Histiocytic and dendritic cell neoplasm
diseaseOn this page
Also known as histiocytic and Dendritic cell neoplasmshistiocytic and Dendritic cell tumorshistiocytic and dendritic cell tumourhistiocytic and Dendritic cell tumours
Summary
Histiocytic and dendritic cell neoplasm (MONDO:0006247) is a cancer and 5 clinical trials. Top therapeutic interventions include cetuximab, choline salicylate, and selpercatinib. A subtype of lymphoid hemopathy — broader associated-gene and molecular evidence is on the parent page (see Disease family below).
At a glance
- Classification: Cancer
- Prevalence: <1 / 1 000 000 (Europe) [Orphanet-validated]
- Clinical trials: 5
Clinical features
Epidemiology
Prevalence records
1 prevalence record(s), Orphanet:
| Type | Class | Value | Geography | Validation |
|---|---|---|---|---|
| Annual incidence | <1 / 1 000 000 | 0.05 | Europe | Validated |
Identifiers
Disease identifiers
| Field | Value |
|---|---|
| Canonical name | histiocytic and dendritic cell neoplasm |
| Mondo ID | MONDO:0006247 |
| EFO | EFO:1000297 |
| Orphanet | 98287 |
| DOID | DOID:5621 |
| NCIT | C9294 |
| UMLS | C1292762 |
| MedGen | 714988 |
| GARD | 0019441 |
| Is cancer (heuristic) | yes |
Also known as: histiocytic and dendritic cell neoplasm · histiocytic and Dendritic cell neoplasms · histiocytic and Dendritic cell tumors · histiocytic and dendritic cell tumour · histiocytic and Dendritic cell tumours
Disease family
This is a subtype of lymphoid hemopathy. Genetic, therapeutic, and trial evidence is largely curated at the broader-term level — see the parent page for the associated-gene cohort and molecular evidence.
Classification path: disease › human disease › disease by etiologic mechanism › cancer or benign tumor › neoplastic disease or syndrome › neoplasm › hematopoietic and lymphoid system neoplasm › lymphoid hemopathy › histiocytic and dendritic cell neoplasm
Related subtypes (3): lymphoma, persistent polyclonal B-cell lymphocytosis, immunodeficiency-associated lymphoproliferative disease
Subtypes (3): indeterminate dendritic cell tumor, histiocytic sarcoma, dendritic cell tumor
Genetics & variants
GWAS landscape
No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.
Variant details and genetic-evidence tiers
No tiered GWAS variants or ClinVar records for this disease.
Genes & proteins
No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).
Function
No pathway enrichment — requires an associated-gene cohort.
Therapeutics
No druggable-target or therapeutic data for this disease’s cohort.
Clinical trials & evidence
Clinical trials
Clinical trials: 5.
Phase distribution (across all retrieved trials)
| Phase | Trials |
|---|---|
| PHASE2 | 2 |
| PHASE1 | 2 |
| PHASE1/PHASE2 | 1 |
Top trials by phase / activity
| NCT | Phase | Status | Title |
|---|---|---|---|
| NCT03698994 | PHASE2 | ACTIVE_NOT_RECRUITING | Ulixertinib in Treating Patients With Advanced Solid Tumors, Non-Hodgkin Lymphoma, or Histiocytic Disorders With MAPK Pathway Mutations (A Pediatric MATCH Treatment Trial) |
| NCT04320888 | PHASE2 | ACTIVE_NOT_RECRUITING | Selpercatinib for the Treatment of Advanced Solid Tumors, Lymphomas, or Histiocytic Disorders With Activating RET Gene Alterations, a Pediatric MATCH Treatment Trial |
| NCT05786924 | PHASE1/PHASE2 | RECRUITING | Phase 1/2 Trial of S241656 in Selected RAS/MAPK Mutation- Positive Malignancies |
| NCT03017820 | PHASE1 | RECRUITING | 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 |
| NCT04640779 | PHASE1 | COMPLETED | Low-Dose Selinexor and Choline Salicylate for Non-Hodgkin or Hodgkin Lymphoma, Histiocytic/Dendritic Cell Neoplasm, or Relapsed or Refractory Multiple Myeloma |
Drugs tested across these trials (top 30)
| Molecule | Max phase | Trials referencing |
|---|---|---|
| CETUXIMAB | 4 | 1 |
| CHOLINE SALICYLATE | 4 | 1 |
| SELPERCATINIB | 4 | 1 |
| ULIXERTINIB | 2 | 1 |
| CHEMBL5430810 | 0 | 1 |
Related Atlas pages
- Drugs: Cetuximab, Choline Salicylate, Selpercatinib