Pancreatic acinar cell carcinoma

Summary

Pancreatic acinar cell carcinoma (MONDO:0006346) is a cancer and 28 clinical trials. Molecularly, KANK4::ALK Fusion confers sensitivity to Alectinib in Pancreatic Acinar Cell Adenocarcinoma (CIViC Level C). Top therapeutic interventions include metformin, vismodegib, and cisplatin. A subtype of acinar cell carcinoma — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

• Classification: Cancer
• Prevalence: <1 / 1 000 000 (Europe) [Orphanet-validated]
• Clinical trials: 28
• Precision-medicine evidence (CIViC): 1 subtype–drug association

Clinical features

Epidemiology

Prevalence records

21 prevalence record(s), Orphanet, top 20 (validated / broadest geography first):

Type	Class	Value	Geography	Validation
Annual incidence	<1 / 1 000 000	0.029	Europe	Validated
Annual incidence	<1 / 1 000 000	0.049	Austria	Validated
Annual incidence	<1 / 1 000 000	0.039	Belgium	Validated
Annual incidence	<1 / 1 000 000	0.034	Bulgaria	Validated
Annual incidence	<1 / 1 000 000	0.006	Croatia	Validated
Annual incidence	<1 / 1 000 000	0.044	Czech Republic	Validated
Annual incidence	<1 / 1 000 000	0.083	Estonia	Validated
Annual incidence	<1 / 1 000 000	0.024	Finland	Validated
Annual incidence	<1 / 1 000 000	0.01	France	Validated
Annual incidence	<1 / 1 000 000	0.058	Germany	Validated
Annual incidence	<1 / 1 000 000	0.028	Ireland	Validated
Annual incidence	<1 / 1 000 000	0.039	Italy	Validated
Annual incidence	<1 / 1 000 000	0.07	Latvia	Validated
Annual incidence	<1 / 1 000 000	0.027	Norway	Validated
Annual incidence	<1 / 1 000 000	0.005	Poland	Validated
Annual incidence	<1 / 1 000 000	0.02	Portugal	Validated
Annual incidence	<1 / 1 000 000	0.05	Slovakia	Validated
Annual incidence	<1 / 1 000 000	0.024	Spain	Validated
Annual incidence	<1 / 1 000 000	0.039	Switzerland	Validated
Annual incidence	<1 / 1 000 000	0.017	Netherlands	Validated

Identifiers

Disease identifiers

Field	Value
Canonical name	pancreatic acinar cell carcinoma
Mondo ID	MONDO:0006346
EFO	EFO:1000439
Orphanet	424046
DOID	DOID:5742
ICD-11	449785448
NCIT	C7977
UMLS	C0279661
MedGen	83540
GARD	0021777
Is cancer (heuristic)	yes

Also known as: acinar cell adenocarcinoma of pancreas · acinar cell adenocarcinoma of the pancreas · acinar cell carcinoma of pancreas · acinar cell carcinoma of the pancreas · carcinoma of pancreatic acinar cell · PAAC · pancreas acinar cell adenocarcinoma · pancreatic acinar cell adenocarcinoma · Pancreatic acinar cell cancer · pancreatic acinar cell carcinoma

Data availability: 3 cell lines.

Disease family

This is a subtype of acinar cell carcinoma. 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 etiologic mechanism › cancer or benign tumor › neoplastic disease or syndrome › neoplasm › cancer › carcinoma › adenocarcinoma › acinar cell carcinoma › pancreatic acinar cell carcinoma

Related subtypes (4): prostatic acinar adenocarcinoma, acinic cell breast carcinoma, parotid gland acinic cell carcinoma, salivary gland acinic cell carcinoma

Subtypes (1): pancreatic acinar cell cystadenocarcinoma

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: 28.

Phase distribution (across all retrieved trials)

