Sektionstage Herbst 2023 der Deutschen Gesellschaft für Neurochirurgie

PD Dr. med.

Oncological outcome of subtotal resection due to functional limitations in juveline low grade glioma – value of repeat resections

Prof. Dr. med.

Übersichtsvortrag

PD Dr.

Prof. Dr.

Assoc.Prof.Priv.Doz.Dr.

SIOP-MB6 – Integration of a randomized surgical question into a prospective clinical trial for standard risk medulloblastoma

Abstract-Text Medulloblastoma is the most frequent malignant embryonal tumor during childhood and is typically located in the cerebellum. Neurosurgical therapy remains the initial most important treatment. Among others, cerebellar mutism syndrome is a relevant surgical morbidity associated with medulloblastoma surgery possibly leading to long-term neurocognitive impairment. The MB-6 trial proposal is designed with a prospective randomized surgical goal for standard risk medulloblastoma in order to preserve neurocognitive function and maintain overall survival. Inclusion criteria are 4th ventricular tumors without evidence of metastatic disease in children and adolescence between 4 and 18 years of age. Randomization allocates patients into either group of maximal safe tumor resection or limited de-compressive surgery followed by neo-adjuvant chemotherapy and possible re-surgery. 96 patients are to be included in the trial with a primary endpoint of neurocognitive processing speed testing at 6 months after surgery. Hydrocephalus management is defined to secure regular CSF dynamics. Further oncological treatment is adapted according to molecular risk stratification. Further evidence is needed to optimally stratify surgical treatment of 4th ventricular tumors. Long-term neurocognitive outcome will determine quality of life measures, which is relevantly determined by surgical morbidity. The MB6 trial is designed to contribute for better surgical outcome in children with medulloblastoma.

Medulloblastom

Multizentrische Studien & Projekte

Abstractvortrag

Implementation of patient-specific treatment for pediatric brain tumors into clinical routine workflow

Univ.-Prof. Dr. med.

Screening- und Überwachungsempfehlungen für ZNS-Hämangioblastome bei pädiatrischen Patienten mit von-Hippel-Lindau (VHL) Krankheit

Abstract-Text Fragestellung: Anhand der bisher größten pädiatrischen Fallserie von Hippel-Lindau (VHL) Betroffener sollen Screeningempfehlungen für Hämangioblastome des zentralen Nervensystems (ZNS) ausgesprochen werden. Methoden: Die retrospektive monozentrische Kohortenstudie umfasst zwischen 1976 und 2011 geborene VHL-Patienten, die sich im Kindes-/Jugendalter einer kraniospinalen MRT-Untersuchung unterzogen. Alle Tumorresektionen an ZNS-Hämangioblastomen werden unterteilt hinsichtlich ihrer Indikationsstellung aufgrund geplanter MRT-Untersuchungen (asymptomatische Gruppe) oder ungeplanter MRT-Untersuchungen aufgrund klinischer oder neurologischer Symptome (symptomatische Gruppe). Hierauf basierend sollen Empfehlungen abgeleitet werden, in welchem Alter das MRT-Screening bei pädiatrischen VHL-Patienten eingeleitet und in welchen Zeitabständen es fortgeführt werden sollte. Ergebnisse: Von den 99 eingeschlossenen pädiatrischen VHL-Patienten entwickelten 42 (42,4%) ZNS Hämangioblastome. Bei 18 Patienten (18,2%) erfolgte die Entfernung von ZNS Hämangioblastomen im Kindes-/Jugendalter, beginnend ab einem Alter von 10,2 Jahren in der symptomatischen und 13,2 Jahren in der asymptomatischen Gruppe (Abb. 1). Von den 18 operierten VHL-Patienten waren 11 der symptomatischen (61,1%) und 7 der asymptomatischen Gruppe (38,9%) zuzuordnen. Träger einer trunkierenden VHL-Mutation hatten eine signifikant höhere Prävalenz und Operationsrate im Vergleich zu Trägern einer Missense-Mutation (Abb. 2). Schlussfolgerungen: Anhand der vorliegenden, bislang größten monozentrischen Fallserie an pädiatrischen VHL-Patienten lassen sich folgende Screeningempfehlungen für ZNS-Hämangioblastome bei klinisch-neurologisch unauffälligem Verlauf ableiten: A) Erstes kraniospinales MRT mit 12 Jahren (idealerweise in einer Untersuchung); B) Anschließend kraniospinales MRT alle 1-2 Jahre in Abhängigkeit des individuellen ZNS-Hämangioblastom-Befalls; C) VHL-Betroffene mit trunkierender Mutation haben ein erhöhtes Risiko sowohl für das Auftreten von ZNS-Hämangioblastomen als auch für die Notwendigkeit einer Operation im Kindes-/Jugendalter.

Leben mit (kindlichem) Hirntumor

Pädiatrische Tumoren

Functional outcome after CNS hemangioblastoma resection in pediatric patients affected by von Hippel-Lindau disease

Abstract-Text Objective: Central nervous system (CNS) hemangioblastomas represent the second most common early manifestation &lt; 18 years in von Hippel-Lindau (VHL) disease. Most lesions in childhood remain without relevant mass effect and thus asymptomatic. Surgical treatment has to be considered in case of radiographic progression or symptom development. The study intends to evaluate the clinical outcome of CNS hemangioblastoma removal in a pediatric VHL cohort. Methods: The retrospective monocenter cohort study examines VHL patients born between 1976 and 2010 who underwent surgery for CNS hemangioblastomas in childhood/adolescence. Diagnoses were based on MRI examinations that were either performed routinely for screening or because of neurological symptoms. Tumor characteristics and surgical outcome are evaluated based on clinical and radiological data. Results: 18 pediatric VHL patients underwent a total of 21 surgeries for 24 CNS hemangioblastomas between 1992 and 2023. These included 6 patients with first diagnosis of CNS involvement due to neurological symptoms. The mean age at surgery was 14.9 years (± 1.9). Hemangioblastomas were located in the cerebellum (n=11), brainstem (n=9) or spinal cord (n=4). 13 operations were performed for symptomatic lesions, and 8 operations were performed for asymptomatic lesions due to radiographic progression. In asymptomatic tumors, postoperative modified McCormick Scale (mMS) and modified Rankin Scale (mRS) scores remained stable in all cases (mMS grade 1, mRS grade 0 or 1) except 1 case with deterioration from mRS grade 0 to 1. In symptomatic tumors, postoperative mMS scores deteriorated by 1 grade in 2 cases and remained stable in the others (mMS grade 1-3). Postoperative mRS scores in symptomatic tumors improved by 1 grade in 1 case, deteriorated by 1-2 grades in 2 cases, and remained stable in the others (mRS grade 1-3). Surgery-related transient sensory deficits were present in 3 cases, and 1 patient recovered from postoperative hemiparesis. Cranial nerve affection with severe dysphagia requiring temporary tracheostomy occurred in 1 patient. Conclusion: Overall, surgical outcome following hemangioblastoma resection was favorable in most pediatric VHL cases. Postoperative functional scores were more likely to remain stable in asymptomatic than in symptomatic tumors. Therefore, MRI screening of pediatric patients showing progressive lesions with mass effect should consider surgical resection before potential symptoms develop.

Seminarraum 5

Joint Session Neuroonkologie und Pädiatrische Neurochirurgie – Pädiatrische Tumore

Joint Session

Joint Session Neuroonkologie und Pädiatrische Neurochirurgie – Pädiatrische Tumore

Termin

Vorsitz

Programm