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PMID (29701552)
Authors Himes BT, Ruff MW, Van Gompel JJ, Park SS, Galanis E, Kaufmann TJ, Uhm JH
Title Recurrent papillary craniopharyngioma with BRAF V600E mutation treated with dabrafenib: case report.
Journal Vol Issue Date Pages Journal Short Title
Journal of neurosurgery 130 4 2019 Apr 01 1299-1303 J Neurosurg
URL
Abstract Text The authors present the case of a man with a papillary craniopharyngioma, first diagnosed at 47 years of age, who experienced multiple recurrences. Review of the pathologic specimen from his first resection demonstrated the BRAF V600E mutation. With his most recent recurrence following previous surgery and radiotherapy, at 52 years of age, the decision was made to initiate treatment with the BRAF V600E inhibitor dabrafenib. Imaging following initiation of dabrafenib demonstrated reduction in tumor size. He remained on dabrafenib therapy for approximately 1 year and continued to demonstrate a good clinical result. At that time the decision was made to discontinue dabrafenib therapy and follow up with serial imaging. After more than 1 year of follow-up since stopping dabrafenib, the patient has continued to do well with no radiographic evidence of tumor progression and continues to be monitored with frequent interval imaging.

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Molecular Profile Treatment Approach
Gene Name Source Synonyms Protein Domains Gene Description Gene Role
Therapy Name Drugs Efficacy Evidence Clinical Trials
Drug Name Trade Name Synonyms Drug Classes Drug Description
Gene Variant Impact Protein Effect Variant Description Associated with drug Resistance
Molecular Profile Indication/Tumor Type Response Type Therapy Name Approval Status Evidence Type Efficacy Evidence References
BRAF V600E papillary craniopharyngioma predicted - sensitive Dabrafenib Case Reports/Case Series Actionable In a clinical case study, Tafinlar (dabrafenib) treatment resulted in decreased size of the cystic and solid enhancing components of the tumor in a patient with papillary craniopharyngioma harboring BRAF V600E, with stable disease continuing 1 year after discontinuation of treatment (PMID: 29701552). 29701552