Solid pseudopapillary neoplasm of the pancreas

Summary

Solid pseudopapillary neoplasm of the pancreas (MONDO:0044786) is a cancer and 12 clinical trials. Top therapeutic interventions include tegavivint. A subtype of malignant exocrine pancreas neoplasm — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

• Classification: Cancer
• Clinical trials: 12

Clinical features

No curated clinical features (Orphanet) for this disease.

Identifiers

Disease identifiers

Field	Value
Canonical name	solid pseudopapillary neoplasm of the pancreas
Mondo ID	MONDO:0044786
NCIT	C37212
UMLS	C1336030
MedGen	234800
GARD	0027975
Is cancer (heuristic)	yes

Also known as: Frantz tumor · Frantz tumour · solid pseudopapillary neoplasm of the pancreas · solid pseudopapillary tumor of the pancreas · solid pseudopapillary tumour of the pancreas

Disease family

This is a subtype of malignant exocrine pancreas neoplasm. Genetic, therapeutic, and trial evidence is largely curated at the broader-term level — see the parent page for the associated-gene cohort and molecular evidence.

Classification path: disease › human disease › disease by body system or component › digestive system disorder › digestive system cancer › malignant pancreatic neoplasm › malignant exocrine pancreas neoplasm › solid pseudopapillary neoplasm of the pancreas

Related subtypes (3): pancreatic intraductal papillary-mucinous neoplasm, exocrine pancreatic carcinoma, pancreatoblastoma

Genetics & variants

GWAS landscape

No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.

Variant details and genetic-evidence tiers

No tiered GWAS variants or ClinVar records for this disease.

Genes & proteins

No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).

Function

No pathway enrichment — requires an associated-gene cohort.

Therapeutics

No druggable-target or therapeutic data for this disease’s cohort.

Clinical trials & evidence

Clinical trials

Clinical trials: 12.

Phase distribution (across all retrieved trials)

Phase	Trials
Not specified	11
PHASE1/PHASE2	1

Top trials by phase / activity

NCT	Phase	Status	Title
NCT04851119	PHASE1/PHASE2	RECRUITING	Tegavivint for the Treatment of Recurrent or Refractory Solid Tumors, Including Lymphomas and Desmoid Tumors
NCT05604716	Not specified	RECRUITING	Cross-sectional Follow-up on Digestive and Social Functions of SPN
NCT06167421	Not specified	NOT_YET_RECRUITING	Comparison of Kimura Versus Warshaw Technique for Laparoscopic Spleen-Preserving Distal Pancreatectomy
NCT06753318	Not specified	NOT_YET_RECRUITING	Validation of Joint-AI in Diagnosing Pancreatic Solid Lesions
NCT07183280	Not specified	RECRUITING	Multicentric Database for Solid Pseudopapillary Neoplasm of the Pancreas
NCT02110498	Not specified	COMPLETED	Early Detection of Pancreatic Cystic Neoplasms
NCT02494388	Not specified	TERMINATED	Needle-based Confocal Laser Endomicroscopy on Pancreatic Cystic Lesions
NCT03884179	Not specified	UNKNOWN	Diagnosis of PCL With EUS-FNA and Cross-sectional Imaging - A Report of Accuracy
NCT04728750	Not specified	COMPLETED	Laparoscopic Enucleation of Frantz’s Tumor of the Pancreas
NCT06024343	Not specified	COMPLETED	Minimally Invasive Pancreatic Enucleation With Main Pancreatic Duct Exposure, Repair or Reconstruction
NCT06557408	Not specified	COMPLETED	Pancreatic Fistula After Minimally Invasive Enucleation
NCT07592663	Not specified	COMPLETED	Oncologic Safety and Lymphadenectomy in Parenchyma-Sparing Resection for SPN

Drugs tested across these trials (top 30)

Molecule	Max phase	Trials referencing
TEGAVIVINT	2	1
CHEMBL6083129	0	1