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7 slides generated from the cancer page, with a quiz from the open benchmark and speaker notes that cite the sources. Arrow keys move between slides; Print gives one slide per page.
Solid pseudopapillary neoplasm is a rare, slow-growing pancreatic tumour of young women that is classed as a low-grade cancer. It is driven by a fault in the beta-catenin gene rather than the KRAS mutation of ordinary pancreatic cancer, is often found by chance as a large mass, and surgery cures more than nineteen in twenty patients, although it can recur years later.
What it is. A low-grade malignant epithelial neoplasm of uncertain cell origin, listed by the WHO among the pancreatic neoplasms distinct from ductal adenocarcinoma (Nagtegaal 2020). Almost all carry an activating mutation of CTNNB1 (beta-catenin): nuclear beta-catenin accumulation in 19 of 20 tumours and mutations in 18 of 20, with cyclin D1 overexpression in 74 percent, while none had a KRAS mutation, SMAD4 (DPC4) expression was intact in all and p53 overexpression was seen in only 3 of 19, showing a genetic pathway divergent from ductal cancer and shared with pancreatoblastoma and acinar cell carcinoma (Abraham 2002).
How it differs from its parent. A systematic review of 2,744 patients found 87.8 percent were female with a mean age of 28.5 years; abdominal pain was the commonest symptom (63.6 percent) and 38.1 percent were found incidentally; mean tumour size was 8.6 cm; 2,285 underwent pancreatic resection; among 1,952 with follow-up (mean 36 months), 95.6 percent remained disease-free and 4.4 percent recurred, at a median of 50.5 months (Law 2014). Reports rose seven-fold after 2000 as cross-sectional imaging spread.
| Setting | Approach | Guideline |
|---|---|---|
| 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. | not mapped |
| Recurrent or metastatic | Resection of recurrences and liver metastases where feasible; no established systemic therapy; case reports only. | not mapped |