Pancreatic acinar cell carcinoma
Prepared with OnCo (onco.cc/prep/pancreatic-acinar-cell-carcinoma/). Orientation, not medical advice; your team knows your case.
My details
What I know, what is unclear, changes to discuss
Saved in this browserMy questions
16 on the sheet- 1.What is my exact diagnosis, stage, and grade, and which tests established them?
- 2.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?
- 3.Which subtype is my cancer, and does that change the recommended treatment?
- 4.Is germline (inherited) genetic testing recommended for me or my family?
- 5.For my situation (diagnosis), which of the standard options do you recommend and why?
- 6.For my situation (resectable), which of the standard options do you recommend and why?
- 7.Am I a candidate for FOLFOX (5-FU, leucovorin, oxaliplatin), FOLFIRINOX / mFOLFIRINOX, and what side effects should I expect?
- 8.For my situation (advanced), which of the standard options do you recommend and why?
- 9.Am I a candidate for FOLFOX (5-FU, leucovorin, oxaliplatin), FOLFIRINOX / mFOLFIRINOX, Oxaliplatin or related drugs, and what side effects should I expect?
- 10.For my situation (molecularly selected), which of the standard options do you recommend and why?
- 11.Am I a candidate for Dabrafenib + trametinib, Olaparib, Pembrolizumab, and what side effects should I expect?
- 12.Are there clinical trials I could join, for example of PDAC organoid pharmacotyping, Comprehensive genomic profiling?
- 13.Would a second opinion at a high-volume centre change anything, and can you help arrange it?
- 14.What supportive care (symptom control, nutrition, exercise, mental health, financial help) is available from the start?
- 15.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?
- 16.I read that “BRAF fusions have no approved drug and MEK inhibitor evidence is case reports”. How does that affect my plan?
The words I may hear
- 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.
Tests and results to bring
Diagnosis: Biopsy with acinar markers; CT staging; germline testing and comprehensive tumour sequencing with fusion detection for every patient.
Biomarker results to ask for: Trypsin, chymotrypsin and BCL10 immunohistochemistry (diagnosis), Serum lipase and alpha-fetoprotein (markers in a subset), BRAF or RAF1 fusions (SND1-BRAF and others), Germline and somatic BRCA2, PALB2, ATM and other homologous recombination genes, Mismatch repair status, APC or CTNNB1 alterations; KRAS usually wild-type.
Scans and tests linked to this cancer: Comprehensive genomic profiling, CT (computed tomography), Endoscopic ultrasound and EBUS systems, Germline (hereditary) testing, PDAC organoid pharmacotyping.
Bring copies of scan reports, pathology and blood results, and a list of every medicine and supplement.
The treatments I may be offered
- Resectable: Pancreatoduodenectomy or distal pancreatectomy even for large tumours, with adjuvant chemotherapy by extrapolation from ductal adenocarcinoma, usually a fluoropyrimidine and oxaliplatin regimen. (Whipple procedure (pancreaticoduodenectomy), FOLFOX (5-FU, leucovorin, oxaliplatin), FOLFIRINOX / mFOLFIRINOX, Neoadjuvant / adjuvant / perioperative)
- Advanced: FOLFOX or FOLFIRINOX preferred over gemcitabine-based regimens on retrospective evidence; resection of the primary and limited metastases considered after response. (FOLFOX (5-FU, leucovorin, oxaliplatin), FOLFIRINOX / mFOLFIRINOX, Oxaliplatin, Fluorouracil (5-FU), Oligometastatic disease)
- Molecularly selected: MEK inhibition for BRAF fusions on case-series evidence; platinum and PARP inhibitors for homologous recombination defects; pembrolizumab for mismatch repair deficiency. (Dabrafenib + trametinib, Olaparib, Pembrolizumab, Gene fusion, Homologous recombination deficiency (HRD))
From the standard of care recorded for this cancer; which apply depends on your stage and biomarkers. Ask which the team recommends and why.