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J Pediatr Hematol Oncol. 2018 Aug;40(6):478-482. doi: 10.1097/MPH.0000000000001032.

Sustained Response to Targeted Therapy in a Patient With Disseminated Anaplastic Pleomorphic Xanthoastrocytoma.

Author information

1
Division of Pediatric Hematology/Oncology.
2
Division of Radiology.
3
Division of Pathology.
4
Division of Neurosurgery, King Hussein Cancer Center, Amman, Jordan.
5
Division of Hematology/Oncology.
6
Division of Pathology, The Hospital for Sick Children, Toronto, ON, Canada.

Abstract

Pleomorphic xanthoastrocytoma is a rare brain tumor with unique high frequency of BRAF V600E mutation which is plausible for targeted therapy. The anaplastic variant has generally worse prognosis. We present an adolescent patient with a disseminated relapse of anaplastic pleomorphic xanthoastrocytoma following surgery, radiotherapy, and chemotherapy. She had a dramatic and prolonged response to a BRAF inhibitor (Dabrafinib) and later to addition of a MEK inhibitor (Trametinib) on tumor progression. With minimal side effects and a good quality of life, the patient is alive more than 2 years after initiation of targeted therapy. This experience confirms the potential role of targeted treatments in high-grade BRAF-mutated brain tumors.

PMID:
29200156
DOI:
10.1097/MPH.0000000000001032
[Indexed for MEDLINE]

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