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颅咽管瘤的极长期后遗症。

Very long-term sequelae of craniopharyngioma.

作者信息

Wijnen Mark, van den Heuvel-Eibrink Marry M, Janssen Joseph A M J L, Catsman-Berrevoets Coriene E, Michiels Erna M C, van Veelen-Vincent Marie-Lise C, Dallenga Alof H G, van den Berge J Herbert, van Rij Carolien M, van der Lely Aart-Jan, Neggers Sebastian J C M M

机构信息

Section EndocrinologyDepartment of Medicine, Pituitary Centre Rotterdam, Erasmus University Medical Centre, Rotterdam, the Netherlands.

Department of Paediatric Oncology/HaematologyErasmus MC - Sophia Children's Hospital, Rotterdam, the Netherlands.

出版信息

Eur J Endocrinol. 2017 Jun;176(6):755-767. doi: 10.1530/EJE-17-0044. Epub 2017 Mar 21.

Abstract

OBJECTIVE

Studies investigating long-term health conditions in patients with craniopharyngioma are limited by short follow-up durations and generally do not compare long-term health effects according to initial craniopharyngioma treatment approach. In addition, studies comparing long-term health conditions between patients with childhood- and adult-onset craniopharyngioma report conflicting results. The objective of this study was to analyse a full spectrum of long-term health effects in patients with craniopharyngioma according to initial treatment approach and age group at craniopharyngioma presentation.

DESIGN

Cross-sectional study based on retrospective data.

METHODS

We studied a single-centre cohort of 128 patients with craniopharyngioma treated from 1980 onwards (63 patients with childhood-onset disease). Median follow-up since craniopharyngioma presentation was 13 years (interquartile range: 5-23 years). Initial craniopharyngioma treatment approaches included gross total resection ( = 25), subtotal resection without radiotherapy ( = 44), subtotal resection with radiotherapy ( = 25), cyst aspiration without radiotherapy ( = 8), and Yttrium brachytherapy ( = 21).

RESULTS

Pituitary hormone deficiencies (98%), visual disturbances (75%) and obesity (56%) were the most common long-term health conditions observed. Different initial craniopharyngioma treatment approaches resulted in similar long-term health effects. Patients with childhood-onset craniopharyngioma experienced significantly more growth hormone deficiency, diabetes insipidus, panhypopituitarism, morbid obesity, epilepsy and psychiatric conditions compared with patients with adult-onset disease. Recurrence-/progression-free survival was significantly lower after initial craniopharyngioma treatment with cyst aspiration compared with other therapeutic approaches. Survival was similar between patients with childhood- and adult-onset craniopharyngioma.

CONCLUSIONS

Long-term health conditions were comparable after different initial craniopharyngioma treatment approaches and were generally more frequent in patients with childhood- compared with adult-onset disease.

摘要

目的

关于颅咽管瘤患者长期健康状况的研究受随访时间短的限制,且通常未根据初始颅咽管瘤治疗方法比较长期健康影响。此外,比较儿童期和成年期发病的颅咽管瘤患者长期健康状况的研究结果相互矛盾。本研究的目的是根据初始治疗方法和颅咽管瘤发病时的年龄组,分析颅咽管瘤患者全面的长期健康影响。

设计

基于回顾性数据的横断面研究。

方法

我们研究了1980年起在单中心接受治疗的128例颅咽管瘤患者队列(63例儿童期发病患者)。自颅咽管瘤发病后的中位随访时间为13年(四分位间距:5 - 23年)。初始颅咽管瘤治疗方法包括全切除(n = 25)、未行放疗的次全切除(n = 44)、行放疗的次全切除(n = 25)、未行放疗的囊肿抽吸(n = 8)和钇近距离放疗(n = 21)。

结果

垂体激素缺乏(98%)、视觉障碍(75%)和肥胖(56%)是观察到的最常见的长期健康状况。不同的初始颅咽管瘤治疗方法导致相似的长期健康影响。与成年期发病的患者相比,儿童期发病的颅咽管瘤患者生长激素缺乏、尿崩症、全垂体功能减退、病态肥胖、癫痫和精神疾病更为常见。与其他治疗方法相比,初始采用囊肿抽吸治疗颅咽管瘤后的无复发/进展生存率显著更低。儿童期和成年期发病的颅咽管瘤患者生存率相似。

结论

不同的初始颅咽管瘤治疗方法后的长期健康状况具有可比性,且与成年期发病的患者相比,儿童期发病的患者中这些状况通常更常见。

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