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小儿颅内畸胎瘤的治疗过程及预后:一项15年回顾性病例系列研究

Treatment Course and Outcomes of Intracranial Teratomas in Pediatric Patients: A Retrospective 15-Year Case Series Study.

作者信息

Wu Adela, Jin Michael C, Vogel Hannes, Hiniker Susan, Campen Cynthia, Prolo Laura M, Grant Gerald A

机构信息

Department of Neurosurgery, Stanford Health Care, Palo Alto, California, USA.

Stanford University School of Medicine, Palo Alto, California, USA.

出版信息

Pediatr Neurosurg. 2023;58(6):429-438. doi: 10.1159/000534721. Epub 2023 Oct 25.

Abstract

INTRODUCTION

There is no standard treatment paradigm for intracranial teratomas, a rare subset of primary intracranial non-germinomatous germ cell tumors (NGGCT), which comprise less than 1% of pediatric brain tumors. This case series retrospectively analyzes treatment and outcomes of pediatric intracranial teratomas from a single institution.

METHODS

Authors reviewed a comprehensive pathology database at Stanford's Lucile Packard Children's Hospital for intracranial teratomas in pediatric patients treated from 2006 to 2021; their demographics, treatment, and clinical course were analyzed.

RESULTS

Among 14 patients, median follow-up time was 4.6 years and mean age at diagnosis was 10.5 years. Ten had elevated tumor markers and underwent chemotherapy as initial treatment for NGGCT. Ultimately, these patients all required surgery for progressive or residual disease. Two patients did not undergo radiation. After biopsy or resection, 8 patients had pure mature teratoma, five had mixed germ cell tumor with teratoma component, and one had immature teratoma. The patient with immature teratoma died during chemotherapy from septic shock. No patients experienced recurrence. Common sequelae were endocrine (42.8%) and eye movement (50.0%) abnormalities.

DISCUSSION/CONCLUSION: We highlight the variable treatment course and outcome for pediatric patients with intracranial teratomas. Elevated tumor markers at presentation, along with imaging findings, favor chemotherapy initiation for presumed NGGCT. Resection of residual tumor is recommended even if tumor markers return to normal. Prognosis remains excellent; no patients had recurrence with a median follow-up of 4.6 years.

摘要

引言

颅内畸胎瘤是原发性颅内非生殖细胞瘤(NGGCT)的一个罕见亚型,占儿童脑肿瘤的比例不到1%,目前尚无标准的治疗模式。本病例系列回顾性分析了来自单一机构的儿童颅内畸胎瘤的治疗及预后情况。

方法

作者查阅了斯坦福大学露西尔·帕卡德儿童医院2006年至2021年期间治疗的儿童颅内畸胎瘤患者的综合病理数据库;分析了他们的人口统计学资料、治疗情况及临床病程。

结果

14例患者中,中位随访时间为4.6年,诊断时的平均年龄为10.5岁。10例患者肿瘤标志物升高,接受了化疗作为NGGCT的初始治疗。最终,这些患者均因疾病进展或残留而需要手术治疗。2例患者未接受放疗。活检或切除术后,8例患者为纯成熟畸胎瘤,5例为含畸胎瘤成分的混合性生殖细胞瘤,1例为未成熟畸胎瘤。未成熟畸胎瘤患者在化疗期间死于感染性休克。无患者出现复发。常见的后遗症为内分泌异常(42.8%)和眼球运动异常(50.0%)。

讨论/结论:我们强调了儿童颅内畸胎瘤患者治疗过程及预后的多样性。就诊时肿瘤标志物升高以及影像学表现提示应启动化疗以治疗疑似NGGCT。即使肿瘤标志物恢复正常,也建议切除残留肿瘤。预后仍然良好;中位随访4.6年,无患者复发。

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