RARE DISEASERESEARCH ATLAS

ORPHA:168829

Primary peritoneal carcinoma

low confidenceDisorder

Also known as: EOPPC · Extra-ovarian primary peritoneal carcinoma · PPC · Primary peritoneal serous carcinoma · Serous surface papillary carcinoma

Publications

4,812

Trials

536

Interventional, condition-specific

Researchers

1,350

Distinct authors in sample

Gene link

Readiness

4/6

Stages with a signal

Clinical definition (Orphanet)

Primary peritoneal carcinoma (PPC) is a rare malignant tumor of the peritoneal cavity of extra-ovarian origin, clinically and histologically similar to advanced-stage serous ovarian carcinoma.

How rare: How common this is has not been clearly measured.

Orphanet entry

Cross-references

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

Additional Mondo synonyms (5)

primary peritoneal cancer · primary peritoneal carcinoma · primary peritoneal carcinoma (disease) · primary peritoneal serous carcinoma · serous surface papillary carcinoma

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.”

4/6 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

    4,812 matched papers (3,329 in last 10 years) Source

  3. Phenotype characterisedPresent

    6 HPO annotations (e.g. Nausea and vomiting; Constipation; Peritonitis) Source

  4. Animal modelNot found

    No Alliance genotype “model of” associations via Monarch for these Mondo IDs

  5. Orphan designationPresent

    3 FDA · 1 EMA designations (1 FDA orphan-indication approval) — e.g. pelareorep Source

  6. Interventional trialPresent

    536 matched on ClinicalTrials.gov (73 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?

Not yet — the cause hasn't been pinned down in GenCC.

No strong gene–disease assertion joined for this Orphanet entity.

Phenotypes (Monarch / HPO)

6

Associated phenotypes · MONDO:0015686

  • Nausea and vomiting
  • Constipation
  • Peritonitis
  • Neoplasm
  • Abdominal distention

Showing 5 of 6 — open Monarch for the full list.

Animal models (Monarch / Alliance)

None returned for this Mondo ID. Empty here is not proof that no model organism work exists under another name or gene.

Monarch fetch 2026-07-29

Therapies

Designations, candidates, and chemicals

FDA OOPD and EMA orphan designations, Open Targets clinical candidates, and CTD chemical associations via MyDisease.info. These never change the interventional-trial headline.

Orphan designation (FDA · EMA)

4

Designations · 1 with FDA orphan-indication approval

  • FDA pelareorepPrimary Peritoneal Cancer · 2015-02-24 · Not FDA Approved for Orphan Indication
  • FDA olaparib (Lynparza)Primary Peritoneal Cancer · 2018-05-15
  • FDA bevacizumab (Avastin)Primary peritoneal carcinoma · 2010-11-02
  • EMA patupiloneTreatment of primary peritoneal cancer · 05/11/2009 · WithdrawnEMA designation

Sources: FDA OOPD · EMA orphan designations

Open Targets candidates

252

Drugs / clinical candidates · MONDO_0015686

CTD chemicals (MyDisease.info)

No CTD chemical associations returned for this Mondo ID.

Literature

Is anyone studying this?

4,812

4,812 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=3967) is 59.

4,812 papers since the earliest indexed year in this search — median last-10-year count for a rare disease in this dataset is 59 (publications denominator n=3967).

3,329 in the last 10 years · low confidence

Phrase hits: 4,812 · MeSH hits: 0

Open Europe PMC search

Who's working on it?

1,350

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
    Aida T4 papers · 2026

    Department of Obstetrics and Gynecology, Hachinohe Red Cross Hospital, Aomori 039-1104, Japan.

    Papers in Europe PMC
  2. 02
    Baba T4 papers · 2026

    Department of Obstetrics and Gynecology, Iwate Medical University School of Medicine, Yahaba 028-3695, Japan.

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

    Department of Obstetrics and Gynecology, Iwate Medical University School of Medicine, Yahaba 028-3695, Japan.

