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Pancreatic acinar cell carcinoma is a rare pancreatic cancer that grows from the enzyme-making cells rather than the ducts. It forms large soft masses, can pour lipase into the blood and cause fat lumps under the skin and joint pain, usually lacks the KRAS mutation, often carries DNA repair faults or BRAF fusions, and responds better to bowel-cancer-style chemotherapy than to gemcitabine.
Acinar cell carcinoma arises from the exocrine cells that make digestive enzymes. Tumours are typically large, well circumscribed and soft, more often in the head, and are diagnosed by biopsy with immunohistochemistry for trypsin, chymotrypsin and BCL10; a minority are mixed acinar-neuroendocrine carcinomas. About one in ten patients has the lipase hypersecretion syndrome of subcutaneous fat necrosis, polyarthralgia and eosinophilia, and serum lipase and alpha-fetoprotein can serve as tumour markers. Metastases at diagnosis are common, most often to the liver.
The genetics differ sharply from ductal adenocarcinoma: KRAS mutations are rare, and instead sequencing finds recurrent BRAF or RAF1 fusions (SND1-BRAF and others) in roughly a fifth to a quarter of cases, alterations in homologous recombination genes including germline BRCA2 and PALB2 in a substantial minority, mismatch repair deficiency in a few, and APC or CTNNB1 changes in the Wnt pathway. Every patient should therefore have germline and tumour sequencing, and germline testing has consequences for relatives.
Surgery is the only curative treatment and is offered even for large tumours and selected metastatic disease, because outcomes after resection are better than for ductal adenocarcinoma. There is no randomised evidence for systemic treatment; retrospective series show that fluoropyrimidine and oxaliplatin regimens (FOLFOX, FOLFIRINOX) produce more responses than gemcitabine-based treatment, which is why guidelines favour them. Tumours with BRAF fusions respond to MEK inhibitors in case reports, homologous recombination-deficient tumours to platinum and PARP inhibitors, and mismatch repair deficient tumours to pembrolizumab. Mixed acinar-neuroendocrine tumours are treated as acinar cell carcinoma.
About 1 to 2 percent of pancreatic cancers in adults, and the commonest pancreatic cancer in children after pancreatoblastoma; it affects men more often than women and presents at a median age around 60.
Most pancreatic cancers arise in the head next to the bile duct, which is why jaundice is the presenting sign; bile duct cancers are named by where along the tree they sit.
Same organ: Glucagonoma, VIPoma, Somatostatinoma, Pancreatic ductal adenocarcinoma, Biliary tract cancer (cholangiocarcinoma), Intrahepatic cholangiocarcinoma, Extrahepatic cholangiocarcinoma (perihilar and distal), Biliary tract cancer (all types), Neuroendocrine tumours, Pancreatic neuroendocrine tumours, Grade 3 well-differentiated neuroendocrine tumour, Extrapulmonary neuroendocrine carcinoma, Gallbladder cancer, Gallbladder adenocarcinoma, Papillary carcinoma of the gallbladder, Mucinous carcinoma of the gallbladder, Adenosquamous and squamous carcinoma of the gallbladder, Neuroendocrine carcinoma of the gallbladder, Incidental gallbladder cancer (found after cholecystectomy), Carcinoma in situ and dysplasia of the gallbladder, Cystic duct carcinoma, Ampullary cancer (ampulla of Vater), Resectable pancreatic ductal adenocarcinoma, Borderline resectable pancreatic ductal adenocarcinoma, Locally advanced unresectable pancreatic ductal adenocarcinoma, Metastatic pancreatic ductal adenocarcinoma, KRAS G12C-mutant pancreatic ductal adenocarcinoma, KRAS wild-type pancreatic ductal adenocarcinoma, BRCA or PALB2-mutant pancreatic ductal adenocarcinoma, Mismatch repair deficient (MSI-high) pancreatic ductal adenocarcinoma, Intraductal papillary mucinous neoplasm and other pancreatic cystic precursors, Pancreatoblastoma, Adenosquamous carcinoma of the pancreas, Colloid (mucinous non-cystic) carcinoma of the pancreas, Undifferentiated carcinoma of the pancreas with osteoclast-like giant cells, Invasive carcinoma arising in an intraductal papillary mucinous neoplasm (IPMN-associated carcinoma), Mucinous cystic neoplasm of the pancreas with associated invasive carcinoma (MCN-associated carcinoma), Solid pseudopapillary neoplasm of the pancreas
Biopsy with acinar markers; CT staging; germline testing and comprehensive tumour sequencing with fusion detection for every patient.
Pancreatoduodenectomy or distal pancreatectomy even for large tumours, with adjuvant chemotherapy by extrapolation from ductal adenocarcinoma, usually a fluoropyrimidine and oxaliplatin regimen.
FOLFOX or FOLFIRINOX preferred over gemcitabine-based regimens on retrospective evidence; resection of the primary and limited metastases considered after response.
MEK inhibition for BRAF fusions on case-series evidence; platinum and PARP inhibitors for homologous recombination defects; pembrolizumab for mismatch repair deficiency.
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Acinar cell carcinoma should be sequenced: RAF fusions and DNA repair defects offer targeted and platinum or PARP inhibitor options that ductal adenocarcinoma rarely has.
The paper underpins the WHO diagnostic criteria for acinar cell carcinoma and its variants and supports aggressive surgery for localised disease.
Acinar cell carcinoma justifies an aggressive surgical approach, including for larger tumours and selected metastatic disease, because the natural history is far better than ductal adenocarcinoma.
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.
Query for this cancer: (TITLE:"Pancreatic acinar cell carcinoma" OR ABSTRACT:"Pancreatic acinar cell carcinoma" OR TITLE:"Acinar cell carcinoma" OR ABSTRACT:"Acinar cell carcinoma" OR TITLE:"ACC of the pancreas" OR ABSTRACT:"ACC of the pancreas" OR TITLE:"Acinar carcinoma" OR ABSTRACT:"Acinar carcinoma" OR TITLE:"Mixed acinar-neuroendocrine carcinoma" OR ABSTRACT:"Mixed acinar-neuroendocrine carcinoma") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Pancreatic acinar cell carcinoma, not a curated reading list.
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Bleeding that does not stop by itself, bleeding from more than one site, or new bruising in several places or one large area.
A swollen painful calf, or sudden breathlessness with chest pain; venous thromboembolism including pulmonary embolism is a labelled warning.
Fainting, near-fainting, or an irregular or racing heartbeat; several kinase inhibitors prolong the QT interval and the labels require ECG and electrolyte monitoring.
Persistent headache with extreme tiredness, nausea, dizziness on standing or low blood pressure. Vomiting, severe weakness or collapse is adrenal crisis.
Dabrafenib: take on an empty stomach. Trametinib: take on an empty stomach; both cause pyrexia.
Capecitabine: take within 30 minutes after a meal. DPD deficiency (DPYD variants) causes severe toxicity: pre-treatment genotyping is recommended in Europe.
See all on the product pages:Dabrafenib + trametinibFluorouracil (5-FU)FOLFIRINOX / mFOLFIRINOXFOLFOX (5-FU, leucovorin, oxaliplatin)OlaparibOxaliplatinPembrolizumab·Printable cards in the navigator
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