Department of Pediatric Surgery, Dr. von Hauner Children's Hospital, University Hospital, LMU Munich, Germany.
Department of Pediatric Surgery, Dr. von Hauner Children's Hospital, University Hospital, LMU Munich, Germany.
J Pediatr Surg. 2019 Nov;54(11):2336-2342. doi: 10.1016/j.jpedsurg.2019.07.012. Epub 2019 Jul 23.
Neuroblastoma (NB) is the most common extracranial, solid tumor in childhood, with a peak incidence in children under 6 years of age. Due to its variable course of disease, which ranges from spontaneous regression to metastatic spread, NB still represents a significant therapeutic challenge. Strikingly, a certain number of NBs intraoperatively show vessel adhesion and/or infiltrative growth, which is often not visible in pre-operative imaging. We proposed the term unexpected vessel infiltration of NB (UVIN) to denote this phenomenon. UVIN represents a major surgical challenge.
In this study, we determined frequency and clinical relevance of UVIN in a cohort of 100 NB-patients with subsequent correlation to several unfavorable characteristics of disease. RNA expression levels of MYCN and its co-regulated antisense transcript MYCNOS to identify markers was measured by PCR.
We found UVIN to be present in 34% of cases and significantly correlated with incomplete resection, MYCN amplification, complications, neoadjuvant therapy, tumor grade and MYCNOS expression levels. MYCN expression levels showed no significant results with UVIN.
Collectively, our data show that UVIN represents a frequent surgical problem associated with a poor outcome in NB patients. MYCN and MYCNOS seem to be no appropriate markers for UVIN.
Prognosis study.
Level III.
神经母细胞瘤（NB）是儿童期最常见的颅外实体瘤，发病高峰在 6 岁以下儿童。由于其疾病过程的多变性，从自发性消退到转移性扩散不等，NB 仍然是一个重大的治疗挑战。值得注意的是，有一定数量的 NB 在手术中表现出血管黏附和/或浸润性生长，这在术前影像学中通常不可见。我们提出了 NB 意外血管浸润（UVIN）这个术语来表示这种现象。UVIN 是一个主要的手术挑战。
在这项研究中，我们在 100 名 NB 患者的队列中确定了 UVIN 的频率和临床相关性，随后将其与疾病的几个不利特征相关联。通过 PCR 测量了 MYCN 和其共同调节的反义转录本 MYCNOS 的 RNA 表达水平，以确定标志物。
我们发现 34%的病例存在 UVIN，并且与不完全切除、MYCN 扩增、并发症、新辅助治疗、肿瘤分级和 MYCNOS 表达水平显著相关。UVIN 与 MYCN 表达水平无显著相关性。
综上所述，我们的数据表明，UVIN 是 NB 患者中常见的手术问题，与不良预后相关。MYCN 和 MYCNOS 似乎不是 UVIN 的合适标志物。
预后研究。
III 级。
