The first 60 days: Pancreatic acinar cell carcinoma
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. Below, week by week, is what OnCo's record of Pancreatic acinar cell carcinoma says about the first two months: the order is typical, the timing is yours to ask about. Sections appear only where the record has something to say. Orientation, not medical advice.
What happens now
Staging tests establish exactly what and where the cancer is. Everything else follows from the answers.
Biopsy with acinar markers; CT staging; germline testing and comprehensive tumour sequencing with fusion detection for every patient.
Ask which of these were tested and what the results were; see the report reader for what each value means.
These specialties appear in the standard of care for this cancer. In most centres they meet weekly as a tumour board to agree each plan; you can ask when yours was discussed and what was decided.
- PathologistNamed in the standard of care for: Diagnosis.
- RadiologistNamed in the standard of care for: Diagnosis.
- SurgeonNamed in the standard of care for: Resectable, Advanced.
- Medical oncologistNamed in the standard of care for: Resectable, Advanced, Molecularly selected.
A second opinion from a centre that treats many similar cases is normal, not rude; the standard of care tells you what to compare it against.
Each row is a setting from the standard of care, in the order it usually arises. Not all will apply to you; your stage and biomarkers decide which do. The guideline grade, where recorded, says how strong the evidence is.
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.
Generated from this cancer's standard of care, biomarkers and open problems. Take the group that matches where you are. To tick, add your own and print, open the one-page appointment sheet.
Newly diagnosed
- What is my exact diagnosis, stage, and grade, and which tests established them?Everything else follows from an accurate stage and subtype.
- Which biomarkers have been tested on my tumour (for example Trypsin, chymotrypsin and BCL10 immunohistochemistry, Serum lipase and alpha-fetoprotein, BRAF or RAF1 fusions, Germline and somatic BRCA2, PALB2, ATM and other homologous recombination genes, Mismatch repair status), and what were the results?These results decide eligibility for targeted therapy, immunotherapy, and trials.
- Which subtype is my cancer, and does that change the recommended treatment?Recognised subtypes for this cancer include Pure acinar cell carcinoma, Mixed acinar-neuroendocrine carcinoma, Acinar cell carcinoma with a BRAF or RAF1 fusion.
- Is germline (inherited) genetic testing recommended for me or my family?Inherited variants can change treatment and matter for relatives.
Diagnosis
- For my situation (diagnosis), which of the standard options do you recommend and why?Guideline options include: Biopsy with acinar markers; CT staging; germline testing and comprehensive tumour sequencing with fusion detection for every patient.
Resectable
- For my situation (resectable), which of the standard options do you recommend and why?Guideline options include: Pancreatoduodenectomy or distal pancreatectomy even for large tumours, with adjuvant chemotherapy by extrapolation from ductal adenocarcinoma, usually a fluoropyrimidine and oxaliplatin regimen.
- Am I a candidate for FOLFOX (5-FU, leucovorin, oxaliplatin), FOLFIRINOX / mFOLFIRINOX, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Advanced
- For my situation (advanced), which of the standard options do you recommend and why?Guideline options include: FOLFOX or FOLFIRINOX preferred over gemcitabine-based regimens on retrospective evidence; resection of the primary and limited metastases considered after response.
- Am I a candidate for FOLFOX (5-FU, leucovorin, oxaliplatin), FOLFIRINOX / mFOLFIRINOX, Oxaliplatin or related drugs, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Molecularly selected
- For my situation (molecularly selected), which of the standard options do you recommend and why?Guideline options include: MEK inhibition for BRAF fusions on case-series evidence; platinum and PARP inhibitors for homologous recombination defects; pembrolizumab for mismatch repair deficiency.
- Am I a candidate for Dabrafenib + trametinib, Olaparib, Pembrolizumab, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Any stage
- Are there clinical trials I could join, for example of PDAC organoid pharmacotyping, Comprehensive genomic profiling?Trials are how the next standard of care is set; asking early keeps options open.
- Would a second opinion at a high-volume centre change anything, and can you help arrange it?Rare or high-stakes decisions benefit from a centre that treats many similar patients.
- What supportive care (symptom control, nutrition, exercise, mental health, financial help) is available from the start?Supportive care improves quality of life and helps patients complete treatment.
- I read that “No prospective trial has ever been completed in acinar cell carcinoma; all systemic therapy evidence is retrospective”. How does that affect my plan?Open problems are where trials and second opinions matter most.
- I read that “BRAF fusions have no approved drug and MEK inhibitor evidence is case reports”. How does that affect my plan?Open problems are where trials and second opinions matter most.
Much of what helps in the first weeks is free if you know to ask: testing, helplines, rides and lodging, second opinions, trial travel.
- Pancreatic acinar cell carcinoma: the full pagePancreatic 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.
- One-page appointment sheetYour questions, the words you may hear, what to bring, and space for the answers. Print it.
- Treatment sequencingWhich treatment tends to follow which, line by line.
- NavigatorStandard of care for your stage, what you have tried, and trials near you.
- Homologous recombination deficiency (HRD): A tumour that cannot properly repair double-strand DNA breaks, usually because of BRCA or related gene loss.
- Oligometastatic disease: Cancer that has spread to only a few places, which may still be curable by treating each spot.
- Grade: How abnormal the cancer cells look under the microscope, from grade 1 (close to normal, slow) to grade 3 or 4 (wildly abnormal, fast).
- Germline BRCA mutation (gBRCA): An inherited fault in the BRCA1 or BRCA2 gene, present in every cell from birth, that greatly raises the risk of breast, ovarian, prostate and pancreatic cancer and makes those cancers sensitive to PARP inhibitors and platinum.
- Gene fusion: A gene fusion is two genes broken and joined together, creating a hybrid protein that can drive cancer.
- Whipple procedure (pancreaticoduodenectomy): The big operation for cancers of the head of the pancreas: the surgeon removes the pancreatic head, the duodenum, the gallbladder and part of the bile duct, then reconnects everything.
- Neoadjuvant / adjuvant / perioperative: Neoadjuvant therapy is treatment given before surgery, adjuvant therapy is treatment given after it, and perioperative therapy is both.
- Microsatellite instability (MSI-H) / mismatch repair deficiency (dMMR): Microsatellite instability is the mark of a broken DNA spell-checker (loss of MLH1, MSH2, MSH6 or PMS2) that leaves thousands of mutations, so the tumour displays abnormal proteins that T cells can recognise.
Every term links to the glossary.