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9 slides generated from the cancer page, with a quiz from the open benchmark and speaker notes that cite the sources. Arrow keys move between slides; Print gives one slide per page.
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.
| Setting | Approach | Guideline |
|---|---|---|
| Diagnosis | Biopsy with acinar markers; CT staging; germline testing and comprehensive tumour sequencing with fusion detection for every patient. | not mapped |
| Resectable | Pancreatoduodenectomy or distal pancreatectomy even for large tumours, with adjuvant chemotherapy by extrapolation from ductal adenocarcinoma, usually a fluoropyrimidine and oxaliplatin regimen. | not mapped |
| Advanced | FOLFOX or FOLFIRINOX preferred over gemcitabine-based regimens on retrospective evidence; resection of the primary and limited metastases considered after response. | not mapped |
| Molecularly selected | MEK inhibition for BRAF fusions on case-series evidence; platinum and PARP inhibitors for homologous recombination defects; pembrolizumab for mismatch repair deficiency. | not mapped |