组织学良好、MYCN基因扩增的4S期新生儿神经母细胞瘤。

Favorable histology, MYCN-amplified 4S neonatal neuroblastoma.

作者信息

Chan Edward L, Harris Richard E, Emery Kathleen H, Gelfand Michael J, Collins Margaret H, Gruppo Ralph A

机构信息

Division of Pediatric Hematology/Oncology, State University of New York at Stony Brook, Stony Brook, New York 11776, USA.

出版信息

Pediatr Blood Cancer. 2007 Apr;48(4):479-82. doi: 10.1002/pbc.20705.

DOI:10.1002/pbc.20705

PMID:16333837

Abstract

We report a neonate with 4S neuroblastoma and MYCN amplification, but favorable Shimada histology, successfully treated with chemotherapy and 13-cis-retinoic acid without stem cell transplantation. MYCN amplification in neuroblastoma is usually associated with unfavorable Shimada histology; the presence of these features in infants with 4S disease confers a poor prognosis. A small number of infants with 4S neuroblastoma and MYCN amplification have favorable Shimada histology. In this subgroup of infants, histopathology may be equally important in predicting outcome.

摘要

我们报告了一名患有4S期神经母细胞瘤且MYCN基因扩增，但具有良好岛田组织学特征的新生儿，该患儿在未进行干细胞移植的情况下，通过化疗和13-顺式维甲酸成功治愈。神经母细胞瘤中的MYCN基因扩增通常与不良的岛田组织学特征相关；4S期疾病婴儿中出现这些特征预示预后不良。少数患有4S期神经母细胞瘤且MYCN基因扩增的婴儿具有良好的岛田组织学特征。在这一婴儿亚组中，组织病理学在预测预后方面可能同样重要。