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ORPHA:457246

Clear cell sarcoma of kidney

medium confidenceDisorder

Also known as: CCSK

Publications

888

90.8th percentile

Trials

5

Interventional, condition-specific

Researchers

1,289

Distinct authors in sample

Gene link

—

Readiness

2/6

Stages with a signal

Clinical definition (Orphanet)

Clear cell sarcoma of kidney is a rare, primary, genetic renal tumor usually characterized by a unilateral, unicentric, morphologically diverse tumor that arises from the renal medulla and has a tendency for vascular invasion. Clinically it presents with a palpable abdominal mass, abdominal or flank pain, hematuria, anemia and/or fatigue. Metastatic spread to lymph nodes, bones, lungs, retroperitoneum, brain and liver is common at time of diagnosis and therefore bone pain, cough or neurological compromise may be associated. Metastasis to unusual sites, such as the scalp, neck, nasopharynx, axilla, orbits and epidural space, have been reported.

Orphanet entry

Cross-references

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

Additional Mondo synonyms (12)

childhood clear cell sarcoma of the kidney · childhood kidney clear cell sarcoma · childhood renal clear cell sarcoma · clear cell sarcoma - kidney · clear cell sarcoma of kidney · clear cell sarcoma of the kidney · kidney clear cell sarcoma · paediatric kidney clear cell sarcoma · paediatric renal clear cell sarcoma · pediatric kidney clear cell sarcoma · pediatric renal clear cell sarcoma · renal clear cell sarcoma

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

    888 matched papers (540 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

    5 matched on ClinicalTrials.gov

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?

888

888 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.

888 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).

540 in the last 10 years · medium confidence · 90.8th percentile (publications denominator)

Phrase hits: 888 · MeSH hits: 0

Open Europe PMC search

Who's working on it?

1,289

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 H9 papers · 2025

    Department of Pediatric Surgery, Shandong Provincial Hospital, Shandong University, Jinan, China.

    Papers in Europe PMC
  2. 02
    Mullen EA7 papers · 2026

    Department of Pediatric Oncology, Children's Hospital Boston, Dana-Farber Cancer Institute, Boston, MA 02215, USA.

    Papers in Europe PMC
  3. 03
    Zhang M7 papers · 2026

    Collaborating Center for the WHO Family of International Classifications in China, Beijing, China.

    Papers in Europe PMC
  4. 04
    Geller JI6 papers · 2026

    Division of Hematology/Oncology, Peckham Center for Cancer and Blood Disorders, Rady Children's Hospital, San Diego, CA 92123, USA.

    Papers in Europe PMC
  5. 05
    van den Heuvel-Eibrink MM6 papers · 2026

    Princess Máxima Center for Pediatric Oncology, Utrecht, The Netherlands.

    Papers in Europe PMC
  6. 06
    Alaggio R5 papers · 2026

    University of Pittsburgh School of Medicine, Pittsburgh, PA.

    Papers in Europe PMC
  7. 07
    Chang KTE5 papers · 2025

    VIVA-KKH Paediatric Brain and Solid Tumour Laboratory, KK Women's and Children's Hospital, Singapore.

    Papers in Europe PMC
  8. 08
    Dome JS5 papers · 2026

    Division of Pediatric Oncology, Children's National Medical Center, Washington, DC 20010, USA.

    Papers in Europe PMC
  9. 09
    He L5 papers · 2025

    Department of Radiology, Children's Hospital of Chongqing Medical University, National Clinical Research Center for Child Health and Disorders, Ministry of Education Key Laboratory of Child Development and Disorders, Chongqing Key Laboratory of Pediatrics, Chongqing, 400014, China. doctorheling@yeah.net.

    Papers in Europe PMC
  10. 10
    O'Sullivan MJ5 papers · 2025

    Histology Laboratory, Children's Health Ireland at Crumlin, Dublin, Ireland.

    Papers in Europe PMC

Clinical research

Is a treatment being tested?

5

interventional trials for this specific condition

5 interventional trials matched this specific condition name; none in our sample are currently recruiting. 24 trials are registered for clear cell sarcoma, the broader category — shown separately because they may or may not enrol this specific subtype.

Data as of 27 July 2026

5 interventional trials — more than 73% of diseases in the trials denominator have none at all (5114 of 7003; this disease is at the 87.9th percentile).

medium confidence · 87.9th percentile (trials denominator)

Recruiting interventional trials

From the matched ClinicalTrials.gov set

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

No currently recruiting studies in the matched set. Open the same search on ClinicalTrials.gov.

Broader category: clear cell sarcoma

24

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

2 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.

None of the matched observational studies is currently listed as 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.

"Clear cell sarcoma of kidney" OR "Clear cell sarcoma of the kidney" OR "childhood clear cell sarcoma of the kidney" OR "childhood clear cell sarcoma of kidney" OR "childhood kidney clear cell sarcoma" OR "childhood renal clear cell sarcoma" OR "clear cell sarcoma - kidney" OR "kidney clear cell sarcoma" OR "paediatric kidney clear cell sarcoma" OR "paediatric renal clear cell sarcoma" OR "pediatric kidney clear cell sarcoma" OR "pediatric renal clear cell sarcoma" OR "renal clear cell sarcoma"

Run this search on Europe PMC

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

"Clear cell sarcoma of kidney" OR "Clear cell sarcoma of the kidney" OR "childhood clear cell sarcoma of the kidney" OR "childhood clear cell sarcoma of kidney" OR "childhood kidney clear cell sarcoma" OR "childhood renal clear cell sarcoma" OR "clear cell sarcoma - kidney" OR "kidney clear cell sarcoma" OR "paediatric kidney clear cell sarcoma" OR "paediatric renal clear cell sarcoma" OR "pediatric kidney clear cell sarcoma" OR "pediatric renal clear cell sarcoma" OR "renal clear cell sarcoma"

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: 5 interventional · 2 observational · 0 expanded access. Only interventional studies enter the trial headline.

Parent-category trials query:

"clear cell sarcoma"

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

Run this search on ClinicalTrials.gov

Synonyms dropped by stoplist: CCSK

Confidence reasoning

  • Preferred label is multi-word and distinctive
  • 1 synonym(s) dropped by stoplist (may under-count)
  • No label/synonym collisions with other diseases in this corpus

Ingested 2026-07-27T16:49:23.798Z