Vessel adherent growth represents a major challenge in the surgical resection of neuroblastoma and Is associated with adverse outcome

• Published in: Journal of pediatric surgery Vol. 54; no. 11; pp. 2336 - 2342
• Main Authors: Mühling, Jakob, Eberherr, Corinna, Müller Höcker, Josef, Grote, Veit, von Schweinitz, Dietrich, Kappler, Roland, Fröba - Pohl, Alexandra
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01.11.2019
• Subjects: Adhesion; Blood Vessels - pathology; Cohort Studies; Gene Expression; Humans; Infant; Male; N-Myc Proto-Oncogene Protein - genetics; Neoplasm Invasiveness; Neoplasm Metastasis; Neoplasm Recurrence, Local; Neuroblastoma; Neuroblastoma - genetics; Neuroblastoma - pathology; Neuroblastoma - surgery; Outcome; Real-Time Polymerase Chain Reaction; Retrospective Studies; RNA - metabolism; Tumor markers; UVIN; Vessel adherence; ALK; UVIN; TBP; Tumor markers; OS; INSS; Neuroblastoma; IDRF; Adhesion; EFS; NB; TERT; DNA; Outcome; INRG; Vessel adherence
• ISSN: 0022-3468; 1531-5037
• DOI: 10.1016/j.jpedsurg.2019.07.012

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.