Allelic losses of chromosome 11p are present in 50% of cases. Tumors are generally large average diameter of 810 cm, well circumscribed, and at. Utility of immunohistochemistry in the pancreatobiliary tract. Pancreatic acinar cell carcinoma acc frequently metastasizes to the liver and may mimic hepatocellular carcinoma hcc architecturally and cytologically image 1a. They are typically solid, cellular, stromapoor tumours composed of sheets of relatively uniform cells.
Liver metastases in pancreatic acinar cell carcinoma. Molecular analysis revealed a germline brca2 and chek2 mutation in a patient with a rare. Oct 28, 2019 acinar cell carcinoma of pancreas is a rare, malignant cancer of the acinar cells of the pancreas. The molecular changes of acinar cell carcinoma are poorly understood. Acinar cell carcinoma of the pancreas is a rare malignancy representing less than 1% of all pancreatic malignancies. Acinar cell carcinoma of the pancreas stanford university. Acinar cell carcinoma is also known as acinic cell carcinoma and acinous cell. Two pancreatic acinar cell carcinomas with distinctive morphologic features are described. May 02, 2016 pancreatic acinar cell carcinoma acc is a rare malignant neoplasm that accounts for 12% of all pancreatic neoplasms.
The utility of a novel antibody in the pathological. The differential diagnosis for pet includes cp and ductal adenocarcinoma as well as other rare tumors including solid pseudopapillary tumor spt, acinar cell carcinoma acc, and pancreatoblastoma pb. Both tumors showed a variable combination of different histomorphologic patterns formed by tumor cells in different degrees of acinar differentiation. They show different clinical symptoms at presentation, different morphological features, different outcomes, and different molecular alterations. The signs and symptoms of acinar cell carcinoma of pancreas depend upon the size and location of the tumor.
It is also known as pancreatic acinar cell carcinoma and acinar cell carcinoma. It typically occurs in elderly men it may not cause any signs and symptoms initially. Cytology of pancreatic acinar cell carcinoma article in diagnostic cytopathology 345. Insights into the molecular basis of acc can pave the way for the application of more effective, personalized therapies and detection of patients with hereditary predisposition. We report on a case series of 21 patients with acinar cell carcinoma of the pancreas treated at a highvolume quaternary center. Acinar cell carcinoma acc of the pancreas is a very rare tumor entity, since only 1% of the exocrine pancreatic malignancies derive from acinar cells. Pancreatic acinar cell carcinoma acc is a rare malignant neoplasm that accounts for 12% of all pancreatic neoplasms. We sought to describe the ct and mri features of pure acinar cell carcinoma of the pancreas in adults. Acinar cell carcinomas may arise in any portion of the pancreas. Acinar cell carcinoma acc of the pancreas is extremely uncommon and its cytologic features have rarely been described.
Acinar cell carcinomas accs of the pancreas are rare pancreatic neoplasms accounting for about 12% of pancreatic tumors in adults and about 15% in pediatric subjects. Pancreatic panniculitis associated with acinar cell. Pancreatic acinar cell carcinoma pacc is a rare tumor. Cytology of pancreatic acinar cell carcinoma experts. Acinar cell carcinoma of the pancreas libre pathology. Longterm survival in a patient with acinar cell carcinoma. Longterm survival in a patient with acinar cell carcinoma of. Liver metastases in pancreatic acinar cell carcinoma treated. Nuclei round, usually basal, at most moderately pleomorphic.
This sheetlike arrangement is usually punctuated by variable numbers of acinar structures. Surgical resection is the treatment of choice when feasible, but there are no clear recommendations for patients with advanced disease. Rest binds near endocrine enhancers and blocks the activation of endocrine genes by pdx1 through modulation of histone acetylation and pdx1 binding. The exact incidence of this tumor type is unknown, but probably accounts for less than 5% of all neoplasias of the pancreas. Here we report two cases of acc and describe their clinical features, the therapies used to treat them, and their prognosis. Pathology diagnosis of pancreatic neuroendocrine tumors. Pathology diagnosis of pancreatic neuroendocrine tumors atsuko kasajima samaneh yazdani hironobu sasano. The majority arise in the parotid gland, most commonly in women. Malignant epithelial neoplasm composed of cells with morphological resemblance to acinar cells and with evidence of pancreatic exocrine enzyme production iarc. Acinar cell carcinoma of the pancreas and related neoplasms. Malignant ipmn has a better prognosis than ordinary pancreatic adenocarcinomas.
