Extranodal nasal NK/T cell lymphoma
Synonyms: Angiocentric T-cell lymphoma · Lethal midline granuloma · NK/T-cell lymphoma · NKTCL · Nasal T/natural killer-cell lymphoma
- Type
- Disease
- Group
- Disorder
- Inheritance
- Multigenic/multifactorial, Not applicable
- Age of onset
- Adult
- Status
- active
Extranodal nasal NK/T cell lymphoma (NKTCL) is a rare, malignant neoplasm mainly affecting men in the fifth decade of life, that usually arises in the nose, paranasal sinuses, orbits or upper airway, and that can present with a nasal mass, nasal bleeding, nasal obstruction, palate perforation (i.e. midline perforation of the hard palate), and mid-facial and/or upper airway destructive lesions. In advanced disease stages, which are associated with a poor prognosis, NKTCL may disseminate to other organs. A few cases of NKTCL presenting primarily in the lymph nodes have also been described.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Extranodal nasal NK/T cell lymphoma | Angiocentric T-cell lymphoma · Lethal midline granuloma · NK/T-cell lymphoma · NKTCL · Nasal T/natural killer-cell lymphoma |
| fr | Lymphome NK/T extranodulaire type nasal | NKTCL · Granulome létal de la ligne médiane · Lymphome NK/T · Lymphome T angiocentrique · Lymphome à cellules natural killer/T nasal |
| de | Extranodales NK/T-Zell-Lymphom | Mittelliniengranulom, letales · NK/T-Zell-Lymphom · NKTCL · Nasales T-Zell-/Natürliche-Killer-Zell-Lymphom · T-Zell-Lymphom, angiozentrisches |
| es | Linfoma extranodal nasal de células NK/T | Granuloma letal de la línea media · Linfoma angiocéntrico de células T · Linfoma de células NK/T · Linfoma nasal de células T/natural killer · NKTCL |
| it | Linfoma nasale extranodale a cellule NK/T | Granuloma letale della linea mediana · Linfoma angiocentrico a cellule T · Linfoma nasale a cellule T/natural killer · NKTCL |
| nl | Extranodaal nasaal NK/T-cellymfoom | Angiocentrisch T-cellymfoom · Letaal middellijngranuloom · NK/T-cellymfoom · NKTCL · Nasaal T-cel-/natuurlijke killercellymfoom |
| pt | Linfoma extranodal das células NK/T, tipo nasal | |
| pl | Pozawęzłowy chłoniak z komórek NK/T typu nosowego | Angiocentryczny chłoniak z komórek T · Chłoniak z komórek NK/T · NKTCL · Nosowy chłoniak z komórek T/NK · Ziarniniak pośrodkowy |
| cs | Extranodální nazální NK/T-buněčný lymfom | Angiocentrický T-lymfom · NKTCL · Letální granulom střední čáry · NK/T-buněčný lymfom · Nazální T/natural killer-buněčný lymfom |
| uk | Екстранодальна назальна NK/T-клітинна лімфома | Ангіоцентрична Т-клітинна лімфома · Смертельна гранульома серединної лінії · Т-лімфома носа/природна кілерна лімфома · NK/T-клітинна лімфома · NKTCL |
| zh | 结外鼻型NK/T细胞淋巴瘤 | 鼻型T/自然杀伤细胞淋巴瘤 |
| tr | Ekstranodal nazal NK / T-hücreli lenfoma | NKTCL · NK/T hücreli lenfoma · Letal orta hat granülomu · Anjiyosentrik T hücreli lenfoma · Nazal T/doğal öldürücü hücreli lenfoma |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C86.0 | E | specific | validated |
| ICD-11 | 2A90.6 | E | specific | validated |
| MONDO | 0019472 | E | validated | |
| MeSH | D054391 | E | validated | |
| UMLS | C0392788 | E | validated | |
| GARD | 7041 | E | validated | |
| MedDRA | 10065855 | 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:178551Aggressive primary cutaneous T-cell lymphoma
rare skin diseases; rare hematological diseases; rare neoplastic diseases; rare transplant-related disorders - ORPHA:289644Epstein-Barr virus-associated malignant lymphoproliferative disorder
rare neoplastic diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | 1-9 / 100 000 | 2.25 / 100 000 | France | Value and class | Validated | OTHERitut National du Cancer 2013[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/86879
- This record as JSON: /data/d/86879.json