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305 Low grade glioma BRAF V600E inhibitor monotherapy in a 12-year old child
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  1. Filip Jadrijevic Cvrlje,
  2. Hrvoje Jednacak,
  3. Nada Rajacic,
  4. Tonci Grmoja,
  5. Ana Tripalo Batos,
  6. Jasminka Stepan Giljevic
  1. Department of hematology and oncology „Dr Mladen Cepulic’, Children’s Hospital Zagreb

Abstract

The goal is to report our center experience treating a 12-year old child with unresectable optochiasmatic pilocytic astrocytoma using BRAF V600E inhibitor monotherapy.

Low grade gliomas (LGGs) are the most frequent pediatric tumors of the central nervous system. The outcome of the treated children is satisfactory, albeit at the expense of the poor quality of life due to neurologic impairment linked to the tumor localization and multimodal oncologic treatment, including chemotherapy application. Cytostatic agents are used with the aim of achieving better disease control and increasing survival.

In the modern era of pediatric oncology detecting BRAF V600E mutation in histopathological samples has become a standard procedure which enables BRAF inhibitor therapy commencement. LGGs are stable monogenetic tumors characterized by activated MAPK pathway during all the phases of the illness making these drugs an attractive therapeutic option in relapsed disease.

Taking into account tumor localization, in our patient, only partial surgical reduction of the tumor mass was feasible and followed by chemotherapy administration. Unfortunately disease relapse ensued requiring new neurosurgical procedure, again without the possibility of achieving a complete resection. After BRAF V600E mutation has been found in resected material, a compassionate use of dabrafenib has been initiated.

In our patient oral BRAF inhibitor monotherapy of incompletely resected low grade glioma was easy to implement, with good medication tolerance, resulting in satisfactory disease control and good quality of life.

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