Characterization and treatment outcomes of malignant histiocytoses in a retrospective series of 141 cases in France.

ABSTRACT:

Abstract

Malignant histiocytoses (MH) are rare and poorly understood cancers, with no established therapeutic guidelines. We conducted a national retrospective study of MH diagnosed in France between 2000 and 2023. All cases underwent centralized histological review, and several malignant tumors with a stroma highly enriched in histiocytes were excluded. In total, 141 patients were included, with a median age of 62 years (range, 1-87). The cases comprised either primary MH (64%) or MH associated with other hematologic malignancies (36%). Phenotypes corresponded to histiocytic (43%), interdigitating dendritic cell (37%) or Langerhans cell (12%) sarcomas, or high-grade indeterminate dendritic cell tumors (10%), as per the World Health Organization classification. Tumor cells were alm

SUBMITTER: Bigenwald C 

PROVIDER: S-EPMC12148392 | biostudies-literature | 2025 May

REPOSITORIES: biostudies-literature