Details of Disease

General Information of Disease (ID: DISY1IQQ)

Disease Name Pancreatic acinar cell carcinoma
Synonyms
PAAC; Pancreatic acinar cell cancer; pancreatic acinar cell carcinoma; acinar cell carcinoma of the pancreas; carcinoma of pancreatic acinar cell; pancreas acinar cell adenocarcinoma; pancreatic acinar cell adenocarcinoma; acinar cell adenocarcinoma of pancreas; acinar cell carcinoma of pancreas; acinar cell adenocarcinoma of the pancreas
Definition
An adenocarcinoma arising from the pancreas. It is characterized by the presence of relatively uniform malignant cells which form acinar patterns. It usually occurs during adulthood. Signs and symptoms include abdominal pain, weight loss, nausea, and diarrhea. It may metastasize to regional lymph nodes and the liver. A minority of patients develop lipase hypersecretion syndrome. This syndrome may be seen in patients with liver metastases and it is characterized by excessive secretion of lipase in the serum, polyarthralgia, and subcutaneous fat necrosis.
Disease Hierarchy
DIS37Y0J: Acinar cell carcinoma
DISH9F1N: Carcinoma
DISKHX7S: Pancreatic adenocarcinoma
DISY1IQQ: Pancreatic acinar cell carcinoma
Disease Identifiers
MONDO ID
MONDO_0006346
UMLS CUI
C0279661
MedGen ID
83540
Orphanet ID
424046
SNOMED CT ID
783771003

Drug-Interaction Atlas (DIA) of This Disease

Drug-Interaction Atlas (DIA)
This Disease is Treated as An Indication in 2 Approved Drug(s)
Drug Name Drug ID Highest Status Drug Type REF
Everolimus DM8X2EH Approved Small molecular drug [1]
Sirolimus DMGW1ID Approved Small molecular drug [2]
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Molecular Interaction Atlas (MIA) of This Disease

Molecular Interaction Atlas (MIA)
This Disease Is Related to 1 DTT Molecule(s)
Gene Name DTT ID Evidence Level Mode of Inheritance REF
CELA1 TT3NKIB Strong Biomarker [3]
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This Disease Is Related to 1 DOT Molecule(s)
Gene Name DOT ID Evidence Level Mode of Inheritance REF
BCL10 OT47MCLI Strong Altered Expression [4]
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References

1 Everolimus FDA Label
2 Sirolimus FDA Label
3 Murine Pancreatic Acinar Cell Carcinoma Growth Kinetics Are Independent of Dietary Vitamin D Deficiency or Supplementation.Front Oncol. 2017 Jun 28;7:133. doi: 10.3389/fonc.2017.00133. eCollection 2017.
4 Cytological findings and BCL10 expression in pancreatic acinar cell carcinoma: A Case Report.Diagn Cytopathol. 2017 Mar;45(3):247-251. doi: 10.1002/dc.23634. Epub 2016 Nov 17.