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儿童鞍内和第三脑室颅咽管瘤下丘脑 - 垂体轴功能障碍的比较

Comparison of hypothalamopituitary axis dysfunction of intrasellar and third ventricular craniopharyngiomas in children.

作者信息

Lee Yi-Yen, Wong Tai-Tong, Fang Yi-Ting, Chang Kai-Ping, Chen Yi-Wei, Niu Dau-Ming

机构信息

Department of Pediatrics, Taipei City Hospital, Renai Branch, NO.10, Sec. 4, Renai Road, Taipei 106, Taiwan, ROC.

出版信息

Brain Dev. 2008 Mar;30(3):189-94. doi: 10.1016/j.braindev.2007.07.011. Epub 2007 Sep 17.

Abstract

In this study, we attempted to determine if different locations of a tumor influence the hypothalamopituitary axis function and outcomes with childhood craniopharyngiomas. The preoperative, postoperative, and long-term follow-up endocrinological disturbances of 66 children with a craniopharyngioma were retrospectively studied. The patients were divided into two subgroups according to the location of the tumor (intrasellar and third ventricle floor). The mean age at onset was 8.02 (range, 1.42-17.58)years. These patients were followed-up for a median duration of 7.2 (range, 2-22)years. Vision problems as the first symptom were more common in Group One (with intrasellar tumors) compared to Group Two (55.6% vs 15.4%; p=0.001; Fisher's exact test). Increased intracranial pressure was the most common initial symptom in patients in Group Two (51.3%) and the second most common symptom in Group One (37%). The majority of patients in both Group One and Group Two required some forms of pituitary hormone supplements (96% vs 84%). At the last follow-up, more patients with intrasellar craniopharyngiomas needed cortisone supplements (79.2% in Group One vs 45.9% in Group Two; p=0.016; Fisher's exact test); however, children with third ventricle floor tumors had more prevalent weight gain (4.2% in Group One vs 27.0% in Group Two; p=0.038; Fisher's exact test). There were different initial presentations and endocrinological outcomes between children with intrasellar and third ventricle floor craniopharyngiomas. The intrasellar tumors had greater pituitary hormone disturbance. However, at the long-term follow-up, children with third ventricle floor tumors had a greater prevalence of being overweight and obese, which was associated with hypothalamic dysfunction.

摘要

在本研究中,我们试图确定肿瘤的不同位置是否会影响儿童颅咽管瘤的下丘脑 - 垂体轴功能及预后。我们回顾性研究了66例颅咽管瘤患儿术前、术后及长期随访的内分泌紊乱情况。根据肿瘤位置(鞍内和第三脑室底部)将患者分为两个亚组。发病时的平均年龄为8.02岁(范围1.42 - 17.58岁)。这些患者的中位随访时间为7.2年(范围2 - 22年)。与第二组相比,第一组(鞍内肿瘤)以视力问题为首发症状更为常见(55.6%对15.4%;p = 0.001;Fisher精确检验)。颅内压升高是第二组患者最常见的首发症状(51.3%),是第一组的第二常见症状(37%)。第一组和第二组的大多数患者都需要某种形式的垂体激素补充(96%对84%)。在最后一次随访时,鞍内颅咽管瘤患者中更多人需要补充皮质醇(第一组为79.2%,第二组为45.9%;p = 0.016;Fisher精确检验);然而,第三脑室底部肿瘤患儿体重增加更为普遍(第一组为4.2%,第二组为27.0%;p = 0.038;Fisher精确检验)。鞍内和第三脑室底部颅咽管瘤患儿的初始表现和内分泌预后不同。鞍内肿瘤的垂体激素紊乱更严重。然而,在长期随访中,第三脑室底部肿瘤患儿超重和肥胖的患病率更高,这与下丘脑功能障碍有关。

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