Pathology diagnosis of pancreatic neuroendocrine tumors atsuko kasajima, samaneh yazdani, hironobu sasano. The next most common type, acinar cell carcinoma of the pancreas, arises in the clusters of cells that produce these enzymes, and represents 5% of exocrine pancreas cancers. Clinical practice guidelines exist for pancreatic ductal adenocarcinoma. Case report acinar cell carcinoma of the pancreas springerlink. It occurs mainly in adults, composed of relatively uniform neoplastic cells that are arranged in solid and acinar patterns with production of pancreatic exocrine enzymes. Ct and mri features of pure acinar cell carcinoma of the. These carcinomas mainly occur between the 5 th and 7 th decades of life. Fiveyear survival for pancreatic ductal adenocarcinoma pdac patients remains below 7% due to the lack of effective treatments. Acinar cell carcinoma of the pancreas ductal adenocarcinoma of the pancreas. This study aimed to investigate this rare pancreatic neoplasm through comparing patients with acc to pancreatic ductal cell adenocarcinoma dca. Summary of the immunohistochemical profile for each pancreatic neoplasm. The first patient was a 65yearold woman who had an abdominal ct scan for a urinary infection. The exocrine cells show a strongly basophilic cytoplasm that represents the area occupied by the rough endoplasmic reticulum. During the initial stages, small tumors may not cause any signs and symptoms that are readily recognized.
Acinar cell carcinoma of the pancreas presenting as. Although acinar cells make up most of the pancreatic parenchyma, acinar cell carcinoma represents approximately 1% of exocrine pancreatic tumors 1, 2. Pancreatic acinar cell carcinoma pac is a rare pancreatic tumor for which no information about chromosomal and gene anomalies is available. In chronic pancreatitis, there is acinar loss with fibrosis, and the islets of langerhans are spared until very late in the course of the disease. Rest inhibits direct reprogramming of pancreatic exocrine. Acinic cell carcinoma an overview sciencedirect topics. Acinar cell carcinoma express trypsin, lipase, and.
Aims acinar cell carcinomas accs are rare tumours of the exocrine pancreas accounting for about 12% of all pancreatic neoplasms in adults. Raf1 rearrangements are common in pancreatic acinar cell. Both tumors are characterized by high cellularity, solid nests, acini or trabecular formations, granular cytoplasm, and prominent single nucleoli image 1b. The initial laboratory values revealed a white blood cell count of 8. It represents 5% of all exocrine tumours of the pancreas, making it the second most common type of pancreatic cancer. Nov 24, 2011 acinar cell carcinomas accs are rare tumours of the exocrine pancreas, accounting for up to 2% of all pancreatic neoplasms in adults and 15% of those in children. Acinar cell carcinoma acc is a rare pancreatic neoplasm with dismal prognosis. Pancreatic panniculitis associated with acinar cell carcinoma. Symptoms like weight loss, abdominal pain, nausea and vomiting are nonspecific and mostly related to either locally advanced tumors or metastasis.
Acinar cell carcinoma acc is a rare pancreatic malignancy with poor prognosis accounting for pancreatic tumors in adults and for about 15% in pediatric cases. Pancreatic acinar cell carcinomainduced panniculitis. This heterogeneous clinicopathological spectrum may give rise to difficulties. Acc of the pancreas may show different clinical symptoms, morphological features, and outcomes, which may give rise to. The apical side of the cells is filled with zymogen granules that contain a variety of digestive enzymes. Acinar cell carcinoma is a rare epithelial neoplasm of the pancreas that shows evidence of acinar differentiation, occasionally with an endocrine component 14. May 04, 2020 as previously mentioned, of all pancreatic cancers, 80% are adenocarcinomas of the ductal epithelium. Pancreatic acinar cell carcinoma acc is a rare tumor that frequently metastasizes to the liver and may present a diagnostic challenge due to its morphologic similarity to hepatocellular carcinoma. Acinar to b cell reprogramming holds potential for cell replacement therapy of diabetes. Pancreatic acinar cell carcinoma pubmed central pmc. Abstract histopathology of pancreatic neuroendocrine tu. Liverdirected therapy with y90 selective internal radiation therapy sirt has been used to treat hepatic metastases from pancreatic tumors. What are the signs and symptoms of acinar cell carcinoma of pancreas.
