An 8-year-old girl with secondary histiocytic sarcoma with BRAF^V600 mutation following T-cell acute lymphoblastic leukemia demonstrating stable disease for 3 years on dabrafenib and trametinib - a case report and literature review.

ABSTRACT:

Background

Histiocytic sarcoma as a secondary malignancy following childhood leukemia is extremely uncommon with fewer than 20 cases reported worldwide. They often pose a diagnostic challenge and prognosis is dismal. There is a lack of well-established clinical treatment protocols owing to rarity of disease. Majority were managed with chemotherapy with variable outcomes.

Case presentation

Herein we report a rare case of an 8-year-old girl with secondary BRAF^V600-mutant histiocytic sarcoma following T-cell acute lymphoblastic leukemia. After poor disease control with salvage chemotherapy, she was treated with MAPK-targeted therapy with dabrafenib and trametinib. She demonstrated excellent response and remained in partial remission with no signs of disease progressi

SUBMITTER: Tan SL 

PROVIDER: S-EPMC11889787 | biostudies-literature | 2025 Mar

REPOSITORIES: biostudies-literature