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低收入和中等收入国家小儿颅咽管瘤患者的管理与发病率回顾:12年经验

Review of management and morbidity of pediatric craniopharyngioma patients in a low-middle-income country: a 12-year experience.

作者信息

Amayiri Nisreen, Swaidan Maisa, Yousef Yocoub, Halalsheh Hadeel, Abu-Hijlih Ramiz, Kalaldeh Sima, Barbar Maha, Elayyan Maher, Faqih Nesreen, Al-Hussaini Maysa, Mehyar Mustafa, Bartels Ute, Drake James, Musharbash Awni, Bouffet Eric

机构信息

Pediatric Department, King Hussein Cancer Center, 202 Queen, Rania Al-abdullah Street, Al-Jubeiha, P.O. Box 1269, Amman, 11941, Jordan.

Radiology Department, King Hussein Cancer Center, Amman, Jordan.

出版信息

Childs Nerv Syst. 2017 Jun;33(6):941-950. doi: 10.1007/s00381-017-3411-4. Epub 2017 Apr 28.

Abstract

BACKGROUND

Management of craniopharyngioma in children is challenging, and their quality of life can be significantly affected. Series describing this from low-middle income countries (LMIC) are few.

PATIENTS AND METHODS

The study provides a retrospective chart review of pediatric patients <18 years old, diagnosed with craniopharyngioma between 2003 and 2014, and treated at King Hussein Cancer Center, Jordan.

RESULTS

Twenty-four patients (12 males) were identified. Median age at diagnosis was 7.4 years (0.9-16.4 years). Commonest symptoms were visual impairment and headache (71%). Review of seventeen preoperative MRIs showed hypothalamic involvement in 88% and hydrocephalus in 76%. Thirteen patients (54%) had multiple surgical interventions. Five patients (21%) had initial gross total resection. Eleven patients (46%) received radiotherapy and six (25%) intra-cystic interferon. Five years' survival was 87 ± 7% with a median follow-up of 4.5 years (0.3-12.3 years). Four patients (17%) died; one after post-operative cerebral infarction and three secondary to hypothalamic damage. At their last evaluation, all but one patient required multiple hormonal supplements. Ten patients (42%) had best eye visual acuity (VA) >20/40, and four (16%) were legally blind. Eleven patients (46%) were overweight/obese; one had gastric bypass surgery. Seven patients had hyperlipidemia, and eight developed fatty liver infiltration. Eleven patients (65%) were attending schools and one at college. Nine of the living patients (53%) expressed difficulty to engage in the community.

CONCLUSIONS

Management of pediatric craniopharyngioma is particularly complex and demanding in LMIC. Multidisciplinary care is integral to optimize the care and minimize the morbidities. A management outline for LMIC is proposed.

摘要

背景

儿童颅咽管瘤的治疗具有挑战性,其生活质量会受到显著影响。来自低收入和中等收入国家(LMIC)描述这一情况的系列报道较少。

患者与方法

本研究对2003年至2014年间在约旦侯赛因国王癌症中心接受治疗的18岁以下诊断为颅咽管瘤的儿科患者进行了回顾性病历审查。

结果

共确定了24例患者(12例男性)。诊断时的中位年龄为7.4岁(0.9 - 16.4岁)。最常见的症状是视力障碍和头痛(71%)。对17例术前MRI的检查显示,88%的患者下丘脑受累,76%的患者有脑积水。13例患者(54%)接受了多次手术干预。5例患者(21%)首次实现了肿瘤全切。11例患者(46%)接受了放疗,6例(25%)接受了囊内注射干扰素。5年生存率为87±7%,中位随访时间为4.5年(0.3 - 12.3年)。4例患者(17%)死亡;1例死于术后脑梗死,3例死于下丘脑损伤。在最后一次评估时,除1例患者外,所有患者都需要多种激素补充。10例患者(42%)的最佳视力(VA)>20/40,4例(16%)为法定失明。11例患者(46%)超重/肥胖;1例接受了胃旁路手术。7例患者有高脂血症,8例出现脂肪肝浸润。11例患者(65%)在上学,1例在上大学。9例在世患者(53%)表示难以融入社区。

结论

在低收入和中等收入国家,儿童颅咽管瘤的治疗尤为复杂且要求高。多学科护理对于优化治疗和将发病率降至最低至关重要。本文提出了一份针对低收入和中等收入国家的管理大纲。

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