Secondary BRAF-mutated histiocytic/dendritic cell sarcoma transdifferentiated from follicular lymphoma with prolonged response to BRAF/MEK inhibition and subsequent evolution to high-grade B-cell lymphoma.

Abstract excerpt

Transdifferentiation from follicular lymphoma (FL) to histiocytic/dendritic cell sarcoma (HDS) is rare and requires molecular confirmation of shared clonal origin. Targetable mutations such as BRAF V600E may offer therapeutic opportunities in such aggressive neoplasms. We report an exceptional case of untreated localised FL transdifferentiated to an HDS after 18 years. Shared BCL2 rearrangement and mutation...

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