Hubertus J, Pohl A, Schmid I, von Schweinitz D
Pediatric Surgery, Ludwig-Maximilians University, Munich, Germany.
Department of Pediatric Surgery, Dr. von Hauners' childrens hospital, Munich, Germany.
Klin Padiatr. 2016 Apr;228(3):135-8. doi: 10.1055/s-0042-101030. Epub 2016 Feb 29.
Tumors of the adrenal gland are rare in children younger than 24 months of age. While neuroblastomas are most important in this age group, adrenal hemorrhage and other tumors are sometimes difficult to distinguish. Harvesting biopsies is mandatory in these young patients to obtain information on molecular markers, namely, MYCN and 1p deletion.
Between 03/2012 and 10/2013, 11 patients younger than 24 months of age with suspicious adrenal tumors were operated on laparoscopically.
The diagnostic workup was coordinated by our pediatric oncologists according to the terms of the NB2004 trial protocol.
9 out of 11 had a diagnosis of neuroblastoma, the others were adenoma respective complete necrosis of the adrenal gland. All of the neuroblastomas were negative for both MYCN amplification and 1p deletion. A complete resection was successful in 9 out of 11 cases. 3 complications occurred, 1 major and 2 minor.
Behind the recognition that laparoscopic adrenalectomy is technically feasible, the fact that all neuroblastomas were negative for MYCN amplification and 1p deletion raises the issue of whether biopsy is mandatory for risk stratification in this age group.
肾上腺肿瘤在24个月以下的儿童中较为罕见。虽然神经母细胞瘤在这个年龄组中最为重要，但肾上腺出血和其他肿瘤有时难以区分。对于这些年轻患者，进行活检以获取分子标志物（即MYCN和1p缺失）的信息是必不可少的。
在2012年3月至2013年10月期间，11名年龄小于24个月、患有可疑肾上腺肿瘤的患者接受了腹腔镜手术。
诊断检查由我们的儿科肿瘤学家根据NB2004试验方案的条款进行协调。
11例中有9例诊断为神经母细胞瘤，其他为腺瘤或肾上腺完全坏死。所有神经母细胞瘤的MYCN扩增和1p缺失均为阴性。11例中有9例成功进行了完整切除。发生了3例并发症，1例严重，2例轻微。
在认识到腹腔镜肾上腺切除术在技术上可行之后，所有神经母细胞瘤的MYCN扩增和1p缺失均为阴性这一事实引发了一个问题，即在这个年龄组中进行活检以进行风险分层是否必不可少。
