Durable Remission in a Patient with Erdheim-Chester Disease Carrying a BRAF V600E Mutation Under Dabrafenib Treatment

Ulrich Schneeberger, Christoph Woernle, Kiriaki Kollia, Katrin D Stumpe, Elisabeth J Rushing, Rene Bernays and Christoph Renner

Department of Oncology, Onkozentrum Hirslanden, Switzerland Department of Neurosurgery, Onkozentrum Hirslanden, Switzerland Departement of Radiology, Onkozentrum Hirslanden, Switzerland Department of Neuropathology, University Hospital Zurich, Switzerland

^*Correspondance to: Ulrich Schneeberger 

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Abstract:

We report on a patient with Erdheim-Chester Disease (ECD) carrying a BRAF V600E mutation with a complete regression of a dural tumor as well as brainstem signal alterations following therapy with dabrafenib for more than 2 years. Initial neuroimaging studies including CT and MRI showed dural masses bifrontoparietal and a right-sided tentorial mass in the cerebellopontine angle. Biopsy revealed ECD with a BRAF V600E mutation. The patient underwent initial therapy with vemurafenib. However, treatment was terminated due to adverse effects. Accordingly, dabrafenib therapy at a dose of 75 mg twice daily was started, which was well tolerated. Following more than two years of dabrafenib treatment, the patient remains in complete remission regarding the CNS lesions with stable skeletal lesions.

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Cite the Article:

Schneeberger U, Woernle C, Kollia K, Stumpe KD, Rushing EJ, Bernays R, et al. Durable Remission in a Patient with Erdheim-Chester Disease Carrying a BRAF V600E Mutation Under Dabrafenib Treatment. Clin Case Rep Int. 2020; 4: 1152.