Division of Anatomic Pathology, Department of Pathology and Laboratory Medicine, Vancouver, British Columbia.
Hum Pathol. 2012 Oct;43(10):1668-76. doi: 10.1016/j.humpath.2011.12.011. Epub 2012 Apr 10.
The factors that impact the long-term functional outcome for infants with brain tumor are unclear. The clinicopathologic features of all infant brain tumors occurring at our institution (1982-2005) were reexamined to explore the factors influencing prognosis. The details of the neuropathologic review are reported herein. Thirty-five cases were identified and included 7 astrocytomas (6 low grade and 1 glioblastoma), 6 atypical teratoid rhabdoid tumors, 5 choroid plexus papillomas, 4 ependymomas (3 anaplastic), 4 teratomas (3 immature), 2 supratentorial primitive neuroectodermal tumors, 2 gangliogliomas, 2 desmoplastic tumors of infancy, and 1 each of "medulloblastoma with extensive nodularity," adamantinomatous craniopharyngioma, and 1 "malignancy not otherwise specified." The original diagnosis was changed in 8 cases (23%), and atypical teratoid rhabdoid tumors was the most common revision (n = 5). Case 9 was unusual in that both the patient and her 2-year-old sister displayed INI-1 immunonegative posterior fossa tumors and extended survival. Tumor grade was altered in 6 cases (17%), the most significant instance being the downgrading from the World Health Organization grade IV to I (case 18: supratentorial primitive neuroectodermal tumors to desmoplastic tumors of infancy). As opposed to other reports in the literature, our cohort contained a substantially higher frequency of atypical teratoid rhabdoid tumors and a lower frequency of medulloblastoma. Changes in the histologic diagnosis/grade in a significant subset of cases most likely reflect the continual evolution of brain tumor classification schemes. INI-1 immunohistochemistry was instrumental in the pathologic assessment of select cases and raised the possibility that atypical teratoid rhabdoid tumors may be the most common infant brain malignancy.
脑肿瘤患儿长期功能预后的影响因素尚不清楚。为了探讨影响预后的因素，我们重新检查了我院（1982-2005 年）所有婴儿脑肿瘤的临床病理特征。本文报告了神经病理学检查的详细情况。共确定 35 例，包括 7 例星形细胞瘤（6 例低级别，1 例胶质母细胞瘤）、6 例非典型畸胎样横纹肌样瘤、5 例脉络丛乳头状瘤、4 例室管膜瘤（3 例间变性）、4 例畸胎瘤（3 例不成熟）、2 例幕上原始神经外胚层肿瘤、2 例神经节细胞瘤、2 例婴儿促纤维增生性肿瘤和 1 例“广泛结节性髓母细胞瘤”、造釉细胞瘤、1 例“未特指的恶性肿瘤”。8 例（23%）原始诊断发生改变，最常见的改变是非典型畸胎样横纹肌样瘤（n=5）。病例 9 很不寻常，患者及其 2 岁的妹妹均患有 INI-1 免疫阴性的后颅窝肿瘤且延长了生存期。肿瘤分级发生改变 6 例（17%），最显著的实例是从世界卫生组织分级 IV 级降为 I 级（病例 18：幕上原始神经外胚层肿瘤降为婴儿促纤维增生性肿瘤）。与文献中的其他报道不同，我们的队列中，非典型畸胎样横纹肌样瘤的频率明显较高，而髓母细胞瘤的频率较低。在相当一部分病例中，组织学诊断/分级的改变很可能反映了脑肿瘤分类方案的不断发展。INI-1 免疫组化在部分病例的病理评估中发挥了重要作用，并提出了非典型畸胎样横纹肌样瘤可能是最常见的婴儿脑恶性肿瘤的可能性。
