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ORPHA:319298

Papillary renal cell carcinoma

high confidenceDisorder

Also known as: Papillary renal cell adenocarcinoma

Publications

4,945

96.6th percentile

Trials

38

Interventional, condition-specific

Researchers

1,338

Distinct authors in sample

Gene link

MET

Definitive

Readiness

3/6

Stages with a signal

Clinical definition (Orphanet)

Papillary renal cell carcinoma is a rare subtype of renal cell carcinoma, arising from the renal tubular epithelium and showing a papillary growth pattern, which typically manifests with hematuria, flank pain, palpable abdominal mass or nonspecific symptoms, such as fatigue, weight loss or fever. Symptoms related to metastatic spread, such as bone pain or persistent cough, are frequently associated since early diagnosis is not common. It is typically multifocal, bilateral, and in most cases sporadic, although different syndromes, such as leiomyoma renal cell carcinoma, Birt-Hogg-Dubé syndrome and Tuberous sclerosis, may predispose to the development of papillary renal cell carcinoma.

How rare: 1-9 / 1 000 000 — roughly one to nine people per million. In a city the size of Kolkata, perhaps a few dozen.

Orphanet entry

Cross-references

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

Additional Mondo synonyms (9)

HPRCC · chromophil carcinoma of kidney · chromophil carcinoma of the kidney · chromophil renal cell carcinoma · papillary (chromophil) renal cell carcinoma · papillary renal cell adenocarcinoma · papillary renal cell cancer · papillary renal cell carcinoma · renal cell carcinoma, papillary, type 1

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 identifiedPresent

    Definitive — MET

  2. LiteraturePresent

    4,945 matched papers (3,433 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

    38 matched on ClinicalTrials.gov (6 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?

Yes — we know a specific gene responsible (MET).

GenCC classification: Definitive.

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?

4,945

4,945 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.

4,945 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).

3,433 in the last 10 years · high confidence · 96.6th percentile (publications denominator)

Phrase hits: 4,945 · MeSH hits: 0

Open Europe PMC search

Who's working on it?

1,338

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
    Wang Y10 papers · 2026

    Department of Central Laboratory, Peking University First Hospital, Beijing, China.

    Papers in Europe PMC
  2. 02
    Li X8 papers · 2026

    Department of Pathology, Central Hospital Affiliated to Shandong First Medical University, Jinan, China.

    Papers in Europe PMC
  3. 03
    Liu Y5 papers · 2026

    Department of Pathology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China.

    Papers in Europe PMC
  4. 04
    Rouleau E5 papers · 2025

    Service de Génétique, Institut Gustave Roussy, Villejuif, France.

    Papers in Europe PMC
  5. 05
    Zhou J5 papers · 2026

    Department of Urology, Peking University First Hospital, Beijing, China.

    Papers in Europe PMC
  6. 06
    Bernreuther C4 papers · 2026

    Institute of Pathology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.

    Papers in Europe PMC
  7. 07
    Chen Y4 papers · 2026

    Department of Urology, Sir Run Run Shaw Hospital, Zhejiang University School of Medicine, Hangzhou, China.

    Papers in Europe PMC
  8. 08
    Clauditz TS4 papers · 2026

    Institute of Pathology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.

    Papers in Europe PMC
  9. 09
    Gorbokon N4 papers · 2026

    Institute of Pathology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.

    Papers in Europe PMC
  10. 10
    Heidenreich A4 papers · 2026

    Department of Urology, Uro-Oncology, Robot-Assisted and Specialized Urologic Surgery, University Hospital Cologne, Kerpener Str. 62, 50937 , Cologne, Germany.

    Papers in Europe PMC

Clinical research

Is a treatment being tested?

38

interventional trials for this specific condition

38 interventional trials matched this specific condition name; 6 currently recruiting in our sample. 1,135 trials are registered for renal cell carcinoma, the broader category — shown separately because they may or may not enrol this specific subtype.

Data as of 27 July 2026

38 interventional trials — more than 73% of diseases in the trials denominator have none at all (5114 of 7003; this disease is at the 96.4th percentile).

high confidence · 96.4th percentile (trials denominator)

Recruiting interventional trials

From the matched ClinicalTrials.gov set

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

Broader category: renal cell carcinoma

1,135

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

Observational and natural-history studies

5 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.

"Papillary renal cell carcinoma" OR "Papillary renal cell adenocarcinoma" OR "HPRCC" OR "chromophil carcinoma of kidney" OR "chromophil carcinoma of the kidney" OR "chromophil renal cell carcinoma" OR "papillary (chromophil) renal cell carcinoma" OR "papillary renal cell cancer" OR "renal cell carcinoma, papillary, type 1"

Run this search on Europe PMC

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

"Papillary renal cell carcinoma" OR "Papillary renal cell adenocarcinoma" OR "HPRCC" OR "chromophil carcinoma of kidney" OR "chromophil carcinoma of the kidney" OR "chromophil renal cell carcinoma" OR "papillary (chromophil) renal cell carcinoma" OR "papillary renal cell cancer" OR "renal cell carcinoma, papillary, type 1"

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: 38 interventional · 5 observational · 0 expanded access. Only interventional studies enter the trial headline.

Parent-category trials query:

"renal cell carcinoma"

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 2026-07-27T13:24:16.125Z