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ORPHA:86897

Langerhans cell sarcoma

high confidenceDisorder

Publications

453

84.6th percentile

Trials

0

Interventional, condition-specific

Researchers

1,223

Distinct authors in sample

Gene link

—

Readiness

2/6

Stages with a signal

Clinical definition (Orphanet)

A rare dendritic cell tumor characterized by an aggressive, high-grade neoplasm derived from Langerhans cells, most commonly extranodal and multifocal, involving the skin and underlying soft tissue, as well as lung, liver, spleen, and bone. Primary nodal involvement is seen in a minority of patients. Immune-phenotyping and the presence of Birbeck granules on ultrastructural examination reveal the Langerhans cell derivation of the neoplastic cells. Prognosis is generally poor.

Orphanet entry

Cross-references

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

Additional Mondo synonyms (1)

sarcoma of Langerhans cell

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

No specific-condition interventional trial, but broader-category trials exist — discuss eligibility with a clinician.

  1. Gene identifiedNot found

    No GenCC disease–gene assertion in this build

  2. LiteraturePresent

    453 matched papers (297 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 trialPartial

    None under the specific name; 1655 for broader category sarcoma

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?

453

453 papers have ever been published on this condition (under this name). For scale, breast cancer has over 700,000. Median papers in the last 10 years for a rare disease in this dataset (publications denominator n=5449) is 41.

453 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).

297 in the last 10 years · high confidence · 84.6th percentile (publications denominator)

Phrase hits: 453 · MeSH hits: 4

Open Europe PMC search

Who's working on it?

1,223

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

Who's working on it?

No interventional trial matched this name; these authors publish on it in the sampled literature — a practical starting point for contact.

  1. 01
    Goyal G10 papers · 2026

    Division of Hematology/Medical Oncology, Department of Medicine, University of Alabama at Birmingham, Birmingham, AL 35233, USA.

    Papers in Europe PMC
  2. 02
    Go RS9 papers · 2026

    Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, MN 55905, USA.

    Papers in Europe PMC
  3. 03
    Abeykoon JP8 papers · 2025

    Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, MN 55905, USA.

    Papers in Europe PMC
  4. 04
    Ravindran A8 papers · 2025

    Division of Laboratory Medicine-Hematopathology Section, Department of Pathology, University of Alabama at Birmingham, Birmingham, AL 35249, USA.

    Papers in Europe PMC
  5. 05
    Rech KL8 papers · 2026

    Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN.

    Papers in Europe PMC
  6. 06
    Young JR6 papers · 2025

    Department of Radiology, Mayo Clinic, Jacksonville, FL.

    Papers in Europe PMC
  7. 07
    Ohshima K5 papers · 2023

    Department of Pathology, Kurume University School of Medicine, Kurume, Japan.

    Papers in Europe PMC
  8. 08
    Zanwar S5 papers · 2025

    Division of Hematology, Mayo Clinic, Rochester, MN.

    Papers in Europe PMC
  9. 09
    Zhang W5 papers · 2026

    Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China. vv1223@vip.sina.com.

    Papers in Europe PMC
  10. 10
    Bennani NN4 papers · 2025

    Division of Hematology, Mayo Clinic, Rochester, Minnesota, USA.

    Papers in Europe PMC

Clinical research

Is a treatment being tested?

0

interventional trials for this specific condition

No interventional trial testing a treatment matched this specific condition name on ClinicalTrials.gov (observational studies and pan-disease registries are listed separately when present). 1,655 trials are registered for sarcoma, the broader category — shown separately because they may or may not enrol this specific subtype.

Data as of 27 July 2026

No matched interventional trials. This is true for 73% of diseases in the trials denominator (5114 of 7003). Here are the researchers publishing on it.

high confidence · 36.5th percentile (trials denominator)

Recruiting interventional trials

From the matched ClinicalTrials.gov set

No interventional trial testing a treatment was found for this specific condition name on ClinicalTrials.gov.

1,655 interventional trials matched sarcoma, the broader category — listed below. Those studies are not counted in the condition-specific total.

Broader category: sarcoma

1,655

Interventional trials for the parent category, exclusive of NCT IDs already counted above. Eligibility for this subtype is not guaranteed.

Worth raising with a clinician. How we count trials.

Recruiting under the broader category

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.

"Langerhans cell sarcoma" OR "sarcoma of Langerhans cell" OR "sarcoma of the Langerhans cell"

Run this search on Europe PMC

MeSH descriptor terms unioned into the query: Langerhans Cell Sarcoma

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

"Langerhans cell sarcoma" OR "sarcoma of Langerhans cell" OR "sarcoma of the Langerhans cell"

Study-type breakdown: 0 interventional · 0 observational · 0 expanded access. Only interventional studies enter the trial headline.

Parent-category trials query:

"sarcoma"

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

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 2026-07-27T03:17:51.596Z