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