Histiocytic / Dendritic Cell Neoplasms
Prognosis
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🔬 Histological Types
📚 Latest Research
Successful treatment of systemic juvenile xanthogranuloma with an ABL2::GAB2 fusion using dasatinib after failure of conventional chemotherapy and MEK inhibition.
Li J, et al
A case report published in Haematologica documents successful disease control in a patient with systemic juvenile xanthogranuloma (JXG) harboring a rare ABL2::GAB2 kinase fusion, achieved with the tyrosine kinase inhibitor dasatinib after both conventional chemotherapy and MEK inhibitor therapy had failed. The finding establishes that comprehensive molecular profiling of refractory histiocytic neoplasms can uncover actionable fusion oncoproteins beyond the well-known BRAF and RAS/MAPK pathway alterations. This case highlights the clinical importance of next-generation sequencing in histiocytic disorders and positions ABL-class inhibitors as a viable rescue strategy for a subset of patients with aggressive, treatment-resistant JXG.
Haematologica
Source →Langerhans cell histiocytosis: molecular mechanisms underlying pathogenesis and emerging targeted therapeutics.
Li Q, et al
A comprehensive review reveals that Langerhans cell histiocytosis (LCH), a myeloid neoplasm driven by somatic mutations in the MAPK signaling pathway — most commonly BRAF(V600E) and MAP2K1 (MEK1) — relies on distinct structural mechanisms including constitutive kinase activation via K507-E600 salt bridge formation, BRAF/CRAF heterodimerization, and enhanced MEK1 homodimerization to sustain aberrant signaling. Current targeted therapies including BRAF inhibitors (vemurafenib, dabrafenib) and MEK inhibitors (trametinib, cobimetinib) show clinical benefit but are frequently limited by acquired resistance driven by RAF and MEK dimerization-dependent MAPK pathway reactivation. The review highlights emerging next-generation RAF and MEK inhibitors, as well as combination RAFi plus MEKi strategies designed to overcome resistance mechanisms and achieve more durable pathway suppression, with the goal of improving long-term outcomes for LCH patients.
Cancer metastasis reviews
Source →Atrial Pseudomasses in Erdheim-Chester Disease.
Azoulay L, et al
A publication in JAMA Cardiology highlights the occurrence of atrial pseudomasses—cardiac lesions that mimic true tumours on imaging—as a manifestation of Erdheim-Chester Disease (ECD), a rare histiocytic neoplasm. These pseudomasses arise from the infiltration of foamy histiocytes into cardiac tissue, particularly the right atrium and pericardium, and can be mistakenly diagnosed as primary cardiac tumours or thrombi. Accurate recognition of this finding is critical, as misdiagnosis may lead to unnecessary surgery, while correct identification enables prompt initiation of ECD-directed therapy such as BRAF/MEK inhibitors. Clinicians evaluating atrial masses should include ECD in the differential diagnosis, especially when systemic features of histiocytic infiltration are present.
JAMA cardiology
Source →💊 Therapies
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🧪 Tumor markers
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