{"entity":{"id":"paper-klimstra-acinar-cell-carcinoma-pancreas-28-cases-ajsp-1992","kind":"paper","name":"Klimstra 1992: acinar cell carcinoma of the pancreas, a clinicopathologic study of 28 cases","aka":[],"tldr":"The reference description of acinar cell carcinoma, the rare pancreatic cancer that grows from enzyme-producing cells: how it looks under the microscope, how to prove it with enzyme stains, and how it behaves, which is aggressive but somewhat less so than ordinary pancreatic cancer.","summary":"Clinicopathological study of 28 acinar cell carcinomas from the Armed Forces Institute of Pathology and Memorial Sloan Kettering. The tumours were large, mostly in older men, and about half had metastasised at diagnosis; a minority presented with the lipase hypersecretion syndrome of subcutaneous fat necrosis and polyarthralgia.\n\nHistologically the tumours showed acinar and solid growth with minimal stroma, and immunohistochemistry for trypsin, chymotrypsin and lipase confirmed acinar differentiation. Median survival was about 18 months, better than ductal adenocarcinoma but still poor, with occasional long-term survivors after resection.","asOf":"2026-09-21","links":[{"label":"Am J Surg Pathol 1992","url":"https://doi.org/10.1097/00000478-199209000-00001"},{"label":"PubMed","url":"https://pubmed.ncbi.nlm.nih.gov/1384374/"}],"tags":[],"related":[],"cancers":["pancreatic-acinar-cell-carcinoma","pancreatic"],"sections":[],"technologies":[],"targets":[],"drugs":[],"companies":[],"institutions":[],"pathways":[],"terms":[],"trials":[],"people":[],"bottlenecks":[],"keyPapers":[],"journals":[],"dependsOn":[],"notes":[],"journal":"American Journal of Surgical Pathology","year":1992,"doi":"10.1097/00000478-199209000-00001","pmid":"1384374","authors":"Klimstra DS, Heffess CS, Oertel JE, Rosai J.","paperType":"observational","findings":["Acinar cell carcinoma makes up about 1 to 2 percent of pancreatic exocrine tumours; large tumours, older men, about half metastatic at diagnosis.","Trypsin, chymotrypsin and lipase immunostains confirm acinar differentiation.","Median survival about 18 months, better than ductal adenocarcinoma."],"whatItMeans":"This series defined the diagnostic criteria still used for acinar cell carcinoma and established that it should be classified and treated as a distinct disease from ductal adenocarcinoma.","caveats":["Retrospective consultation series from before modern chemotherapy; survival figures are historical.","Mixed acinar-neuroendocrine tumours were only partly separated."],"participants":28},"route":"/key-papers/paper-klimstra-acinar-cell-carcinoma-pancreas-28-cases-ajsp-1992/","neighbours":{"cancer":[{"id":"pancreatic-acinar-cell-carcinoma","kind":"cancer","name":"Pancreatic acinar cell carcinoma","route":"/cancers/pancreatic-acinar-cell-carcinoma/"},{"id":"pancreatic","kind":"cancer","name":"Pancreatic ductal adenocarcinoma","route":"/cancers/pancreatic/"}]}}