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在中等收入国家治疗小儿颅内生殖细胞肿瘤的可行性:约旦的经验。

Feasibility of treating pediatric intracranial germ cell tumors in a middle-income country: The Jordanian experience.

机构信息

Division of Pediatric Hematology/Oncology, King Hussein Cancer Center, Amman, Jordan.

Radiation Oncology Department, King Hussein Cancer Center, Amman, Jordan.

出版信息

Pediatr Blood Cancer. 2022 Dec;69(12):e30011. doi: 10.1002/pbc.30011. Epub 2022 Sep 21.

Abstract

BACKGROUND

Pediatric intracranial germ cell tumors (iGCT) are rare, with limited data available from Arabic countries.

METHODS

We retrospectively reviewed the medical charts of children <18 years diagnosed with iGCT at King Hussein Cancer Center/Jordan (January 2003 to December 2020) for clinical characteristics, treatment, and morbidities.

RESULTS

Sixteen patients had germinoma; median age was 6.9 years and median symptoms duration 8 months. Nine tumors were suprasellar, five pineal, and two bifocal. Four were metastatic. Eight patients had slightly elevated beta subunit human chorionic gonadotropin and 11 patients had resection/biopsy. Fifteen patients received chemotherapy; mostly carboplatin (450 mg/m )/etoposide, which had low toxicity. All patients received radiotherapy (different doses and fields). At median follow-up of 7.7 years, one tumor recurred (progression-free survival: 91% ± 8%). Twelve patients who continued follow-up had stable visual and endocrine deficits to their initial presentation. Five finished or are finishing diploma and seven had poor school performance (four left school). Six patients were diagnosed with nongerminomatous germ cell tumor; median symptom duration was 1 month. Three tumors were pineal, two suprasellar, and one at quadrigeminal plate. Three were metastatic. Five tested patients had high tumor markers and four had resection/biopsy. All patients received chemotherapy, and then five received craniospinal radiation. Two patients are alive, two died with tumor progression, one died in remission with electrolyte imbalance, and one developed leukemia and died with septic shock.

CONCLUSIONS

We achieved excellent survival in treating germinoma using a feasible protocol for low middle-income countries. However, patients encountered significant morbidities exacerbated by delayed diagnosis and unnecessary surgical interventions despite abnormal tumor markers. Raising awareness on iGCT symptomatology and diagnosis may help limit these morbidities.

摘要

背景

小儿颅内生殖细胞肿瘤(iGCT)罕见,来自阿拉伯国家的数据有限。

方法

我们回顾性分析了 2003 年 1 月至 2020 年 12 月在侯赛因国王癌症中心/约旦诊断为 iGCT 的<18 岁儿童的病历,以了解其临床特征、治疗方法和并发症。

结果

16 例患者为生殖细胞瘤;中位年龄为 6.9 岁,中位症状持续时间为 8 个月。9 例肿瘤位于鞍上,5 例位于松果体,2 例位于双侧。4 例为转移性肿瘤。8 例患者β亚单位人绒毛膜促性腺激素略有升高,11 例患者行肿瘤切除/活检。15 例患者接受化疗;大多数采用卡铂(450mg/m )/依托泊苷,毒性较低。所有患者均接受放疗(不同剂量和野)。中位随访 7.7 年后,1 例肿瘤复发(无进展生存率:91%±8%)。12 例继续随访的患者初始表现出的视力和内分泌缺陷稳定。5 名患者完成或正在完成学业,7 名患者学习成绩较差(4 名辍学)。6 例诊断为非生殖细胞瘤生殖细胞肿瘤;中位症状持续时间为 1 个月。3 例肿瘤位于松果体,2 例位于鞍上,1 例位于四叠体板。3 例为转移性肿瘤。5 例肿瘤标志物升高的患者中有 4 例行肿瘤切除/活检。所有患者均接受化疗,然后 5 例患者接受全脑全脊髓放疗。2 例患者存活,2 例死于肿瘤进展,1 例死于缓解期电解质失衡,1 例发生白血病并死于感染性休克。

结论

我们采用适合中低收入国家的可行方案治疗生殖细胞瘤,取得了优异的生存结果。然而,尽管肿瘤标志物异常,由于诊断延迟和不必要的手术干预,患者仍出现了显著的并发症。提高对 iGCT 症状和诊断的认识可能有助于限制这些并发症。

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