颅内肿瘤在生命的第一年。

José Francisco M Salomão, Tatiana Protzenko
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摘要

颅内肿瘤在生命的第一年是罕见的,在这个年龄组中,是仅次于白血病的第二常见的儿科癌症类型。作为新生儿和婴幼儿最常见的实体瘤,其具有恶性肿瘤发生率高的特点。常规超声检查更容易发现子宫内肿瘤,但由于缺乏或缺乏可识别的症状,诊断可能会延迟。这些肿瘤通常很大,血管密集。切除它们是具有挑战性的,与年龄较大的儿童、青少年和成人相比,它们的发病率和死亡率更高。他们在发病部位、组织学特征、临床行为和处理方面也与大龄儿童不同。小儿低级别胶质瘤占该年龄组肿瘤的30%,包括局限性和弥漫性肿瘤。其次是成神经管细胞瘤和室管膜瘤。其他非髓母细胞瘤胚胎性肿瘤,以前称为PNETS,也常见于新生儿和婴儿。畸胎瘤在新生儿中发病率很高,但在生命的第一年结束前会逐渐下降。免疫组织化学、分子和基因组学的进步正在影响对某些肿瘤治疗的理解和靶向性,但是,尽管取得了这些进步,切除的程度仍然是影响几乎所有类型肿瘤预后和生存的最重要因素。结果很难估计,5年生存率从1 / 4到3 / 4不等。
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Intracranial Tumors in the First Year of Life.

Intracranial tumors in the first year of life are rare and, in this age group, are the second most common type of pediatric cancer after leukemias. As the more common solid tumor in neonates and infants, they present some peculiarities such as the high incidence of malignancies. Routine ultrasonography made easier to detect intrauterine tumors, but diagnosis can be delayed due to the lack or scarcity of recognizable symptoms. These neoplasms are often very large and highly vascular. Their removal is challenging, and there is a higher rate of morbidity and mortality than seen in older children, adolescents, and adults. They also differ from older children with respect to location, histological features, clinical behavior, and management. Pediatric low-grade gliomas represent 30% of the tumors in this age group and comprise circumscribed and diffuse tumors. They are followed by medulloblastoma and ependymoma. Other non-medulloblastoma embryonal neoplasms, former known as PNETS, are also commonly diagnosed in neonates and infants. Teratomas have an expressive incidence in newborns but decline gradually until the end of the first year of life. Immunohistochemical, molecular, and genomic advances are impacting the understanding and targeting of the treatment of some tumors, but, despite all these advances, the extent of resection remains the most important factor in the prognosis and survival of almost all types of tumors. The outcome is difficult to estimate, and 5-year survival ranges from one-quarter to three-quarters of the patients.

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