后 OR 为 7.3（95%CI，3.0 至 17.8；P<0.001）。在 137 例（42.7%）患者中确定了明确诊断，包括肿瘤（75.2%）、炎症（13.1%）和先天性异常（11.7%）。颅内生殖细胞瘤（66.7%）是儿童的主要病因，而组织细胞增多症（20.0%）和恶性转移（14.7%）是成人的最常见病因。38 例无法明确病因的 PST 患者在中位时间 4.4 个月后进行了第二次 MRI。在这些患者中，17 例（44.7%）在中位时间 8.5 个月后出现自发性缓解，其中 14 例（36.8%）完全缓解，3 例（7.0%）部分缓解；5 例（13.2%）患者病情进展，其余 16 例（42.1%）病情稳定。
PST 高度异质性，大多数明确诊断的病例归因于肿瘤。病因谱随年龄而变化。医生必须熟悉主要的鉴别诊断、必要的检查和随访。当发现影像学进展和/或垂体功能恶化时，应进行活检。