Pancreatic acinar cell carcinoma acc is an uncommon malignancy of exocrine pancreas. Acinar cell carcinoma of the pancreas, also acinar cell carcinoma, is a rare malignant exocrine tumour of the pancreas. However, treatment protocols for acinar cell carcinoma of the pancreas have not been standardized. It should not to be confused with acinic cell carcinoma, a tumour of the salivary gland. Only 2% of tumors of the exocrine pancreas are benign. Acinar cell carcinomas accs are rare tumours of the exocrine pancreas, accounting for up to 2% of all pancreatic neoplasms in adults and 15% of those in children.
Acinar differentiation within the exocrine pancreas is characterized by the expression of pancreatic enzymes including trypsin, chymotrypsin, and lipase stored within large 1251,000 nm cytoplasmic zymogen granules. Here, we report that combined ablation of egfr and craf expression results in complete regression of a significant percentage of pdac tumors driven by krastrp53 mutations in genetically engineered mice. Acinar cell carcinoma of the pancreas is a rare malignant epithelial tumour of the pancreas. Acinar cell carcinoma of the pancreas presenting as diffuse. Which histologic findings are characteristic of pancreatic.
Curative resection of a primarily unresectable acinar cell. The utility of a novel antibody in the pathological diagnosis. We performed genomewide allelotyping of 9 pacs using. Moreover, systemic elimination of these targets induces. Like the functioning endocrine cancers described below, acinar cell carcinomas may cause overproduction of certain molecules, in this case digestive enzymes, which may. Pancreatic carcinoma with multilineage acinar, neuroendocrine, and ductal differentiation ccr7 signaling as an essential regulator of cns infiltration in t cell leukemia rag1 and atm coordinate monoallelic recombination and nuclear positioning of immunoglobulin loci. Pancreatic acinar cell carcinoma is associated with brca2. Acinic cell carcinoma is the second most common salivary gland malignancy, representing approximately 4% to 6% of all salivary gland neoplasms and up to 17% of malignancies. Acinar cell carcinoma of pancreatic which was first descripted by bener in 1908 is a very rare malignant of the exocrine pancreas, accounting for pancreatic neoplasms, even though acinar cells occupy 85% of the volume of the pancreas 1,2. Allelotype of pancreatic acinar cell carcinoma request pdf. Expression of markers of hepatocellular differentiation in.
Jun 28, 2016 acinar cell carcinoma acc is a relatively rare pancreatic neoplasm with poorly defined prognosis. Pancreatic cancer arises when cells in the pancreas, a glandular organ behind the stomach, begin to multiply out of control and form a mass. Carcinoma of the pancreas exhibiting exocrine differentiation and lacking significant cell carcinoma note that solidpseudopapillary neoplasm was erroneously considered in some early reports to be acinar and designated cystic acinar cell carcinoma diagnostic criteria. In a recently reported series in which macroscopic information was available for 58 accs, 22 tumors were in the head, 2 interested the head and the body, 5 the body, 12 the body and tail, 16 the tail, and 1 the entire pancreas. They are typically solid, cellular, stromapoor tumours. Pancreatic acinar cell carcinomainduced panniculitis jaad. Since accs present a wide variety of morphological patterns, immunohistochemical analysis is useful. These cancerous cells have the ability to invade other parts of the body.
Surgical and molecular pathology of pancreatic neoplasms. May 26, 2007 histopathology pancreas adenocarcinoma. There is now evidence that gene fusions activating the mapk pathway are relatively common in pancreatic acinar cell carcinoma with potentially actionable braf or ret fusions being found in 30%. The most common, pancreatic adenocarcinoma, accounts for about 90% of cases, and the term pancreatic cancer is sometimes. As previously mentioned, of all pancreatic cancers, 80% are adenocarcinomas of the ductal epithelium.
Context acinar cell carcinoma of the pancreas is a rare malignancy that may have acinar and endocrine differentiation. Pancreatic cancer is the fourth most common cause of cancerrelated death among men and women in the united states. Tumors originating in the exocrine glands are rare, and constitute 12 % of all acinic cell carcinoma in the pancreatic gland. Acinar cell carcinoma of pancreatic which was first descripted by bener in 1908 is a very rare malignant of the exocrine pancreas, accounting for acinar cells make up most of the pancreatic parenchyma, acinar cell carcinoma represents approximately 1% of exocrine pancreatic tumors 1, 2.
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