Acinar Cell CA : Clinica Features
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Acinar Cell CA of Pancreas - Clinical Features: The average age at diagnosis is 60 years but it may occur in children. It is more common in men (M:F=2:1). Most cases are sporadic. Rare cases have been associated with Lynch syndrome, Carney complex, or familial adenomatous polyposis.
The clinical presentation may be similar to that for pancreatic ductal adenocarcinoma. Most patients have symptoms related to tumor growth, including abdominal pain, nausea, vomiting, and weight loss. Jaundice is rare. Young patients may develop elevated AFP levels.
Lipase Hypersecretion Syndrome (LHS): LHS is a paraneoplastic syndrome that develops in about 10%-15% of patients with pancreatic acinar cell carcinoma, usually in the setting of liver metastases. LHS consists of peripheral fat necrosis, polyarthralgias, and eosinophilia. Pancreatic acinar enzymes (lipase, trypsin and chymotrypsin) which are normally secreted to the duodenum for digestion, are instead released into the blood by the tumor cells - a phenomenon that has been aptly described as endocrine-ization of an exocrine function. High serum lipase levels cause uncontrolled lipolysis, especially in subcutaneous tissues in the pressure points of legs as well as in the intra-articular and peri-articular adipose tissues. The resultant inflammation as well as bacterial super-infection causes painful symmetric enlargement of knee and ankle joints.
About this image:: Acinar cell carcinoma showing an encapsulated area of the tumor which has predominantly acinar architectural pattern. Normal pancreas is on the upper left. Image courtesy of: @Kusaurap; used with permission.
The clinical presentation may be similar to that for pancreatic ductal adenocarcinoma. Most patients have symptoms related to tumor growth, including abdominal pain, nausea, vomiting, and weight loss. Jaundice is rare. Young patients may develop elevated AFP levels.
Lipase Hypersecretion Syndrome (LHS): LHS is a paraneoplastic syndrome that develops in about 10%-15% of patients with pancreatic acinar cell carcinoma, usually in the setting of liver metastases. LHS consists of peripheral fat necrosis, polyarthralgias, and eosinophilia. Pancreatic acinar enzymes (lipase, trypsin and chymotrypsin) which are normally secreted to the duodenum for digestion, are instead released into the blood by the tumor cells - a phenomenon that has been aptly described as endocrine-ization of an exocrine function. High serum lipase levels cause uncontrolled lipolysis, especially in subcutaneous tissues in the pressure points of legs as well as in the intra-articular and peri-articular adipose tissues. The resultant inflammation as well as bacterial super-infection causes painful symmetric enlargement of knee and ankle joints.
About this image:: Acinar cell carcinoma showing an encapsulated area of the tumor which has predominantly acinar architectural pattern. Normal pancreas is on the upper left. Image courtesy of: @Kusaurap; used with permission.