Pancreatoblastoma: the decisions you may face
5 treatment settings, 3 with more than one named option. Each section lays out the options the standard of care names, what each is for, the trials behind them with their recorded results, the side effects and cautions on record, and questions to ask. Built from the cancer page's standard-of-care rows; nothing here is advice for your case.
Diagnosis and staging
Imaging of abdomen and chest, serum alpha-fetoprotein and biopsy; genetic review for Beckwith-Wiedemann syndrome and familial adenomatous polyposis.
A CT scan is a fast 3D X-ray that shows the size and shape of tumours and whether they have spread.
- Fast, ubiquitous
- Sub-millimetre resolution
- Standard for RECIST response
MRI uses a strong magnet and radio waves, with no ionising radiation, to picture soft tissue in finer contrast than CT, so it is the standard scan for brain tumours, prostate, rectal cancer staging, liver lesions and breast screening in high-risk women. It is slow, expensive and blurred by movement.
- No ionising radiation
- Best soft-tissue and brain imaging
- Functional sequences (diffusion, perfusion)
A test of the DNA you were born with, to find inherited risk genes such as BRCA or Lynch syndrome.
- Actionable for patient and relatives
- Cheap
No trial record is attached to this row yet. The trials tab lists what is recruiting and the landmark trials for this cancer.
- Anatomic only; cannot distinguish scar from live tumour
- Radiation dose
- Poor for brain, marrow, and small peritoneal disease
- Slow and expensive
- Motion artefacts
- Gadolinium concerns in renal impairment
- VUS burden
- Uptake and counselling capacity
- Between CT (computed tomography), MRI and Germline (hereditary) testing, which do you recommend for me, and what about my case would make you choose differently?Why: Guidelines list several reasonable options; the choice turns on details of your tumour, your health and your priorities.
- What is each option trying to achieve: cure, long control, or relief of symptoms, and over what time?Why: The aim shapes how much side effect and disruption is worth accepting.
- What happens if I delay, or decline this step for now? Is the decision reversible?Why: Some decisions can wait for a second opinion or a trial slot; others cannot. Knowing which is part of the choice.
- Does your recommendation follow the current guideline (EXPeRT consensus on pancreatoblastoma (Pediatric Blood and Cancer 2021)), and if it departs from it, why?Why: Departures from guidelines are sometimes right for an individual; they should be explained.
- For my situation (diagnosis and staging), which of the standard options do you recommend and why?Why: Guideline options include: Imaging of abdomen and chest, serum alpha-fetoprotein and biopsy; genetic review for Beckwith-Wiedemann syndrome and familial adenomatous polyposis.
Add these to your appointment list, or take the full question set for this cancer.
Resectable at diagnosis
Complete resection (pancreatoduodenectomy or distal pancreatectomy); adjuvant chemotherapy after incomplete resection.
This setting names no product or technology record yet; the approach above is the standard as written. Ask your team which specific treatments they mean.
No trial record is attached to this row yet. The trials tab lists what is recruiting and the landmark trials for this cancer.
No side-effect rates or interaction flags are recorded for these options yet. The side-effect lookup and interaction checker cover the products that have them.
- Which specific treatments are you proposing for this setting, and what are the alternatives?Why: The standard of care here is described in words rather than named products; ask for the names.
- What is each option trying to achieve: cure, long control, or relief of symptoms, and over what time?Why: The aim shapes how much side effect and disruption is worth accepting.
- What happens if I delay, or decline this step for now? Is the decision reversible?Why: Some decisions can wait for a second opinion or a trial slot; others cannot. Knowing which is part of the choice.
- Does your recommendation follow the current guideline (EXPeRT consensus on pancreatoblastoma (Pediatric Blood and Cancer 2021)), and if it departs from it, why?Why: Departures from guidelines are sometimes right for an individual; they should be explained.
- For my situation (resectable at diagnosis), which of the standard options do you recommend and why?Why: Guideline options include: Complete resection (pancreatoduodenectomy or distal pancreatectomy); adjuvant chemotherapy after incomplete resection.
Add these to your appointment list, or take the full question set for this cancer.
Unresectable at diagnosis
Neoadjuvant cisplatin and doxorubicin (PLADO, as in hepatoblastoma) followed by delayed resection when the tumour shrinks.
Cisplatin is the original platinum chemotherapy, discovered by accident in 1965; it cures testicular cancer and makes radiation work better in cervical and head and neck cancer.
The red chemotherapy drug from a soil bacterium that is still the backbone of treatment for sarcoma, lymphoma and breast cancer, limited by cumulative heart damage.
No trial record is attached to this row yet. The trials tab lists what is recruiting and the landmark trials for this cancer.
- Dose reduce or avoid for CrCl below 60 (carboplatin is the alternative).
