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ORPHA:86879

Extranodal nasal NK/T cell lymphoma

high confidenceDisorder

Also known as: Angiocentric T-cell lymphoma · Lethal midline granuloma · NK/T-cell lymphoma · NKTCL · Nasal T/natural killer-cell lymphoma

Publications

7,150

94.2th percentile

Trials

181

Interventional, condition-specific

Researchers

1,084

Distinct authors in sample

Gene link

—

Readiness

3/6

Stages with a signal

Clinical definition (Orphanet)

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.

How rare: 1-9 / 100 000 — about one to nine people per hundred thousand.

Orphanet entry

Cross-references

Joined from Mondo / Orphanet. MeSH labels may enter searches; UMLS / OMIM / NCIT are stored for reference.

Additional Mondo synonyms (7)

Extranodal NK/T lymphoma-nasal · Extranodal NK/T-cell lymphoma, nasal type · angiocentric T-cell lymphoma · lethal midline granuloma · nasal T/natural killer-cell lymphoma · nasal type Extranodal NK/T-cell lymphoma · reticulosis, malignant

Research stages

Trial readiness signals

Where this condition sits on an open-data research pipeline — not how close a treatment is, and not medical advice. Empty stages often mean “not in these databases under this Mondo ID,” not “impossible.”

3/6 stages with a signal

An interventional trial matched this condition name on ClinicalTrials.gov — see trials below.

  1. Gene identifiedNot found

    No GenCC disease–gene assertion in this build

  2. LiteraturePresent

    7,150 matched papers (4,594 in last 10 years) Source

  3. Phenotype characterisedNot found

    No HPO disease–phenotype associations via Monarch for these Mondo IDs

  4. Animal modelNot found

    No Alliance genotype “model of” associations via Monarch for these Mondo IDs

  5. Orphan designationPartial

    1 EMA designation (none yet with FDA orphan-indication approval) — e.g. autologous Epstein-Barr virus specific T cells derived from peripheral blood mononuclear cells, expanded ex vivo Source

  6. Interventional trialPresent

    181 matched on ClinicalTrials.gov (29 recruiting in sample)

Biology

Genes and phenotypes

Gene–disease validity from GenCC, plus phenotypes and animal models joined from Monarch Initiative via Mondo ID — not a clinical diagnosis aid.

Do we know what causes it?

Not yet — the cause hasn't been pinned down in GenCC.

No strong gene–disease assertion joined for this Orphanet entity.

Phenotypes (Monarch / HPO)

None returned for this Mondo ID. That often means “not linked under this ID,” not “no clinical features.”

Animal models (Monarch / Alliance)

None returned for this Mondo ID. Empty here is not proof that no model organism work exists under another name or gene.

Monarch fetch 2026-07-29

Therapies

Designations, candidates, and chemicals

FDA OOPD and EMA orphan designations, Open Targets clinical candidates, and CTD chemical associations via MyDisease.info. These never change the interventional-trial headline.

Orphan designation (FDA · EMA)

1

Designation · no FDA orphan-indication approval yet

  • EMA autologous Epstein-Barr virus specific T cells derived from peripheral blood mononuclear cells, expanded ex vivoTreatment of extranodal NK/T-cell lymphoma, nasal type · 14/07/2016 · WithdrawnEMA designation

Sources: FDA OOPD · EMA orphan designations

Open Targets candidates

31

Drugs / clinical candidates · MONDO_0019472

CTD chemicals (MyDisease.info)

No CTD chemical associations returned for this Mondo ID.

Literature

Is anyone studying this?

7,150

7,150 papers — among the better-studied rare conditions, though still a fraction of common-disease literature (breast cancer: over 700,000). Median papers in the last 10 years for a rare disease in this dataset (publications denominator n=3967) is 59.

7,150 papers since the earliest indexed year in this search — median last-10-year count for a rare disease in this dataset is 59 (publications denominator n=3967).

4,594 in the last 10 years · high confidence · 94.2th percentile (publications denominator)

Phrase hits: 7,150 · MeSH hits: 0

Open Europe PMC search

Who's working on it?

1,084

Distinct author names in 200 sampled papers — named people below.

Who's working on it?

People publishing on this condition (sampled Europe PMC records). Affiliation is the most recent found in that sample.

  1. 01
    Zhang Y11 papers · 2026

    Department of Radiation Oncology, the First Affiliated Hospital of Bengbu Medical University, Bengbu, China.

    Papers in Europe PMC
  2. 02
    Liu Y10 papers · 2026

    Department of Oral and Maxillofacial Surgery, The Second Affiliated Hospital of Zhejiang University, Hangzhou, Zhejiang, 310000, China.

    Papers in Europe PMC
  3. 03
    Wang L10 papers · 2026

    Department of Hematology, Beijing Tongren Hospital, Capital Medical University, Beijing, China.

    Papers in Europe PMC
  4. 04
    Wang X10 papers · 2026

    Beijing Gobroad Boren Hospital, Beijing, China.

    Papers in Europe PMC
  5. 05
    Wang Y10 papers · 2026

    Department of Pathology, The First Affiliated Hospital of Kunming Medical University, Kunming, Yunnan, China.

    Papers in Europe PMC
  6. 06
    Li J9 papers · 2026

    Beijing Gobord Boren Hospital, Beijing, China.

