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pancreatoblastoma - Clinical Profile, Genes & Research | Kisho | Kisho

Rare Disease

pancreatoblastoma

MONDO:0019035

Also known as:pancreatoblastoma (morphologic abnormality)PB

Overview

Pancreatoblastoma is a rare malignant epithelial pancreatic neoplasm, most often found in children, which usually presents with the non-specific symptoms of a palpable mass, vomiting abdominal pain, jaundice, and weight loss/failure to thrive, and is characterized histologically by multiple lines of differentiation (acinar, ductal, mesenchymal, neuroendocrine) and the presence of squamoid nests.

Diagnosis

Biomarker and diagnostic research for pancreatoblastoma has been reported in the published literature.

Prognosis

Estimated prevalence: <1 in 1,000,000 (VERY_RARE).

Research Landscape

Sourced from ClinicalTrials.gov and PubMed. Publication types are classified using NLM-curated article metadata; research type breakdowns reflect the nature of published work for this disease.

No clinical trials have been registered for pancreatoblastoma.

44 publications have been identified in PubMed for pancreatoblastoma. Research spans Case Report / Case Series (32%), Review / Meta-Analysis (20%), and Epidemiology / Natural History (14%).

Research Type	Count	% of Total
Patient case studies	14	32%

Data assembled from 3 of 12 sources · Last updated Sep 19, 2026, 2:25 PM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

Orphanet

European rare disease database

Orphanet:677

GARD

Genetic and Rare Diseases Info Center

GARD:4210

Questions for Your Care Team

→Are there specialists or centers of excellence for this condition in my area?
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pancreatoblastoma

Overview

Diagnosis

Prognosis

Research Landscape

Medical References

External Resources

Orphanet

GARD

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Questions for Your Care Team

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