Outcome des thorakalen Neuroblastoms

Bielefeld, Timon (2026): Outcome des thorakalen Neuroblastoms: Korrelation von image-defined risk factors und MYCN-Amplifikation auf chirurgische Ergebnisse und Überleben. Dissertation, LMU München: Medizinische Fakultät

Abstract

The present study investigates the characteristics and outcomes of thoracic neuroblastomas within the context of a total cohort of 266 cases, of which thoracic tumors accounted for 12.4% (n = 33). Patients with thoracic primary localization demonstrated a significantly improved 5-year overall survival compared to patients with non-thoracic neuroblastomas (94% vs. 74%, Fig. 8, p = 0.05). Furthermore, the subgroup of thoracic neuroblastoma cases analyzed in this study exhibited a more favorable molecular profile relative to the overall cohort, with only 1 out of 33 patients presenting MYCN amplification (Fig. 16). Despite the biologically favorable features of thoracic neuroblastomas, no statistically significant difference was observed in the incidence of peri- and postoperative complications in correlation with tumor localization. Complications occurred in 29.3% of all surgical interventions (Fig. 2), with an incidence of 30.0% in non-thoracic surgeries and 24.2% in thoracic neuroblastoma surgeries (Section 4.1.5.1, p = 0.535). Similarly, MYCN status had no significant impact on complication risk (Fig. 4, p = 1). The Image-Defined Risk Factors (IDRF), introduced in 2009, serve as a tool for preoperative risk stratification. As corroborated by previous studies, an increasing number of IDRFs correlates with a higher risk of surgical complications (Monclair et al., 2009; Phelps et al., 2019; Temple et al., 2021). Consistent with this, the present study demonstrated a significant association between the presence of IDRFs and the occurrence of surgical complications (Fig. 5, OR 2.5, p < 0.004). Notably, no significant association was found between age at initial diagnosis and the number of IDRFs (Table 9, p = 0.221). Five-year overall survival declined significantly with increasing IDRF burden, dropping from 83.5% in the absence of IDRFs to 49.0% in the presence of three IDRFs (Fig. 21, p = 0.005). Additionally, the prognostic significance of IDRFs in the management of non-thoracic, particularly abdominal neuroblastomas, was reaffirmed: the proportion of patients undergoing complete macroscopic resection was significantly lower in those with IDRFs (INRGSS stage L1: 56.3% vs. INRGSS stage L2: 11.1%, Fig. 14, p < 0.001). Furthermore, a rising number of IDRFs correlated with a significant decrease in complete resections (Fig. 11, p < 0.001) and increased the risk of surgical complications by 11 percentage points per additional IDRF identified (Table 10, p < 0.001). Interestingly, these associations did not apply to patients with thoracic tumors. No significant correlation was observed between the presence of one or more IDRFs and the occurrence of complications in this subgroup (Table 11, p = 0.161). Similarly, the extent of resection was not influenced by IDRF burden (Fig. 11, p = 0.292), nor was the extent of resection associated with complication rates in thoracic neuroblastomas (Fig. 15, p = 0.189). In contrast, a significant relationship between extent of resection and the incidence of operative complications was observed in non-thoracic neuroblastomas (Fig. 15, p < 0.001). The findings of this study suggest that the individual predictive value of IDRFs for surgical complications in patients with thoracic neuroblastoma warrants further evaluation. Given the limited sample size of this subgroup, a multicenter study incorporating a larger patient population appears justified in order to more precisely assess the prognostic relevance of IDRFs in this specific entity.

• Dokumententyp: Dissertationen (Dissertation, LMU München)
• Keywords: Neuroblastom, IDRF, MYCN, operative Komplikationen
• Themengebiete: 600 Technik, Medizin, angewandte Wissenschaften ; 600 Technik, Medizin, angewandte Wissenschaften > 610 Medizin und Gesundheit
• Fakultäten: Medizinische Fakultät
• Sprache der Hochschulschrift: Deutsch
• Datum der mündlichen Prüfung: 29. Januar 2026
• 1. Bericht­erstatter:in: Fröba-Pohl, Alexandra