Vergleich klinischer und epidemiologischer Parameter der primären Gliomatosis cerebri und primär multifokaler hochgradiger Gliome im Kindes- und Jugendalter

Comparison of clinical and epidemiological parameters of primary Gliomatosis cerebri and primary multifocal high-grade gliomas in childhood and adolescence

by Anna Tacke
Doctoral thesis
Date of Examination:2024-09-03
Date of issue:2024-08-26
Advisor:Prof. Dr. Christof Kramm
Referee:Prof. Dr. Christof Kramm
Referee:PD Dr. Hans Christoph Bock
Persistent Address: http://dx.doi.org/10.53846/goediss-10699

 

 

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Abstract

English

To date no differentiation is being made between varying growth phenotypes of pediatric high-grade gliomas (pHGG). This thesis compares primary pediatric Gliomatosis cerebri (GC) and primary multifocal/multicentric (PMF) pediatric high-grade gliomas as rare growth phenotypes in regard to epidemiological, clinical and prognostic parameters as opposed to pHGG that primarily grow as solitary tumor nodules. For this purpose, 51 patients with primary pediatric GC, 37 patients with PMF pediatric high-grade gliomas and a total of 997 control patients, divided into specific, adapted comparison cohorts, were identified from the database of the HIT-HGG study group of the Society for Pediatric Oncology and Hematology in Germany, Austria and German-speaking Switzerland (GPOH). In regard to epidemiological and clinical parameters, primary pediatric GC and PMF pediatric high-grade gliomas differed significantly in terms of histological diagnoses, extent of tumor resection and radiation dose. An interesting and potentially clinically relevant observation, although not yet statistically significant, was the presence of tumor predisposition syndromes, which were present in 20% of PMF pediatric high-grade gliomas, but in only 3% of the primary pediatric GC cases. A comparison of the survival data shows that both primary pediatric GC and PMF pediatric high-grade gliomas have an even worse prognosis than the usual growth phenotypes of pHGG, which already have a dismal prognosis. Only diffuse intrinsic pontine gliomas/diffuse midline gliomas have a similarly poor prognosis. The current WHO classification of tumors of the central nervous system does not do justice to these relevant differences in survival.
Keywords: pediatric high-grade glioma; Gliomatosis cerebri; multifocal; pHGG
Schlagwörter: Gliomatosis cerebri; multifokal; pädiatrisches hochgradiges Gliom; pHGG