The first 60 days: Pancreatoblastoma
Pancreatoblastoma is the pancreatic cancer of young children, a tumour of immature pancreatic cells that behaves quite unlike adult pancreatic cancer. It grows as a large abdominal mass, often raises the blood marker alpha-fetoprotein, and is treated like the childhood liver cancer hepatoblastoma: chemotherapy to shrink it, then surgery, which cures most children whose tumour has not spread. Below, week by week, is what OnCo's record of Pancreatoblastoma 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.
Imaging of abdomen and chest, serum alpha-fetoprotein and biopsy; genetic review for Beckwith-Wiedemann syndrome and familial adenomatous polyposis.
Complete resection (pancreatoduodenectomy or distal pancreatectomy); adjuvant chemotherapy after incomplete resection.
Neoadjuvant cisplatin and doxorubicin (PLADO, as in hepatoblastoma) followed by delayed resection when the tumour shrinks.
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 and staging.
- RadiologistNamed in the standard of care for: Diagnosis and staging.
- SurgeonNamed in the standard of care for: Resectable at diagnosis, Unresectable at diagnosis, Metastatic or relapsed.
- Medical oncologistNamed in the standard of care for: Resectable at diagnosis, Unresectable at diagnosis, Metastatic or relapsed, Survivorship.
- Clinical oncologist (radiotherapy)Named in the standard of care for: Metastatic or relapsed.
- Palliative and supportive care teamNamed in the standard of care for: Unresectable at diagnosis, Metastatic or relapsed, Survivorship.
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.
Cisplatin and doxorubicin-based chemotherapy with resection of residual disease where possible; radiotherapy for unresectable residual tumour; international registry enrolment.
Lifelong follow-up for pancreatic insufficiency, diabetes and the late effects of platinum and anthracycline chemotherapy.
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 Serum alpha-fetoprotein, Squamoid nests and acinar differentiation on histology; trypsin and BCL10 immunohistochemistry, CTNNB1 mutation or APC loss; nuclear beta-catenin staining, 11p loss of heterozygosity, Germline APC testing where familial adenomatous polyposis is suspected), 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 Localised pancreatoblastoma, resected at diagnosis, Locally advanced pancreatoblastoma requiring neoadjuvant chemotherapy before resection, Metastatic pancreatoblastoma.
- Is germline (inherited) genetic testing recommended for me or my family?Inherited variants can change treatment and matter for relatives.
Diagnosis and staging
- For my situation (diagnosis and staging), which of the standard options do you recommend and why?Guideline options include: Imaging of abdomen and chest, serum alpha-fetoprotein and biopsy; genetic review for Beckwith-Wiedemann syndrome and familial adenomatous polyposis.
Resectable at diagnosis
- For my situation (resectable at diagnosis), which of the standard options do you recommend and why?Guideline options include: Complete resection (pancreatoduodenectomy or distal pancreatectomy); adjuvant chemotherapy after incomplete resection.
Unresectable at diagnosis
- For my situation (unresectable at diagnosis), which of the standard options do you recommend and why?Guideline options include: Neoadjuvant cisplatin and doxorubicin (PLADO, as in hepatoblastoma) followed by delayed resection when the tumour shrinks.
- Am I a candidate for Cisplatin, Doxorubicin, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Metastatic or relapsed
- For my situation (metastatic or relapsed), which of the standard options do you recommend and why?Guideline options include: Cisplatin and doxorubicin-based chemotherapy with resection of residual disease where possible; radiotherapy for unresectable residual tumour; international registry enrolment.
- Am I a candidate for Cisplatin, Doxorubicin, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Survivorship
- For my situation (survivorship), which of the standard options do you recommend and why?Guideline options include: Lifelong follow-up for pancreatic insufficiency, diabetes and the late effects of platinum and anthracycline chemotherapy.
Any stage
- 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 exists or is likely; treatment is extrapolated from hepatoblastoma and pooled registries”. How does that affect my plan?Open problems are where trials and second opinions matter most.
- I read that “Relapsed and metastatic disease has no established second-line therapy”. 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.
- Pancreatoblastoma: the full pagePancreatoblastoma is the pancreatic cancer of young children, a tumour of immature pancreatic cells that behaves quite unlike adult pancreatic cancer. It grows as a large abdominal mass, often raises the blood marker alpha-fetoprotein, and is treated like the childhood liver cancer hepatoblastoma: chemotherapy to shrink it, then surgery, which cures most children whose tumour has not spread.
- 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.
- Metastasis: Cancer that has spread from where it started to distant parts of the body, travelling through the blood or lymph.
- Resection margins (R0 / R1 / R2): Whether the edge of the removed tissue is free of cancer.
- Cancer cachexia: Severe loss of weight and muscle in advanced cancer that eating more cannot reverse on its own.
- 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).
- 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.
Every term links to the glossary.