Daubenbüchel A M M, Hoffmann A, Gebhardt U, Warmuth-Metz M, Sterkenburg A S, Müller H L
Department of PediatricsKlinikum Oldenburg, Medical Campus University Oldenburg, Rahel-Straus-Strasse 10, 26133 Oldenburg, GermanyUniversity of GroningenGroningen, The NetherlandsDepartment of NeuroradiologyUniversity Hospital, Würzburg, Germany Department of PediatricsKlinikum Oldenburg, Medical Campus University Oldenburg, Rahel-Straus-Strasse 10, 26133 Oldenburg, GermanyUniversity of GroningenGroningen, The NetherlandsDepartment of NeuroradiologyUniversity Hospital, Würzburg, Germany.
Department of PediatricsKlinikum Oldenburg, Medical Campus University Oldenburg, Rahel-Straus-Strasse 10, 26133 Oldenburg, GermanyUniversity of GroningenGroningen, The NetherlandsDepartment of NeuroradiologyUniversity Hospital, Würzburg, Germany.
Eur J Endocrinol. 2015 May;172(5):561-9. doi: 10.1530/EJE-14-1029. Epub 2015 Feb 3.
Pediatric patients with sellar masses such as craniopharyngioma (CP) or cyst of Rathke's pouch (CRP) frequently suffer disease- and treatment-related sequelae. We analyzed the impact and prognostic relevance of initial hydrocephalus (HY) and hypothalamic involvement (HI) on long-term survival and functional capacity (FC) in children with CP or CRP.
Using retrospective analysis of patient records, presence of initial HY or HI was assessed in 177 pediatric patients (163 CP and 14 CRP). Twenty-year overall survival (OS) and progression-free survival (PFS), FC, and BMI were analyzed with regard to initial HY, degree of resection, or HI.
Of the 177 patients, 105 patients (103/163 CP and 2/14 CRP) presented with initial HY and 96 presented with HI. HY at diagnosis was associated (P=0.000) with papilledema, neurological deficits, and higher BMI at diagnosis and during follow-up. OS, PFS, and FC were not affected by HY at initial diagnosis. HI at diagnosis (96/177) had major negative impact on long-term prognosis. Sellar masses with HI were associated with lower OS (0.84±0.04; P=0.021), lower FC (P=0.003), and higher BMI at diagnosis and last follow-up (P=0.000) when compared with sellar masses without HI (OS: 0.94±0.05). PFS was not affected by HI or degree of resection.
Initial HY has no impact on outcome in patients with sellar masses. OS and FC are impaired in survivors presenting with initial HI. PFS is not affected by HY, HI, or degree of resection. Accordingly, gross-total resection is not recommended in sellar masses with initial HI to prevent further hypothalamic damage.
患有鞍区肿物（如颅咽管瘤（CP）或拉克氏囊肿（CRP））的儿科患者经常遭受与疾病和治疗相关的后遗症。我们分析了初始脑积水（HY）和下丘脑受累（HI）对CP或CRP患儿长期生存及功能能力（FC）的影响和预后相关性。
通过对患者记录进行回顾性分析，评估了177例儿科患者（163例CP和14例CRP）初始HY或HI的存在情况。分析了初始HY、切除程度或HI与20年总生存（OS）、无进展生存（PFS）、FC及体重指数（BMI）的关系。
177例患者中，105例（103/163例CP和2/14例CRP）存在初始HY，96例存在HI。诊断时的HY与视乳头水肿、神经功能缺损以及诊断时和随访期间较高的BMI相关（P = 0.000）。初始诊断时的HY不影响OS、PFS和FC。诊断时的HI（96/177）对长期预后有重大负面影响。与无HI的鞍区肿物相比（OS：0.94±0.05），伴有HI的鞍区肿物的OS较低（0.84±0.04；P = 0.021）、FC较低（P = 0.003），且诊断时和末次随访时BMI较高（P = 0.000）。PFS不受HI或切除程度的影响。
初始HY对鞍区肿物患者的预后无影响。初始HI的幸存者的OS和FC受损。PFS不受HY、HI或切除程度的影响。因此，对于初始HI的鞍区肿物，不建议进行全切除以防止进一步的下丘脑损伤。
