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ORPHA:158019

Indeterminate cell histiocytosis

high confidenceDisorder

Also known as: Indeterminate dendritic cell neoplasm · Indeterminate dendritic cell tumor

Publications

576

87.2th percentile

Trials

0

Interventional, condition-specific

Researchers

1,235

Distinct authors in sample

Gene link

—

Readiness

2/6

Stages with a signal

Clinical definition (Orphanet)

A rare neoplastic disease characterized by multiple, and on occasion single, asymptomatic, smooth, red-brown papulonodules located on the face, neck, trunk and/or extremities which present a nonepidermotrophic histiocytic infiltrate with immunohistochemical features of both Langerhans and non-Langerhans cells (i.e. immunopositive for S100 protein and CD1a in the absence of Birbeck granules and langerin expression).

Orphanet entry

Cross-references

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

Additional Mondo synonyms (4)

indeterminate Dendritic cell tumor · indeterminate Dendritic cell tumour · indeterminate cell histiocytosis · indeterminate dendritic cell tumor

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

    576 matched papers (364 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; 68 for broader category histiocytosis

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?

576

576 papers have been published on this condition. That is a real research literature — still far smaller than common diseases (breast cancer: over 700,000 papers). Median papers in the last 10 years for a rare disease in this dataset (publications denominator n=5449) is 41.

576 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).

364 in the last 10 years · high confidence · 87.2th percentile (publications denominator)

Phrase hits: 576 · MeSH hits: 0

Open Europe PMC search

Who's working on it?

1,235

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
    Li J5 papers · 2025

    Department of Oncology, The University of Hong Kong-Shenzhen Hospital, Shenzhen, China.

    Papers in Europe PMC
  2. 02
    Wang L4 papers · 2026

    Department of Hematology, The Central Hospital of Wuhan, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.

    Papers in Europe PMC
  3. 03
    Zhang Y4 papers · 2026

    Institute of Hematology, Union Hospital, Tongji Medicine College, Huazhong University of Science and Technology, Wuhan, China.

    Papers in Europe PMC
  4. 04
    Abeykoon JP3 papers · 2025

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

    Papers in Europe PMC
  5. 05
    Cai H3 papers · 2022

    Department of Hematology, Peking Union Medical Hospital, Dongcheng District, Chinese Academy of Medical Sciences and Peking Union Medical College, 1 Shuai Fu Yuan Hu Tong, Beijing, 100730, People's Republic of China.

    Papers in Europe PMC
  6. 06
    Cao X3 papers · 2026

    Institute of Hematology, Union Hospital, Tongji Medicine College, Huazhong University of Science and Technology, Wuhan, China.

    Papers in Europe PMC
  7. 07
    Chen Y3 papers · 2026

    College of Traditional Chinese Medicine, Hubei University of Chinese Medicine, Wuhan, China.

    Papers in Europe PMC
  8. 08
    Emile JF3 papers · 2026

    Paris-Saclay University, Versailles SQY University, EA4340-BECCOH, Assistance Publique-Hôpitaux de Paris (AP-HP), Ambroise-Paré Hospital, Smart Imaging, Service de Pathologie, 92100 Boulogne, France.

    Papers in Europe PMC
  9. 09
    Li Y3 papers · 2023

    *Department of Dermatology, Second Xiangya Hospital, Central South University, Changsha, China; and†Department of Radiology, Hospital of the University of Pennsylvania, Philadelphia, PA.

    Papers in Europe PMC
  10. 10
    Tzankov A3 papers · 2026

    Institute of Medical Genetics and Pathology, University Hospital Basel, Basel, Switzerland.

    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). 68 trials are registered for histiocytosis, 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.

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

Broader category: histiocytosis

68

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.

"Indeterminate cell histiocytosis" OR "Indeterminate dendritic cell neoplasm" OR "Indeterminate dendritic cell tumor" OR "indeterminate Dendritic cell tumour"

Run this search on Europe PMC

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

"Indeterminate cell histiocytosis" OR "Indeterminate dendritic cell neoplasm" OR "Indeterminate dendritic cell tumor" OR "indeterminate Dendritic cell tumour"

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

Parent-category trials query:

"histiocytosis"

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 2026-07-27T08:05:35.334Z