Department of Pathology, Boston Children's Hospital, Harvard Medical School, 300 Longwood Avenue, Boston, MA 02115, USA.
Department of Pathology, Boston Children's Hospital, Harvard Medical School, 300 Longwood Avenue, Boston, MA 02115, USA.
Surg Pathol Clin. 2020 Jun;13(2):235-247. doi: 10.1016/j.path.2020.01.003. Epub 2020 Apr 7.
Embryonal tumors of the central nervous system (CNS) are rare, high-grade neoplasms predominantly affecting the pediatric population. Well-defined embryonal tumors include medulloblastoma, atypical teratoid/rhabdoid tumor, embryonal tumor with multilayered rosettes, C19MC-altered and embryonal tumor with multilayered rosettes, not otherwise specified, pineoblastoma, pituitary blastoma, CNS neuroblastoma, and ganglioneuroblastoma. Although their prognosis is nearly uniformly poor, the rapidly evolving understanding of their molecular biology contributes to diagnosis, prognosis, treatment, and clinical trial participation. Knowledge of current tumor stratification and diagnostic techniques will help pathologists guide care and preserve tissue for necessary or desired additional testing.
中枢神经系统（CNS）胚胎性肿瘤罕见，是主要影响儿童人群的高级别肿瘤。明确的胚胎性肿瘤包括髓母细胞瘤、非典型畸胎瘤/横纹肌样瘤、具有多层菊形团的胚胎性肿瘤、C19MC 改变的胚胎性肿瘤、具有多层菊形团的胚胎性肿瘤、未特指、松果体母细胞瘤、垂体胚细胞瘤、中枢神经细胞瘤和神经节神经母细胞瘤。尽管它们的预后几乎普遍较差，但对其分子生物学的快速发展的认识有助于诊断、预后、治疗和临床试验参与。了解当前的肿瘤分层和诊断技术将帮助病理学家指导治疗并保存组织，以备进行必要或所需的进一步检测。
