Plasmacytoma
Synonyms: Solitary plasmacytoma
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult
- Status
- active
Plasmacytoma is a localized mass of neoplastic monoclonal plasma cells that represents approximately 5% of all plasma cell neoplasms. There are two separate entities: primary plasmacytoma of the bone and extramedullary plasmacytoma of the soft tissues. Of the extramedullary plasmacytomas, 80% occur in the head and neck, usually in the upper respiratory tract. The median age at diagnosis is 50 years and the male to female ratio is 3:1. Long-term survival is possible following local radiotherapy, particularly for soft tissue presentations.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Plasmacytoma | Solitary plasmacytoma |
| fr | Plasmocytome | Plasmacytome solitaire |
| de | Plasmozytom | Plasmozytom, solitäres |
| es | Plasmacitoma | Plasmacitoma solitario |
| it | Plasmocitoma | Plasmocitoma solitario |
| nl | Plasmacytoom | Solitair plasmacytoom |
| pt | Plasmocitoma | |
| pl | plazmocytoma | Odosobniony guz plazmocytowy |
| cs | Plazmocytom | Solitární plazmocytom |
| uk | Плазмоцитома | Солітарна плазмоцитома |
| zh | 浆细胞瘤 | 孤立性浆细胞瘤 |
| tr | Plazmasitom | Soliter plazmasitom |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C90.2 | BTNT | specific | validated |
| ICD-10 | C90.3 | BTNT | specific | validated |
| ICD-11 | 2A83.2 | E | specific | validated |
| MONDO | 0005615 | E | validated | |
| MeSH | D010954 | E | validated | |
| UMLS | C0032131 | E | validated | |
| GARD | 13446 | E | validated | |
| MedDRA | 10035484 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (2):
- ORPHA:209016Hematological disease associated with an acquired peripheral neuropathy
rare neurological diseases - ORPHA:98282Plasma cell tumor
rare hematological diseases; rare neoplastic diseases; rare transplant-related disorders
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only | Not yet validated | ORPHANET |
| Annual incidence | 1-9 / 1 000 000 | 0.34 / 100 000 | United States | Value and class | Validated | 19016727[PMID]_SEER Surveillance Epidemiology and End Results 1992-2004[OTHER] |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/86855
- This record as JSON: /data/d/86855.json