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Rare tumors that affect the hematopoietic and lymphoid tissues. The cells of origin are the histiocytes and accessory cells. They can occur at any age and show no significant variations in geographical distribution. This category includes the histiocytic sarcoma, Langerhans cell histiocytosis, Langerhans cell sarcoma, interdigitading dendritic cell sarcoma/tumor, follicular dendritic cell sarcoma/tumor, and dendritic cell sarcoma, not otherwise specified. (WHO, 2001)
Biomarker and diagnostic research for histiocytic and dendritic cell neoplasm has been reported in the published literature.
5 clinical trials registered, 2 recruiting. Interventions under study include drug therapy, procedural interventions, other interventions, and biologic therapy. Pipeline includes 2 PHASE2, 3 PHASE1. Research is primarily sponsored by academic and government institutions.
NCT ID | Title | Phase | Sponsor | Status |
|---|---|---|---|---|
[NCT04320888](https://clinicaltrials.gov/study/NCT04320888) |
Data assembled from 4 of 12 sources · Last updated Sep 20, 2026, 3:07 PM UTC
European rare disease database
Genetic and Rare Diseases Info Center
Selpercatinib for the Treatment of Advanced Solid Tumors, Lymphomas, or Histiocytic Disorders With Activating RET Gene Alterations, a Pediatric MATCH Treatment Trial |
PHASE2 |
National Cancer Institute (NCI) |
ACTIVE_NOT_RECRUITING |
[NCT03698994](https://clinicaltrials.gov/study/NCT03698994) | Ulixertinib in Treating Patients With Advanced Solid Tumors, Non-Hodgkin Lymphoma, or Histiocytic Disorders With MAPK Pathway Mutations (A Pediatric MATCH Treatment Trial) | PHASE2 | National Cancer Institute (NCI) | ACTIVE_NOT_RECRUITING |
[NCT04640779](https://clinicaltrials.gov/study/NCT04640779) | Low-Dose Selinexor and Choline Salicylate for Non-Hodgkin or Hodgkin Lymphoma, Histiocytic/Dendritic Cell Neoplasm, or Relapsed or Refractory Multiple Myeloma | PHASE1 | Mayo Clinic | UNKNOWN |
[NCT05786924](https://clinicaltrials.gov/study/NCT05786924) | Phase 1/2 Trial of S241656 in Selected RAS/MAPK Mutation- Positive Malignancies | PHASE1 | Institut de Recherches Internationales Servier | RECRUITING |
[NCT03017820](https://clinicaltrials.gov/study/NCT03017820) | A Vaccine (VSV-hIFNβ-NIS) With or Without Cyclophosphamide and Combinations of Ipilimumab, Nivolumab, and Cemiplimab in Treating Relapsed or Refractory Multiple Myeloma, Acute Myeloid Leukemia or Lymphoma | PHASE1 | Mayo Clinic | RECRUITING |
62 publications have been identified in PubMed for histiocytic and dendritic cell neoplasm. Research spans Review / Meta-Analysis (45%), Case Report / Case Series (34%), and Diagnostic / Biomarker (9%).
Research Type | Count | % of Total |
|---|---|---|
Research summaries | 25 | 45% |
Patient case studies | 19 | 34% |
Testing and diagnosis research | 5 | 9% |
Laboratory research | 3 | 5% |
Other research | 1 | 2% |
Clinical study results | 1 | 2% |
Disease patterns and progression | 1 | 2% |
New treatment approaches | 1 | 2% |
Lee YJ (2026). [PMID: 41580890](https://pubmed.ncbi.nlm.nih.gov/41580890/). *Am J Case Rep*. [Review / Meta-Analysis]
Kostares E (2026). [PMID: 42200865](https://pubmed.ncbi.nlm.nih.gov/42200865/). *Reports (MDPI)*. [Case Report / Case Series]
Däniker M (2026). [PMID: 41170802](https://pubmed.ncbi.nlm.nih.gov/41170802/). *Pediatr Dev Pathol*. [Review / Meta-Analysis]
Oyama Y (2026). [PMID: 41922228](https://pubmed.ncbi.nlm.nih.gov/41922228/). *J Clin Exp Hematop*. [Case Report / Case Series]
Fend F (2026). [PMID: 40210741](https://pubmed.ncbi.nlm.nih.gov/40210741/). *Virchows Arch*. [Review / Meta-Analysis]
Goyal G (2026). [PMID: 41915766](https://pubmed.ncbi.nlm.nih.gov/41915766/). *Blood*. [Review / Meta-Analysis]
Boyko G (2026). [PMID: 39171855](https://pubmed.ncbi.nlm.nih.gov/39171855/). *Turk Patoloji Derg*. [Review / Meta-Analysis]
Kemps PG (2026). [PMID: 42200684](https://pubmed.ncbi.nlm.nih.gov/42200684/). *J Pathol*. [Review / Meta-Analysis]
Li J (2026). [PMID: 41058101](https://pubmed.ncbi.nlm.nih.gov/41058101/). *Appl Immunohistochem Mol Morphol*. [Review / Meta-Analysis]
Batista JS (2025). [PMID: 40898278](https://pubmed.ncbi.nlm.nih.gov/40898278/). *Acta Vet Scand*. [Case Report / Case Series]