Diez B, Balmaceda C, Matsutani M, Weiner H L
Children's Hospital of Buenos Aires, Billinghurst 2135 9/b, 1425 Buenos Aires, Argentina.
Childs Nerv Syst. 1999 Oct;15(10):578-85. doi: 10.1007/s003810050546.
Primary germ cell tumors of the central nervous system are rare neoplasms, accounting for no more than 2% of all malignancies in children and young people under 20 in the Western hemisphere. They have unique features related to age at diagnosis and sites of origin, as well as race and gender predilection. Prognosis has been clearly shown to be strongly related to pathological classification as either pure germinoma or nongerminomatous germ cell tumor, although many of these lesions are comprised of mixed elements. The presence of serum or cerebrospinal fluid tumor marker elevation has been an essential determinant of response to treatment. Because of the deleterious effects of irradiation on the immature nervous system, investigators have used chemotherapeutic strategies that either reduce or eliminate radiation therapy. In this article, we review the most recent advances in therapy for CNS germ cell tumors in the pediatric population and highlight the importance of cooperative trials in this setting.
中枢神经系统原发性生殖细胞肿瘤是罕见肿瘤，在西半球20岁以下儿童和年轻人中占所有恶性肿瘤的比例不超过2%。它们在诊断年龄、起源部位以及种族和性别偏好方面具有独特特征。尽管许多这些病变由混合成分组成，但预后已明确显示与病理分类为纯生殖细胞瘤或非生殖细胞性生殖细胞肿瘤密切相关。血清或脑脊液肿瘤标志物升高一直是治疗反应的重要决定因素。由于辐射对未成熟神经系统有有害影响，研究人员采用了减少或消除放射治疗的化疗策略。在本文中，我们回顾了儿科人群中枢神经系统生殖细胞肿瘤治疗的最新进展，并强调了在这种情况下合作试验的重要性。
