International Expert Recommendations for the Diagnosis and Treatment of Malignant Histiocytic Neoplasms - PubMed
4 hours ago
- #diagnosis
- #treatment recommendations
- #histiocytic neoplasms
- International experts created recommendations to improve diagnosis and treatment of malignant histiocytic neoplasms (MHNs), rare tumors from the mononuclear phagocyte system.
- MHNs include histiocytic sarcoma, Langerhans cell sarcoma, interdigitating dendritic cell sarcoma, and other high-grade tumors, but current classification systems are inconsistent.
- A Histiocyte Society working group used a Delphi process to produce over 40 consensus statements on classification, pathology, molecular testing, clinical evaluation, and therapy.
- Key recommendations: unified MHN designation, minimum immunophenotypic panel, broad molecular profiling, and documenting prior hematopoietic malignancies.
- Treatment algorithms recommend surgical resection for unifocal disease and targeted therapy or immune checkpoint inhibition for multifocal disease with actionable mutations or PD-L1 expression.
- Future priorities include international registries and biomarker-driven trials to refine risk stratification and explore targeted therapy, immunotherapy, and combination approaches.