- Cumulative dose: risk rises steeply above 400-550 mg/m² (see the anthracycline calculator).
- Reduce by 50% for bilirubin 20-50 µmol/L and 75% for 50-85 µmol/L.
- Between Cisplatin and Doxorubicin, which do you recommend for me, and what about my case would make you choose differently?Why: Guidelines list several reasonable options; the choice turns on details of your tumour, your health and your priorities.
- What is each option trying to achieve: cure, long control, or relief of symptoms, and over what time?Why: The aim shapes how much side effect and disruption is worth accepting.
- What happens if I delay, or decline this step for now? Is the decision reversible?Why: Some decisions can wait for a second opinion or a trial slot; others cannot. Knowing which is part of the choice.
- Does your recommendation follow the current guideline (EXPeRT consensus on pancreatoblastoma (Pediatric Blood and Cancer 2021)), and if it departs from it, why?Why: Departures from guidelines are sometimes right for an individual; they should be explained.
- For my situation (unresectable at diagnosis), which of the standard options do you recommend and why?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?Why: Knowing the expected toxicities helps you plan work, family, and supportive care.
Add these to your appointment list, or take the full question set for this cancer.
Metastatic or relapsed
Cisplatin and doxorubicin-based chemotherapy with resection of residual disease where possible; radiotherapy for unresectable residual tumour; international registry enrolment.
Cisplatin is the original platinum chemotherapy, discovered by accident in 1965; it cures testicular cancer and makes radiation work better in cervical and head and neck cancer.
The red chemotherapy drug from a soil bacterium that is still the backbone of treatment for sarcoma, lymphoma and breast cancer, limited by cumulative heart damage.
No trial record is attached to this row yet. The trials tab lists what is recruiting and the landmark trials for this cancer.
- Dose reduce or avoid for CrCl below 60 (carboplatin is the alternative).
- Cumulative dose: risk rises steeply above 400-550 mg/m² (see the anthracycline calculator).
- Reduce by 50% for bilirubin 20-50 µmol/L and 75% for 50-85 µmol/L.
- Between Cisplatin and Doxorubicin, which do you recommend for me, and what about my case would make you choose differently?Why: Guidelines list several reasonable options; the choice turns on details of your tumour, your health and your priorities.
- What is each option trying to achieve: cure, long control, or relief of symptoms, and over what time?Why: The aim shapes how much side effect and disruption is worth accepting.
- What happens if I delay, or decline this step for now? Is the decision reversible?Why: Some decisions can wait for a second opinion or a trial slot; others cannot. Knowing which is part of the choice.
- Does your recommendation follow the current guideline (EXPeRT consensus on pancreatoblastoma (Pediatric Blood and Cancer 2021)), and if it departs from it, why?Why: Departures from guidelines are sometimes right for an individual; they should be explained.
- For my situation (metastatic or relapsed), which of the standard options do you recommend and why?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?Why: Knowing the expected toxicities helps you plan work, family, and supportive care.
Add these to your appointment list, or take the full question set for this cancer.
Survivorship
Lifelong follow-up for pancreatic insufficiency, diabetes and the late effects of platinum and anthracycline chemotherapy.
Protecting the ability to have children before cancer treatment that damages eggs, sperm or the womb: sperm and egg or embryo freezing, ovarian tissue freezing, ovarian shielding and, for some breast cancers, temporary ovarian suppression.
- Established live-birth outcomes for sperm, oocyte, embryo and ovarian tissue
- Random-start protocols avoid treatment delay
- POSITIVE trial reassures about pregnancy after breast cancer
No trial record is attached to this row yet. The trials tab lists what is recruiting and the landmark trials for this cancer.
- Cost and insurance coverage (mandated in only some US states)
- Prepubertal boys have only experimental options
- Referral gaps, especially in men, adolescents and LMICs
- Is Oncofertility and fertility preservation the only reasonable path for me, or is there a trial, a different sequence or a wait-and-see option?Why: A single standard does not mean a single choice; timing and trials are decisions too.
- What is each option trying to achieve: cure, long control, or relief of symptoms, and over what time?Why: The aim shapes how much side effect and disruption is worth accepting.
- What happens if I delay, or decline this step for now? Is the decision reversible?Why: Some decisions can wait for a second opinion or a trial slot; others cannot. Knowing which is part of the choice.
- Does your recommendation follow the current guideline (EXPeRT consensus on pancreatoblastoma (Pediatric Blood and Cancer 2021)), and if it departs from it, why?Why: Departures from guidelines are sometimes right for an individual; they should be explained.
- For my situation (survivorship), which of the standard options do you recommend and why?Why: Guideline options include: Lifelong follow-up for pancreatic insufficiency, diabetes and the late effects of platinum and anthracycline chemotherapy.
Add these to your appointment list, or take the full question set for this cancer.