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Klin Padiatr 2022; 234(06): 391-394
DOI: 10.1055/a-1849-1478
Short Communication

DIPG- Very Long-Term Survivors – are There Factors Which May Predict a Better Outcome?

DIPG (Ponsgliome)-Langzeitüberleber – gibt es Faktoren, die mit einem besseren Überleben assoziiert sind?
Annika Stock
1   Department of Neuroradiology, University Clinic of Würzburg, Wuerzburg, Germany
,
Heike Brackmann
2   Childrens Hospitals, University of Würzburg, Würzburg, Germany
,
Monika Warmuth-Metz
1   Department of Neuroradiology, University Clinic of Würzburg, Wuerzburg, Germany
,
Elisabeth Miller
2   Childrens Hospitals, University of Würzburg, Würzburg, Germany
,
Matthias Eyrich
2   Childrens Hospitals, University of Würzburg, Würzburg, Germany
,
Christof Maria Kramm 
3   Division of Pediatric Hematology and Oncology, Department of Child and Adolescent Health, Universitätsmedizin Goettingen, Göttingen, Germany
,
Paul G Schlegel
2   Childrens Hospitals, University of Würzburg, Würzburg, Germany
,
Verena A Wiegering
2   Childrens Hospitals, University of Würzburg, Würzburg, Germany
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Introduction

Diffuse intrinsic pontine gliomas (DIPG) account for 15–20% of all central nervous system tumors in children, and are the main cause of death in children with brain tumors [Hargrave DR et al., J Neurooncol 2006; 77: 267 f]. Although stereotactic biopsies seem to regain acceptance, due to neurosurgical techniques, the diagnosis of DIPG is currently still based on characteristic MRI findings (tumor centered in the pons associated with an expansion of more than 50%, dark on T1-weighted images, bright on T2-weighted images and little or no enhancement after gadolinium application) and clinical symptoms (less than three month duration with cranial nerve deficits and long track signs) [Kieran MW et al. PBC 2015,62: 3 f]. Nowadays, DIPGs are recorded under diffuse midline glioma, H3 K27M-mutant [Louis DN et al. 2016]. They show predominantly astrocytic differentiation and a K27M mutation in either H3F3A or HIST1H3B/C [Louis DN et al. 2016] 4th ed. L. IARC Press 2016. Histologically DIPGs can be astrocytoma of WHO grade II or III, but the grade does not predict the outcome and as one appearance of diffuse midline glioma they are finally rated WHO grade IV. Various clinical studies over the last decades have not shown an improvement in outcome by any adjuvant therapy such as chemotherapy, targeted therapy or radiation sensitizers. The current standard of care still is fractionated external-beam radiotherapy, to a dose of 60 Gy over a six-week period, and oral chemotherapy with temozolomide. So far, the median survival of DIPG patients is 11 months, the 5-year survival rate amounts to 2.3% [Hoffman LM et al., JCO 2018, 36: 1963f]. Patients surviving for more than 5 years are so called “very long-term survivors” (VLTS). Until august 2021 there were 3 DIPG of 17 patients (n=5 male [29.4%] and female n=12 [70.6%] patients; median age at diagnosis 7 years, range 3.8–17.8 years) at our single-center institution with an atypical benign course of disease, fulfilling the criteria of VLTS. However, none of the patients received a biopsy and a histologic examination of tumor tissue. Diagnosis was made on the basis of MRI scans only.



Publication History

Article published online:
26 September 2022

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