    Papers in Europe PMC
  4. 04
    Jo A4 papers · 2026

    Department of Obstetrics and Gynecology, Iwate Medical University School of Medicine, Yahaba 028-3695, Japan.

    Papers in Europe PMC
  5. 05
    Jonai N4 papers · 2026

    Department of Obstetrics and Gynecology, Iwate Medical University School of Medicine, Yahaba 028-3695, Japan.

    Papers in Europe PMC
  6. 06
    Kagabu M4 papers · 2026

    Department of Obstetrics and Gynecology, Iwate Medical University School of Medicine, Yahaba 028-3695, Japan.

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

    Department of Obstetrics and Gynecology, Iwate Medical University School of Medicine, Yahaba 028-3695, Japan.

    Papers in Europe PMC
  8. 08
    Mądry R4 papers · 2025

    Oncological Gynaecology Department, Poznan University of Medical Sciences, 60-569 Poznań, Poland.

    Papers in Europe PMC
  9. 09
    Nagasawa T4 papers · 2026

    Department of Obstetrics and Gynecology, Iwate Medical University School of Medicine, Yahaba 028-3695, Japan.

    Papers in Europe PMC
  10. 10
    Sato S4 papers · 2026

    Department of Obstetrics and Gynecology, Iwate Medical University School of Medicine, Yahaba 028-3695, Japan.

    Papers in Europe PMC

Clinical research

Is a treatment being tested?

536

interventional trials for this specific condition

536 interventional trials matched this specific condition name; 73 currently recruiting in our sample. 30 trials are registered for peritoneal carcinoma, the broader category — shown separately because they may or may not enrol this specific subtype.

Data as of 11 September 2026

536 interventional trials — more than 77.2% of diseases in the trials denominator have none at all (5501 of 7126; this disease is at the 99.7th percentile).

low confidence · 99.7th percentile (trials denominator)

Recruiting interventional trials

From the matched ClinicalTrials.gov set

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

Broader category: peritoneal carcinoma

30

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

44 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

Other registries (secondary)

Broader net from EU CTIS, ISRCTN, and ICTRP when available — deduped against ClinicalTrials.gov IDs already counted above. Dual-model LLM relevance gates what we keep. These rows are not added to the interventional headline.

raw 43 · after dedupe 43 · already on CT.gov 0 · kept 0 · parent 0 · uncertain 43 · dropped 0 · fetched 2026-07-30

Source notes: ictrp: Error: ICTRP public search unavailable (WHO portal is SPA-only; SOAP needs partnership). Tried: https://apps.who.int/tri

No secondary-registry studies passed dual-model relevance for this condition name (after dedupe).

Uncertain / not reviewed (43)

Where to find support

Condition-specific patient organisations, when Orphanet lists them, are on the disease’s Orphanet page. We also link umbrella groups that support undiagnosed and ultra-rare families.

Orphanet entry for Primary peritoneal carcinoma — check Associations / patient organisations on that page.

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.

"Primary peritoneal carcinoma" OR "EOPPC" OR "Extra-ovarian primary peritoneal carcinoma" OR "Primary peritoneal serous carcinoma" OR "Serous surface papillary carcinoma" OR "primary peritoneal cancer" OR "primary peritoneal carcinoma (disease)"

Run this search on Europe PMC

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

"Primary peritoneal carcinoma" OR "EOPPC" OR "Extra-ovarian primary peritoneal carcinoma" OR "Primary peritoneal serous carcinoma" OR "Serous surface papillary carcinoma" OR "primary peritoneal cancer" OR "primary peritoneal carcinoma (disease)"

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: 536 interventional · 44 observational · 1 expanded access. Only interventional studies enter the trial headline.

Parent-category trials query:

"peritoneal carcinoma"

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

Run this search on ClinicalTrials.gov

Synonyms dropped by stoplist: PPC

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
  • Publication count (4812) is extremely high with unknown/missing prevalence — treat as possible over-matching, not proven research intensity

Ingested 2026-07-27T08:31:31.271Z