Solid pseudopapillary neoplasm of the pancreas
Prepared with OnCo (onco.cc/prep/solid-pseudopapillary-neoplasm/). 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
10 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 Nuclear beta-catenin on immunohistochemistry and CTNNB1 exon 3 mutation, Loss of membranous E-cadherin; CD10, progesterone receptor and cyclin D1 positive; chromogranin negative, No KRAS mutation and intact SMAD4), 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 (localised), which of the standard options do you recommend and why?
- 6.For my situation (recurrent or metastatic), which of the standard options do you recommend and why?
- 7.Would a second opinion at a high-volume centre change anything, and can you help arrange it?
- 8.What supportive care (symptom control, nutrition, exercise, mental health, financial help) is available from the start?
- 9.I read that “No systemic therapy is established for the few patients with unresectable recurrence”. How does that affect my plan?
- 10.I read that “The optimal length of follow-up is unknown because recurrences are late and rare”. How does that affect my plan?
The words I may hear
- Oligometastatic disease: Cancer that has spread to only a few places, which may still be curable by treating each spot.
- Distal pancreatectomy (removal of the body and tail of the pancreas, usually with the spleen): Distal pancreatectomy removes the body and tail of the pancreas, the part to the left of the main vessels, usually with the spleen when the cause is 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.
- 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).
Tests and results to bring
Biomarker results to ask for: Nuclear beta-catenin on immunohistochemistry and CTNNB1 exon 3 mutation (about 90 percent), Loss of membranous E-cadherin; CD10, progesterone receptor and cyclin D1 positive; chromogranin negative (distinguishes it from neuroendocrine tumour), No KRAS mutation and intact SMAD4 (unlike ductal adenocarcinoma).
Scans and tests linked to this cancer: CT (computed tomography), Histopathology & immunohistochemistry, MRI.
Bring copies of scan reports, pathology and blood results, and a list of every medicine and supplement.
The treatments I may be offered
- Localised: Complete resection: distal pancreatectomy for body and tail tumours, pancreatoduodenectomy for the head, parenchyma-sparing surgery considered in young patients; no adjuvant therapy; long-term follow-up imaging for late recurrence. (Distal pancreatectomy (removal of the body and tail of the pancreas, usually with the spleen), Whipple procedure (pancreaticoduodenectomy), Robotic & minimally invasive surgery, MRI)
- Recurrent or metastatic: Resection of recurrences and liver metastases where feasible; no established systemic therapy; case reports only. (Oligometastatic disease, Distal pancreatectomy (removal of the body and tail of the pancreas, usually with the spleen))
From the standard of care recorded for this cancer; which apply depends on your stage and biomarkers. Ask which the team recommends and why.