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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

97.4th percentile

Trials

181

Interventional, condition-specific

Researchers

1,084

Distinct authors in sample

Gene link

—

Readiness

2/6

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.”

2/6 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 checked

    Not yet enriched from Monarch / HPO

  4. Animal modelNot checked

    Not yet enriched from Monarch / Alliance

  5. Orphan designationNot checked

    FDA/EMA orphan-drug designation not enriched yet

  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)

Not enriched in this build — Monarch phenotype joins were not run for this record.

Animal models (Monarch / Alliance)

Not enriched in this build.

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=5449) is 41.

7,150 papers since the earliest indexed year in this search — median last-10-year count for a rare disease in this dataset is 41 (publications denominator n=5449).

4,594 in the last 10 years · high confidence · 97.4th 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 27 July 2026

181 interventional trials — more than 73% of diseases in the trials denominator have none at all (5114 of 7003; 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

Where to find support

We do not yet link condition-specific patient organisations. These umbrella groups support undiagnosed and ultra-rare families:

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