Pancreatic acinar cell carcinomas with prominent ductal differentiation: Mixed acinar ductal carcinoma and mixed acinar endocrine ductal carcinoma Journal Article
| Authors: | Stelow, E. B.; Shaco-Levy, R.; Bao, F.; Garcia, J.; Klimstra, D. S. |
| Article Title: | Pancreatic acinar cell carcinomas with prominent ductal differentiation: Mixed acinar ductal carcinoma and mixed acinar endocrine ductal carcinoma |
| Abstract: | Background: Pancreatic acinar cell carcinomas (ACCs) are clinically and pathologically distinct from pancreatic ductal adenocarcinomas (PDAs). Whereas endocrine differentiation has been well shown in ACCs, significant ductal components are rare. This paper reviews the clinicopathologic features of a series of ACCs with prominent ductal differentiation. DESIGN: Cases of pancreatic ACCs with significant ductal differentiation were identified in the surgical pathology databases of 2 academic centers. Patient clinical information, gross and histologic features, and histochemical and immunohistochemical (IHC) Results were recorded. Cases were tested for KRAS2 mutations. Results: Eleven cases were identified (10 men and 1 woman; age range 52 to 79y). Four patients presented with jaundice. At last follow-up, 7 patients died of disease and 2 others had recurrences. Tumors measured between 2 and 5.5cm and were ill-defined, nodular, and multilobulated. Ten were located in the head of the pancreas. All but 2 exhibited extrapancreatic invasion. All cases showed significant evidence of both acinar and ductal differentiation, estimated to be at least 25% of the neoplastic cells, and 3 cases in addition had endocrine differentiation in more than 25% of cells. Five cases were predominately acinar with intracellular and sometimes extracellular mucin ("mucinous acinar cell carcinoma" pattern). Six cases seemed more mixed with areas recapitulating typical PDAs whereas the other portions of the tumors seemed akin to typical acinar cell carcinomas ("combined acinar and ductal" pattern). IHC positive staining Results were as: trypsin (92%), chymotrypsin (92%), monoclonal carcinoembryonic antigen (100%), CK19 (100%), B72.3 (73%), CA19.9 (73%), CD56 (18%), synaptophysin (36%), and chromogranin (36%). One case showed p53 over-expression aznd none showed DPC4/Smad4 loss. Two cases had KRAS2 mutations. CONCLUSION: Despite the early embryologic divergence of acinar and ductal cell lineages, rare pancreatic tumors have both acinar and ductal differentiation, usually predominantly the former. The clinical course is highly aggressive. © 2010 Lippincott Williams & Wilkins. |
| Keywords: | aged; middle aged; survival rate; oncoprotein; genetics; mutation; proto-oncogene proteins; mortality; multimodality cancer therapy; united states; pancreas resection; combined modality therapy; pancreatic neoplasms; pancreas; metabolism; carcinoma, pancreatic ductal; tumor markers, biological; pathology; tumor marker; cell transformation, neoplastic; pancreas carcinoma; enzyme immunoassay; immunoenzyme techniques; dna; cell transformation; nucleotide sequence; pancreas tumor; dna, neoplasm; pancreatectomy; ras protein; dna mutational analysis; ras proteins; new york; acinar cell carcinoma; carcinoma, acinar cell; multiple cancer; neoplasms, multiple primary; kras protein, human; mucinous; pancreas islet cell carcinoma; mucin; mucins; carcinoma, islet cell; ductal adenocarcinoma; mixed; virginia |
| Journal Title: | American Journal of Surgical Pathology |
| Volume: | 34 |
| Issue: | 4 |
| ISSN: | 0147-5185 |
| Publisher: | Lippincott Williams & Wilkins |
| Date Published: | 2010-04-01 |
| Start Page: | 510 |
| End Page: | 518 |
| Language: | English |
| DOI: | 10.1097/PAS.0b013e3181cfcac7 |
| PUBMED: | 20182344 |
| PROVIDER: | scopus |
| DOI/URL: | |
| Notes: | --- - "Cited By (since 1996): 1" - "Export Date: 20 April 2011" - "CODEN: AJSPD" - "Source: Scopus" |
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