    Papers in Europe PMC
  7. 07
    Zhang W9 papers · 2026

    Department of Hematology, Shaanxi Provincial People's Hospital, Xi'an, Shaanxi 710000, P.R. China.

    Papers in Europe PMC
  8. 08
    Zhou H9 papers · 2026

    Division of Medical Oncology, Cancer Center and State Key Laboratory of Biotherapy, Sichuan University West China Hospital, Chengdu, China.

    Papers in Europe PMC
  9. 09
    Chen X7 papers · 2026

    Division of Medical Oncology, Cancer Center and State Key Laboratory of Biotherapy, Sichuan University West China Hospital, Chengdu, China.

    Papers in Europe PMC
  10. 10
    Li Y7 papers · 2026

    Department of Lymphoma and Hematology, The Affiliated Cancer Hospital of Xiangya School of Medicine, Central South University/Hunan Cancer Hospital, Changsha, Hunan, China.

    Papers in Europe PMC

Clinical research

Is a treatment being tested?

181

interventional trials for this specific condition

181 interventional trials matched this specific condition name; 29 currently recruiting in our sample.

Data as of 11 September 2026

181 interventional trials — more than 77.2% of diseases in the trials denominator have none at all (5501 of 7126; this disease is at the 99.2th percentile).

high confidence · 99.2th percentile (trials denominator)

Recruiting interventional trials

From the matched ClinicalTrials.gov set

181 interventional trials matched after quoted-phrase search and title/condition post-filter.

Observational and natural-history studies

10 observational studies match this condition. These do not test a treatment and are not counted in the interventional-trial headline, but they are genuine research: natural-history work often defines the endpoints needed for a future rare-disease trial, and families may be able to enroll.

Recruiting or not-yet-recruiting

Open the complete matched search on ClinicalTrials.gov

Other registries (secondary)

Broader net from EU CTIS, ISRCTN, and ICTRP when available — deduped against ClinicalTrials.gov IDs already counted above. Dual-model LLM relevance gates what we keep. These rows are not added to the interventional headline.

raw 1 · after dedupe 1 · already on CT.gov 0 · kept 0 · parent 0 · uncertain 1 · dropped 0 · fetched 2026-07-30

Source notes: ictrp: Error: ICTRP public search unavailable (WHO portal is SPA-only; SOAP needs partnership). Tried: https://apps.who.int/tri

No secondary-registry studies passed dual-model relevance for this condition name (after dedupe).

Uncertain / not reviewed (1)

Where to find support

Condition-specific patient organisations, when Orphanet lists them, are on the disease’s Orphanet page. We also link umbrella groups that support undiagnosed and ultra-rare families.

Orphanet entry for Extranodal nasal NK/T cell lymphoma — check Associations / patient organisations on that page.

India — NPRD

Last verified 2026-07-26

This ORPHAcode is not on our curated NPRD list (direct or Mondo-parent match). That does not decide clinical eligibility; families in India should ask a notified Centre of Excellence about current coverage.

Hand-curated for this project. ORPHAcode mappings are best-effort and may be incomplete or imprecise for umbrella categories. Parent (Mondo) matches mean the policy lists a broader category — confirm eligibility with a Centre of Excellence. Financial entitlements summarised from public policy statements and may change. This is not official government guidance.

How we counted this

Europe PMC query (preferred label + any corrected label + Orphanet and Mondo exact synonyms, stoplisted; unioned with resolved MeSH labels when available). UMLS / OMIM / NCIT cross-references are stored on the overview but are not added to the query string.

"Extranodal nasal NK/T cell lymphoma" OR "Angiocentric T-cell lymphoma" OR "Lethal midline granuloma" OR "NK/T-cell lymphoma" OR "NKTCL" OR "Nasal T/natural killer-cell lymphoma" OR "Extranodal NK/T lymphoma-nasal" OR "Extranodal NK/T-cell lymphoma, nasal type" OR "nasal type Extranodal NK/T-cell lymphoma" OR "reticulosis, malignant"

Run this search on Europe PMC

ClinicalTrials.gov query (quoted phrases + MeSH via query.cond, plus recall-expansion terms when used):

"Extranodal nasal NK/T cell lymphoma" OR "Angiocentric T-cell lymphoma" OR "Lethal midline granuloma" OR "NK/T-cell lymphoma" OR "NKTCL" OR "Nasal T/natural killer-cell lymphoma" OR "Extranodal NK/T lymphoma-nasal" OR "Extranodal NK/T-cell lymphoma, nasal type" OR "nasal type Extranodal NK/T-cell lymphoma" OR "reticulosis, malignant"

Interventional trials matched via: phrase (mesh = registered under a MeSH descriptor no name phrase would catch; recall-expansion = gene / selected parent terms used only for trials).

Study-type breakdown: 181 interventional · 10 observational · 1 expanded access. Only interventional studies enter the trial headline.

Query health: ok — strategies attempted: phrase; with hits: phrase

Run this search on ClinicalTrials.gov

Confidence reasoning

  • Preferred label is multi-word and distinctive
  • No synonyms dropped by stoplist
  • No label/synonym collisions with other diseases in this corpus

Ingested 2026-07-27T03:16:11.249Z