RARE DISEASERESEARCH ATLAS

ORPHA:251919

Pineal parenchymal tumor of intermediate differentiation

high confidenceDisorder

Publications

202

73.8th percentile

Trials

1

Interventional, condition-specific

Researchers

1,341

Distinct authors in sample

Gene link

Readiness

2/6

Stages with a signal

Clinical definition (Orphanet)

A rare type of pineal parenchymal tumor (PPT) of intermediate-grade malignancy manifesting with visual disturbances, headaches, loss of coordination and balance, nausea and vomiting due to obstructive hydrocephalus, and that is classified as either grade II PPTID (pineal parenchymal tumor of intermediate differentiation) or grade III PPTID according to the degree of neuronal differentiation and mitotic activity.

Orphanet entry

Cross-references

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

Additional Mondo synonyms (3)

pineal parenchymal tumor of intermediate differentiation · pineal parenchymal tumor of intermediate differentiation (morphologic abnormality) · pineal parenchymal tumour of intermediate differentiation (morphologic abnormality)

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

    202 matched papers (149 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

    1 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?

202

202 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.

202 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).

149 in the last 10 years · high confidence · 73.8th percentile (publications denominator)

Phrase hits: 202 · MeSH hits: 0

Open Europe PMC search

Who's working on it?

1,341

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
    Orr BA7 papers · 2026

    Department of Pathology, St. Jude Children's Research Hospital, Memphis, TN, USA.

    Papers in Europe PMC
  2. 02
    Snuderl M7 papers · 2025

    Division of Neuropathology, NYU Langone Health, New York, USA.

    Papers in Europe PMC
  3. 03
    Capper D6 papers · 2025

    Department of Neuropathology, University Hospital Heidelberg, Heidelberg, Germany. david.capper@charite.de.

    Papers in Europe PMC
  4. 04
    Pfister SM6 papers · 2025

    Hopp Children's Cancer Center, at the NCT Heidelberg (KiTZ), Heidelberg, Germany.

    Papers in Europe PMC
  5. 05
    Figarella-Branger D5 papers · 2021

    Aix-Marseille University, National Center for Scientific Research, Institute of Neuro-Physiopathology, Marseille, France.

    Papers in Europe PMC
  6. 06
    Gajjar A5 papers · 2026

    Department of Oncology, St. Jude Children's Research Hospital, Memphis, TN, USA.

    Papers in Europe PMC
  7. 07
    Jones DTW5 papers · 2026

    Hopp Children's Cancer Center, at the NCT Heidelberg (KiTZ), Heidelberg, Germany.

    Papers in Europe PMC
  8. 08
    Klimo P5 papers · 2026

    Department of Neurosurgery, University of Tennessee Health Science Center, USA Le Bonheur Neuroscience Institute, Le Bonheur Children's Hospital, USA Semmes-Murphey Neurologic and Spine Institute, USA Division of Neurosurgery, St Jude Children's Hospital, USA.

    Papers in Europe PMC
  9. 09
    Liu APY5 papers · 2026

    Department of Oncology, St. Jude Children's Research Hospital, Memphis, TN, USA.

    Papers in Europe PMC
  10. 10
    Ng HK5 papers · 2024

    Department of Anatomical and Cellular Pathology, The Chinese University of Hong Kong, Shatin, New Territories, Hong Kong, China.

    Papers in Europe PMC

Clinical research

Is a treatment being tested?

1

interventional trials for this specific condition

1 interventional trial matched this specific condition name; none in our sample are currently recruiting.

Data as of 27 July 2026

1 interventional trial — more than 73% of diseases in the trials denominator have none at all (5114 of 7003; this disease is at the 76.8th percentile).

high confidence · 76.8th percentile (trials denominator)

Recruiting interventional trials

From the matched ClinicalTrials.gov set

1 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.

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.

"Pineal parenchymal tumor of intermediate differentiation" OR "Pineal parenchymal tumor of the intermediate differentiation" OR "pineal parenchymal tumor of intermediate differentiation (morphologic abnormality)" OR "pineal parenchymal tumor of the intermediate differentiation (morphologic abnormality)" OR "pineal parenchymal tumour of intermediate differentiation (morphologic abnormality)" OR "pineal parenchymal tumour of the intermediate differentiation (morphologic abnormality)"

Run this search on Europe PMC

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

"Pineal parenchymal tumor of intermediate differentiation" OR "Pineal parenchymal tumor of the intermediate differentiation" OR "pineal parenchymal tumor of intermediate differentiation (morphologic abnormality)" OR "pineal parenchymal tumor of the intermediate differentiation (morphologic abnormality)" OR "pineal parenchymal tumour of intermediate differentiation (morphologic abnormality)" OR "pineal parenchymal tumour of the intermediate differentiation (morphologic abnormality)"

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: 1 interventional · 0 observational · 0 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 2026-07-27T10:55:06.808Z