ORPHA:458768
Papillary intralymphatic angioendothelioma
Also known as: Dabska tumor · PILA · Primary intralymphatic angioendothelioma
Publications
509
Trials
0
Interventional, condition-specific
Researchers
949
Distinct authors in sample
Gene link
—
Readiness
1/6
Stages with a signal
Clinical definition (Orphanet)
A rare vascular tumor characterized by an ill-defined, slowly growing, asymptomatic cutaneous plaque or nodule mostly involving the limbs, in fewer cases the trunk. The tumor is composed of lymphatic-like channels with prominent intraluminal papillary tufts with hyaline cores lined by hobnail endothelial cells. It is locally aggressive, while metastasis is rare. Infants and children are much more often affected than adults.
How rare: <1 / 1 000 000 — fewer than one in a million. In a city the size of Kolkata, that might mean on the order of fifteen people.
Cross-references
Joined from Mondo / Orphanet. MeSH labels may enter searches; UMLS / OMIM / NCIT are stored for reference.
- MONDO:0018714
- UMLS:C0346087
- NCIT:C7526
Additional Mondo synonyms (5)
Dabska tumour · malignant endothelial papillary angioendothelioma · papillary Endovascular angioendothelioma · papillary intralymphatic angioendothelioma · pila
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.”
1/6 stages with a signal
Research-stage checklist from open sources (GenCC, literature, Monarch when enriched, ClinicalTrials.gov). Not a prognosis or care recommendation.
- Gene identifiedNot found
No GenCC disease–gene assertion in this build
- LiteraturePresent
509 matched papers (256 in last 10 years) Source
- Phenotype characterisedNot checked
Not yet enriched from Monarch / HPO
- Animal modelNot checked
Not yet enriched from Monarch / Alliance
- Orphan designationNot checked
FDA/EMA orphan-drug designation not enriched yet
- Interventional trialNot found
No matched interventional trial under our ClinicalTrials.gov rules
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?
509
509 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.
509 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).
256 in the last 10 years · low confidence
Phrase hits: 509 · MeSH hits: 0
Who's working on it?
949
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.
- 01Díaz-Flores L4 papers · 2024
Department of Physical Medicine and Pharmacology, Faculty of Medicine, University of La Laguna, Tenerife, Spain.
Papers in Europe PMC - 02Li B4 papers · 2026
Department of General Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, No. 1 Shuaifuyuan, Dongcheng District, Beijing, 100730, China. pumchlibinglu@163.com.
Papers in Europe PMC - 03Fletcher CDM3 papers · 2026
Department of Pathology, Brigham and Women's Hospital, Boston, MA.
Papers in Europe PMC - 04Fraitag S3 papers · 2022
Department of Pathology, Necker-Enfants Malades Hospital, APHP, 75015 Paris, France.
Papers in Europe PMC - 05Hornick JL3 papers · 2026
Department of Pathology, Brigham and Women's Hospital and Harvard Medical School, 75 Francis Street, Boston, MA, USA. jhornick@bwh.harvard.edu.
Papers in Europe PMC - 06Li Y3 papers · 2024
Department of Cardiothoracic Surgery, National Clinical Research Center for Child Health and Disorders, Ministry of Education Key Laboratory of Child Development and Disorders Chongqing Key Laboratory of Pediatrics, Children's Hospital of Chongqing Medical University, No.136, Zhongshan 2nd Road, Yuzhong District, Chongqing, 400014, China.
Papers in Europe PMC - 07Miller JB3 papers · 2025
From the Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD.
Papers in Europe PMC - 08Requena L3 papers · 2021
Department of Dermatology, Fundación Jiménez Díaz, Universidad Autónoma, Madrid, Spain. lrequena@fjd.es
Papers in Europe PMC - 09Abdelwahed M2 papers · 2024
Department of Pathology and Laboratory Medicine, North Shore University Hospital and Long Island Jewish Medical Center, Donald and Barbara Zucker School of Medicine at Hofstra/Northwell Health, Greenvale, NY, USA.
Papers in Europe PMC - 10Al-Rohil RN2 papers · 2024
Department of Pathology, Duke University Medical Center, Durham, NC 27710-0001, USA.
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).
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.
low 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.
See who's working on it — people publishing on this disease are often the practical next contact when no trial is listed.
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 2026-07-26This 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 intralymphatic angioendothelioma" OR "Dabska tumor" OR "Primary intralymphatic angioendothelioma" OR "Dabska tumour" OR "malignant endothelial papillary angioendothelioma" OR "papillary Endovascular angioendothelioma"
ClinicalTrials.gov query (quoted phrases + MeSH via query.cond, plus recall-expansion terms when used):
"Papillary intralymphatic angioendothelioma" OR "Dabska tumor" OR "Primary intralymphatic angioendothelioma" OR "Dabska tumour" OR "malignant endothelial papillary angioendothelioma" OR "papillary Endovascular angioendothelioma"
Study-type breakdown: 0 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
Synonyms dropped by stoplist: PILA
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 (509) is implausibly high for prevalence class "<1 / 1 000 000" — treat as possible over-matching, not a measure of research intensity
Ingested 2026-07-27T16:52:31.272Z