Acinar cell carcinoma of the pancreas, also acinar cell carcinoma, is a rare

malignant Malignancy () is the tendency of a medical condition to become progressively worse. Malignancy is most familiar as a characterization of cancer. A ''malignant'' tumor contrasts with a non-cancerous ''benign'' tumor in that a malignancy is not s ...

exocrine Exocrine glands are glands that secrete substances on to an epithelial surface by way of a duct. Examples of exocrine glands include sweat, salivary, mammary, ceruminous, lacrimal, sebaceous, prostate and mucous. Exocrine glands are one of two ...

tumour of the

pancreas The pancreas is an organ of the digestive system and endocrine system of vertebrates. In humans, it is located in the abdomen behind the stomach and functions as a gland. The pancreas is a mixed or heterocrine gland, i.e. it has both an end ...

. It represents 5% of all exocrine tumours of the pancreas, making it the second most common type of

pancreatic cancer Pancreatic cancer arises when cell (biology), cells in the pancreas, a glandular organ behind the stomach, begin to multiply out of control and form a Neoplasm, mass. These cancerous cells have the malignant, ability to invade other parts of t ...

.Tobias Jeffrey S., Hochhauser, Daniel, ''Cancer and its Management'', p. 276, 2010 (6th edn), , 9781118713259 It is abbreviated ACC. It typically has a guarded prognosis.

Signs and symptoms

Azinuszellkarzinom des Pankreasschwanz - CT axial 001

The disease is more common in men than women and the average age at diagnosis is about 60.Von Hoff": Daniel D. Von Hoff, Douglas Brian Evans, Ralph H. Hruban, eds. ''Pancreatic Cancer'', 2005, Jones & Bartlett Learning, , 9780763721787 Symptoms are often non-specific and include weight loss. A classic presentation, found in around 15% of cases includes subcutaneous nodules (due to

fat necrosis Fat necrosis is a form of necrosis characterized by the action upon fat by digestive enzymes. In fat necrosis the enzyme lipase releases fatty acids from triglycerides. The fatty acids then complex with calcium to form soaps. These soaps appe ...

) and

arthralgias Arthralgia (from Greek ''arthro-'', joint + ''-algos'', pain) literally means ''joint pain''. Specifically, arthralgia is a symptom of injury, infection, illness (in particular arthritis), or an allergic reaction to medication. According to MeSH, ...

, caused by release of

lipase Lipase ( ) is a family of enzymes that catalyzes the hydrolysis of fats. Some lipases display broad substrate scope including esters of cholesterol, phospholipids, and of lipid-soluble vitamins and sphingomyelinases; however, these are usually tr ...

Pathology

ACC are associated with increased serum

and manifest in the classic presentation known as the ''Schmid triad'' (subcutaneous

, polyarthritis, eosinophilia). ACC are typically large, up to 10 cm, and soft compared to pancreatic adenocarcinoma, lacking its dense stroma. They can arise in any part of the pancreas. Histomorphologically, the tumour resembles the cells of the pancreatic acini and, typically, have moderate granular

cytoplasm In cell biology, the cytoplasm is all of the material within a eukaryotic cell, enclosed by the cell membrane, except for the cell nucleus. The material inside the nucleus and contained within the nuclear membrane is termed the nucleoplasm. The ...

that stain with both PAS and PASD.

Diagnosis

Histopathology of acinar cell carcinoma of the pancreas

Relative incidence of pancreatic neoplasms

Light microscopy Microscopy is the technical field of using microscopes to view objects and areas of objects that cannot be seen with the naked eye (objects that are not within the resolution range of the normal eye). There are three well-known branches of micr ...

of an acinar cell carcinoma

biopsy A biopsy is a medical test commonly performed by a surgeon, interventional radiologist, or an interventional cardiologist. The process involves extraction of sample cells or tissues for examination to determine the presence or extent of a diseas ...

typically shows granular appearance.

Immunohistochemistry Immunohistochemistry (IHC) is the most common application of immunostaining. It involves the process of selectively identifying antigens (proteins) in cells of a tissue section by exploiting the principle of antibodies binding specifically to an ...

is usually positive for

trypsin Trypsin is an enzyme in the first section of the small intestine that starts the digestion of protein molecules by cutting these long chains of amino acids into smaller pieces. It is a serine protease from the PA clan superfamily, found in the dig ...

chymotrypsin Chymotrypsin (, chymotrypsins A and B, alpha-chymar ophth, avazyme, chymar, chymotest, enzeon, quimar, quimotrase, alpha-chymar, alpha-chymotrypsin A, alpha-chymotrypsin) is a digestive enzyme component of pancreatic juice acting in the duodenu ...

and

. On

genetic testing Genetic testing, also known as DNA testing, is used to identify changes in DNA sequence or chromosome structure. Genetic testing can also include measuring the results of genetic changes, such as RNA analysis as an output of gene expression, or ...

, altered genes/proteins are typically found for

p53 p53, also known as Tumor protein P53, cellular tumor antigen p53 (UniProt name), or transformation-related protein 53 (TRP53) is a regulatory protein that is often mutated in human cancers. The p53 proteins (originally thought to be, and often s ...

SMAD4 SMAD4, also called SMAD family member 4, Mothers against decapentaplegic homolog 4, or DPC4 (Deleted in Pancreatic Cancer-4) is a highly conserved protein present in all metazoans. It belongs to the SMAD family of transcription factor proteins, ...

, APC,

ARID1A AT-rich interactive domain-containing protein 1A is a protein that in humans is encoded by the ''ARID1A'' gene. Function ARID1A is a member of the SWI/SNF family, whose members have helicase and ATPase activities and are thought to regulate t ...

and GNAS.

Treatment

ACC can be treated with a

Whipple procedure A pancreaticoduodenectomy, also known as a Whipple procedure, is a major surgical operation most often performed to remove cancerous tumours from the head of the pancreas. It is also used for the treatment of pancreatic or duodenal trauma, or chro ...

or (depending on the location within the pancreas) with left partial resection of pancreas.

References

{{Reflist Digestive system neoplasia Pancreas disorders Pancreatic cancer

Signs and symptoms

Pathology

Diagnosis

Treatment

See also

References