Phase	Trials
PHASE2	12
PHASE1	12
EARLY_PHASE1	2
PHASE2/PHASE3	1
PHASE1/PHASE2	1

Top trials by phase / activity

NCT	Phase	Status	Title
NCT06115499	PHASE2/PHASE3	ACTIVE_NOT_RECRUITING	The PLATINUM Trial: Optimizing Chemotherapy for the Second-Line Treatment of Metastatic BRCA1/2 or PALB2-Associated Metastatic Pancreatic Cancer
NCT02834013	PHASE2	ACTIVE_NOT_RECRUITING	Nivolumab and Ipilimumab in Treating Patients With Rare Tumors
NCT04858334	PHASE2	RECRUITING	APOLLO: A Randomized Phase II Double-Blind Study of Olaparib Versus Placebo Following Curative Intent Therapy in Patients With Resected Pancreatic Cancer and a Pathogenic BRCA1, BRCA2 or PALB2 Mutation
NCT00031694	PHASE2	COMPLETED	Paclitaxel and Bryostatin 1 in Treating Patients With Advanced Pancreatic Cancer
NCT00085371	PHASE2	COMPLETED	Triapine as First-Line or Second-Line Therapy in Treating Patients With Locally Advanced or Metastatic Cancer of the Pancreas
NCT00397787	PHASE2	COMPLETED	Sunitinib in Treating Patients With Metastatic Pancreatic Cancer That Progressed After First-Line Therapy With Gemcitabine
NCT00416793	PHASE2	TERMINATED	Bortezomib and Carboplatin in Treating Patients With Metastatic Pancreatic Cancer
NCT01234935	PHASE2	COMPLETED	Dasatinib and Gemcitabine Hydrochloride or Gemcitabine Hydrochloride Alone in Treating Patients With Pancreatic Cancer Previously Treated With Surgery
NCT01280058	PHASE2	COMPLETED	Carboplatin and Paclitaxel With or Without Viral Therapy in Treating Patients With Recurrent or Metastatic Pancreatic Cancer
NCT01658943	PHASE2	COMPLETED	Selumetinib and Akt Inhibitor MK2206 or mFOLFOX Therapy Comprising Oxaliplatin and Fluorouracil in Treating Patients With Metastatic Pancreatic Cancer Previously Treated With Chemotherapy
NCT01666730	PHASE2	COMPLETED	Metformin Plus Modified FOLFOX 6 in Metastatic Pancreatic Cancer
NCT02047474	PHASE2	COMPLETED	Combination Chemotherapy Before and After Surgery in Treating Patients With Localized Pancreatic Cancer
NCT02178436	PHASE1/PHASE2	COMPLETED	Gemcitabine, Nab-paclitaxel and KPT-330 in Advanced Pancreatic Cancer
NCT05286827	PHASE2	TERMINATED	Olaparib in Subjects With Advanced Pancreatic Acinar Cell Carcinoma
NCT00669734	PHASE1	ACTIVE_NOT_RECRUITING	Vaccine Therapy and Sargramostim in Treating Patients With Pancreas Cancer That Cannot Be Removed By Surgery
NCT00878163	PHASE1	ACTIVE_NOT_RECRUITING	GDC-0449 and Erlotinib Hydrochloride With or Without Gemcitabine Hydrochloride in Treating Patients With Metastatic Pancreatic Cancer or Solid Tumors That Cannot Be Removed by Surgery
NCT04150042	PHASE1	RECRUITING	SHARON: A Clinical Trial for Metastatic Cancer Using Chemotherapy and Patients’ Own Stem Cells
NCT00981162	PHASE1	COMPLETED	Sorafenib Tosylate and Everolimus in Treating Patients With Advanced Solid Tumors and Metastatic Pancreatic Cancer That Does Not Respond to Gemcitabine Hydrochloride
NCT01537107	PHASE1	COMPLETED	Sirolimus and Vismodegib in Treating Patients With Solid Tumors or Pancreatic Cancer That is Metastatic or Cannot Be Removed By Surgery
NCT01643499	PHASE1	COMPLETED	Genotype-guided Dosing of mFOLFIRINOX Chemotherapy in Patients With Previously Untreated Advanced Gastrointestinal Malignancies
NCT01739439	PHASE1	TERMINATED	Chemoradiation and Radiosurgery Boost in Treating Patients With Locally Advance Pancreatic Cancer That May or May Not be Removed by Surgery
NCT01741597	PHASE1	WITHDRAWN	Dynamic Contrast Enhanced MRI in Patients With Advanced Breast or Pancreatic Cancer With Metastases to the Liver or Lung
NCT01770132	PHASE1	COMPLETED	Ultrasound-Guided Photodynamic Therapy With Photofrin & Gemcitabine for Patients With Locally Advanced Pancreatic Cancer
NCT01835041	PHASE1	COMPLETED	CPI-613 and Combination Chemotherapy in Treating Patients With Metastatic Pancreatic Cancer
NCT01839981	PHASE1	COMPLETED	CPI-613 in Treating Patients With Locally Advanced or Metastatic Pancreatic Cancer
NCT01924260	PHASE1	COMPLETED	Alisertib and Gemcitabine Hydrochloride in Treating Patients With Solid Tumors or Pancreatic Cancer
NCT01821612	EARLY_PHASE1	COMPLETED	Chemotherapy and Radiation Therapy Before Surgery Followed by Gemcitabine in Treating Patients with Pancreatic Cancer
NCT02153450	EARLY_PHASE1	COMPLETED	Stereotactic Radiosurgery and Metformin in Patients With Borderline-Resectable or Locally-Advanced Pancreatic Cancer

Drugs tested across these trials (top 30)

Molecule	Max phase	Trials referencing
METFORMIN	4	2
VISMODEGIB	4	2
CISPLATIN	4	1
PORFIMER SODIUM	4	1
SELINEXOR	4	1
SELUMETINIB	4	1
SUNITINIB MALATE	4	1
CPI 613	3	2
ALISERTIB	3	1
TRIAPINE	3	1
AQUACOBALAMIN	2	1
BRYOSTATIN 1	2	1
CHEMBL3109278	0	1
CHEMBL4463209	0	1
CHEMBL454299	0	1

Precision-medicine subtype map (CIViC)

Drug × molecular subtype: 1 predictive associations from 1 curated evidence items.

Molecular subtype	Therapy	Effect	Level	CIViC
KANK4::ALK Fusion	Alectinib	Sensitivity/Response	CIViC C	EID10327