ArticleAmerican journal of hematology2024
Clinical characteristics, molecular aberrations, treatments, and outcomes of malignant histiocytosis.
Article in American journal of hematology, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 12 papers.
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Who cites it
12 citing papers in PubMed, 18 citations in OpenAlex.
- Histiocytosis development and clinical variation through the lens of genomics.The Journal of pathology · 2026Review
- Origin, diagnosis and treatment of periocular Histiocytic disorders- state-of-the-art review.Graefe's archive for clinical and experimental ophthalmology = Albrecht von Graefes Archiv fur klinische und experimentelle Ophthalmologie · 2026Review
- Clinical characteristics, genomic profiling, treatments, and outcomes of Langerhans cell sarcoma.Orphanet journal of rare diseases · 2026Article
- Primary pulmonary histiocytic sarcoma with CNS metastasis: a case report and molecular profiling insights.Frontiers in oncology · 2026Article
- Plasmacytoid dendritic cell proliferations, malignant histiocytic (macrophage/dendritic cell) neoplasms and follicular dendritic cell neoplasms: report from the 2024 Joint CSHP/EA4HP/SH workshop.Virchows Archiv : an international journal of pathology · 2025Article
- Histiocyte Society blueprint for non-Langerhans cell histiocytosis research: unraveling complex diseases through collaboration.Haematologica · 2025Review
- NTRK1-rearranged histiocytosis: clinicopathologic and molecular features.Blood advances · 2025Article
- Characterization and treatment outcomes of malignant histiocytoses in a retrospective series of 141 cases in France.Blood advances · 2025Article
- Langerhans Cell Histiocytosis and Other Histiocytic Lesions.Head and neck pathology · 2025Review
- Synchronous clonally related anaplastic large cell lymphoma and malignant histiocytosis.Diagnostic pathology · 2025Article
- Histiocytic Sarcoma Diagnosed on Repeat Biopsy of Multifocal Extranodal Tumors.Surgical case reports · 2025Article
- Updates on Langerhans cell histiocytosis and other histiocytosis in children: invited review-challenges and novelties in paediatric tumours.Virchows Archiv : an international journal of pathology · 2025Review
Corrections and comments
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Authors and funding
15 authors at 4 institutions in 1 country.
Funding
Abstract
Malignant histiocytosis (MH) is an extremely rare neoplasm of the macrophage-dendritic cell lineage. We report the clinical characteristics, molecular aberrations, treatments, and outcomes of patients with MH seen at two referral centers from January 2000 to May 2023. We identified 43 patients with MH, of which 26 had histiocytic sarcoma (MH-H), 9 interdigitating dendritic cell sarcoma (MH-IDC), and 8 Langerhans cell sarcoma (MH-LC). The median age at diagnosis was 61 years (range, 3-83). Thirty-three patients (77%) had multifocal disease, while 10 had unifocal involvement. Tumor specimens from 22 patients (51%) underwent targeted next generation sequencing, and 19 of 22 (86%) had at least one pathogenic mutation, including mutations in MAPK pathway genes (73%). The median overall survival (OS) among the entire cohort was 16 months (95% CI: 8-50). The outcomes of those with multifocal disease were significantly shorter than their unifocal counterpart: median OS of 10 months versus 50 months (p = .07). Patients with risk organ involvement (bone marrow, spleen, or liver) had significantly inferior outcomes. Chemotherapy and surgery were the most common first-line treatments for multifocal and unifocal disease, respectively. While the outcome for patients with multifocal disease was poor, there was a subset of patients who had durable responses to treatment. Our study highlights that MH has heterogeneous clinical presentation, frequent oncogenic mutations, and prognosis, which is strongly tied to disease extent and type of organ involvement.
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