Plasmacytoma

Summary

Plasmacytoma (MONDO:0005615) is a disease (an umbrella term covering 5 Mondo subtypes) and 15 clinical trials. Top therapeutic interventions include dexamethasone, ixazomib citrate, and dexamethasone sodium phosphate. A subtype of plasma cell neoplasm — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

• Prevalence: 1-9 / 1 000 000 (United States) [Orphanet-validated]
• Umbrella term: 5 Mondo subtypes
• Clinical trials: 15

Clinical features

Epidemiology

Prevalence records

1 prevalence record(s), Orphanet:

Type	Class	Value	Geography	Validation
Annual incidence	1-9 / 1 000 000	0.34	United States	Validated

Identifiers

Disease identifiers

Field	Value
Canonical name	plasmacytoma
Mondo ID	MONDO:0005615
EFO	EFO:0006738
MeSH	D010954
Orphanet	86855
DOID	DOID:3721
ICD-11	1811140613
NCIT	C9349
SNOMED CT	415112005
UMLS	C0032131
MedGen	18506
GARD	0013446
MedDRA	10035484
Is cancer (heuristic)	no

Also known as: anaplastic plasmacytoma · anaplastic skeletal plasmacytoma (type) · anaplastic solitary extramedullary plasmacytoma of the cecum (type) · extramedullary anaplastic plasmacytoma (type) · plasmacytoma · solitary plasmacytoma

Disease family

This is a subtype of plasma cell neoplasm. 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 body system or component › immune system disorder › leukocyte disorder › B-cell neoplasm › neoplasm of mature B-cells › plasma cell neoplasm › plasmacytoma

Related subtypes (6): refractory plasma cell neoplasm, leukemoid reaction, plasma cell myeloma, plasma cell leukemia, non-amyloid monoclonal immunoglobulin deposition disease, heavy chain disease

Subtypes (5): extramedullary plasmacytoma, solitary osseous plasmacytoma, solitary plasmacytoma of chest wall, primary plasmacytoma of the bone, extramedullary soft tissue plasmacytoma

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

Drugs indicated for this disease

0 approved, 4 in late-stage (phase 3) trials. Disease-direct ChEMBL indications, not inferred from the associated-gene cohort below.

Drug	Development status
Bortezomib	Phase 3 (in late-stage trials)
Dexamethasone	Phase 3 (in late-stage trials)
Ixazomib	Phase 3 (in late-stage trials)
Lenalidomide	Phase 3 (in late-stage trials)

Earlier-phase candidates (phase 2, investigational — efficacy not yet established): Busulfan, Daratumumab, Ixazomib Citrate, Melphalan, Panobinostat, Pomalidomide.

Clinical trials & evidence

Clinical trials

Clinical trials: 15.

Phase distribution (across all retrieved trials)

Phase	Trials
PHASE2	8
PHASE1/PHASE2	2
PHASE1	2
PHASE3	1
EARLY_PHASE1	1
Not specified	1

Top trials by phase / activity

NCT	Phase	Status	Title
NCT02544308	PHASE3	ACTIVE_NOT_RECRUITING	Trial of Immunomodulatory Therapy in High Risk Solitary Bone Plasmacytoma
NCT04174196	PHASE2	ACTIVE_NOT_RECRUITING	A Study of Lenalidomide and CC-486 With Radiation Therapy in Patients With Plasmacytoma
NCT05248633	PHASE2	RECRUITING	Chemotherapy Combined With Radiotherapy Versus Radiotherapy Alone for Solitary Plasmacytoma
NCT06429150	PHASE1/PHASE2	RECRUITING	Frontline Combination CAR-T Cell Therapy for Multiple Myeloma or Plasmacytoma
NCT06863584	PHASE2	RECRUITING	SBRT Plus Lenalidomide for Solitary Plasmacytoma: Phase II Trial
NCT00109889	PHASE2	TERMINATED	S0340 MRI and Fludeoxyglucose F18 PET in Diagnosing Solitary Plasmacytoma
NCT00421525	PHASE1/PHASE2	COMPLETED	Phase I/II Study of hLL1 in Multiple Myeloma
NCT02506959	PHASE2	COMPLETED	Panobinostat, Gemcitabine Hydrochloride, Busulfan, and Melphalan Before Stem Cell Transplant in Treating Patients With Refractory or Relapsed Multiple Myeloma
NCT02547662	PHASE2	COMPLETED	Pomalidomide, Ixazomib Citrate, and Dexamethasone in Treating Patients With Previously Treated Multiple Myeloma or Plasma Cell Leukemia
NCT03312868	PHASE2	TERMINATED	Stereotactic Body Radiotherapy for the Treatment of Solitary Bone Plasmacytoma
NCT04065308	PHASE2	UNKNOWN	Daratumumab With DCEP for Multiple Myeloma With Plasmacytoma
NCT02184533	PHASE1	COMPLETED	Sodium Selenite and Radiation Therapy in Treating Patients With Metastatic Cancer
NCT03031730	PHASE1	TERMINATED	Testing the Addition of KRT-232 (AMG 232) to Usual Chemotherapy for Relapsed Multiple Myeloma
NCT02921893	EARLY_PHASE1	COMPLETED	Ixazomib Citrate, Lenalidomide, and Dexamethasone in Treating Patients With POEMS Syndrome
NCT05260203	Not specified	COMPLETED	MargheRITA (Remote Intelligence for Therapeutic Adherence)

Drugs tested across these trials (top 30)

Molecule	Max phase	Trials referencing
DEXAMETHASONE	4	5
IXAZOMIB CITRATE	4	2
DEXAMETHASONE SODIUM PHOSPHATE	4	1
LENALIDOMIDE	4	1
PANOBINOSTAT	4	1
POMALIDOMIDE	4	1
NAVTEMADLIN	3	1
SODIUM SELENATE	3	1
SODIUM SELENITE	3	1
MILATUZUMAB	2	1
CHEMBL1813256	0	2
CHEMBL344227	0	1
CHEMBL2093113	0	1
CHEMBL1200637	0	1
CHEMBL4743806	0	1