Al-Hussaini Maysa, Swaidan Maisa, Al-Jumaily Usama, Musharbash Awni
Department of Pathology, King Hussein Cancer Center, Queen Rania Al Abdullah Street, P.O. Box 1269, Al-Jubeiha, Amman, 11941, Jordan,
Childs Nerv Syst. 2013 Oct;29(10):1883-91. doi: 10.1007/s00381-013-2081-0. Epub 2013 Mar 24.
This study aims to review our experience with central nervous system (CNS) tumors occurring during the first year of life and to report differing features found in our series.
This is a retrospective study of infants with CNS tumors diagnosed at our institution from 2006 to 2011.
A total of 19 cases were identified, with a median age of 232 days and predominance of male gender. Males were younger than females at the time of diagnosis (p value = 0.039). There were 13 low-grade tumors, glial tumors being the most common (11/13, p value = 0.003) and six high-grade tumors, atypical teratoid rhabdoid tumor being the most common (4/6). Low-grade tumors predominated in the supratentorial region, while high-grade tumors were seen in the infratentorial area (p value = 0.035). Males had a predilection to have more supratentorial tumors (p value = 0.058). Four patients underwent gross total resection, and eight received chemotherapy; none received radiotherapy. Two patients had spinal cord tumors; both were of pilomyxoid astrocytoma histology. Rare tumors included hemangiopericytoma (n = 1) and atypical choroid plexus tumor (n = 1), both occurring in the supratentorial area and affecting the youngest patients in this group; they were diagnosed prenatally and at 107 days, respectively. The median progression-free and overall survivals were 269 and 667 days, respectively. Among all tested parameters, only the grade of the tumor affected the outcome.
Diagnosis and management of infant's CNS tumors remain challenging. Pathologists should be aware of the diversity of histological types. Assigning appropriate tumor grade is fundamental in predicting the outcome.
本研究旨在回顾我们在出生后第一年发生的中枢神经系统（CNS）肿瘤方面的经验，并报告我们系列研究中发现的不同特征。
这是一项对2006年至2011年在我们机构诊断为CNS肿瘤的婴儿进行的回顾性研究。
共确定19例，中位年龄为232天，男性占优势。诊断时男性比女性年龄小（p值 = 0.039）。有13例低级别肿瘤，其中神经胶质瘤最为常见（11/13，p值 = 0.003），6例高级别肿瘤，非典型畸胎样横纹肌样瘤最为常见（4/6）。低级别肿瘤在幕上区域占主导，而高级别肿瘤见于幕下区域（p值 = 0.035）。男性更倾向于有更多幕上肿瘤（p值 = 0.058）。4例患者接受了全切术，8例接受了化疗；均未接受放疗。2例患者有脊髓肿瘤；两者均为毛黏液样星形细胞瘤组织学类型。罕见肿瘤包括血管外皮细胞瘤（n = 1）和非典型脉络丛肿瘤（n = 1），均发生在幕上区域，且是该组中年龄最小的患者；它们分别在产前和107天时被诊断出来。无进展生存期和总生存期的中位数分别为269天和667天。在所有测试参数中，只有肿瘤级别影响预后。
婴儿中枢神经系统肿瘤的诊断和管理仍然具有挑战性。病理学家应了解组织学类型多样性。确定合适的肿瘤级别是预测预后的基础。
