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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.
How common it is. Not counted by registries; the literature series above is the best measure. Cancer Research UK lists it among the cystic tumours of the pancreas that may be removed or kept under surveillance.
How it is treated. Resection is the treatment and is curative in the great majority: distal pancreatectomy for body and tail tumours, pancreatoduodenectomy for the head, with parenchyma-sparing operations considered in young patients because lymph node spread is rare. Recurrences and the rare liver metastases are resected where possible; there is no established systemic therapy and no trial. Long follow-up is needed because recurrence can come after four years or more.
Rare but increasingly recognised: 2,744 patients in 484 publications to 2012, 87.8 percent of them reported after 2000; 87.8 percent female, mean age 28.5; no registry count is published.
Most pancreatic cancers arise in the head next to the bile duct, which is why jaundice is the presenting sign; bile duct cancers are named by where along the tree they sit.
Same organ: Glucagonoma, VIPoma, Somatostatinoma, Pancreatic ductal adenocarcinoma, Biliary tract cancer (cholangiocarcinoma), Intrahepatic cholangiocarcinoma, Extrahepatic cholangiocarcinoma (perihilar and distal), Biliary tract cancer (all types), Neuroendocrine tumours, Pancreatic neuroendocrine tumours, Grade 3 well-differentiated neuroendocrine tumour, Extrapulmonary neuroendocrine carcinoma, Gallbladder cancer, Gallbladder adenocarcinoma, Papillary carcinoma of the gallbladder, Mucinous carcinoma of the gallbladder, Adenosquamous and squamous carcinoma of the gallbladder, Neuroendocrine carcinoma of the gallbladder, Incidental gallbladder cancer (found after cholecystectomy), Carcinoma in situ and dysplasia of the gallbladder, Cystic duct carcinoma, Ampullary cancer (ampulla of Vater), Resectable pancreatic ductal adenocarcinoma, Borderline resectable pancreatic ductal adenocarcinoma, Locally advanced unresectable pancreatic ductal adenocarcinoma, Metastatic pancreatic ductal adenocarcinoma, KRAS G12C-mutant pancreatic ductal adenocarcinoma, KRAS wild-type pancreatic ductal adenocarcinoma, BRCA or PALB2-mutant pancreatic ductal adenocarcinoma, Mismatch repair deficient (MSI-high) pancreatic ductal adenocarcinoma, Pancreatic acinar cell carcinoma, Intraductal papillary mucinous neoplasm and other pancreatic cystic precursors, Pancreatoblastoma, Adenosquamous carcinoma of the pancreas, Colloid (mucinous non-cystic) carcinoma of the pancreas, Undifferentiated carcinoma of the pancreas with osteoclast-like giant cells, Invasive carcinoma arising in an intraductal papillary mucinous neoplasm (IPMN-associated carcinoma), Mucinous cystic neoplasm of the pancreas with associated invasive carcinoma (MCN-associated carcinoma)
Nothing recorded yet.
Nothing recorded yet.
Also on OnCo: Symptoms and red flags · Early detection roadmap.
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.
Resection of recurrences and liver metastases where feasible; no established systemic therapy; case reports only.
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Query for this cancer: (TITLE:"Solid pseudopapillary neoplasm of the pancreas" OR ABSTRACT:"Solid pseudopapillary neoplasm of the pancreas" OR TITLE:"Solid pseudopapillary neoplasm young women; CTNNB1; low-grade malignant; cured by resection in about 95 percent" OR ABSTRACT:"Solid pseudopapillary neoplasm young women; CTNNB1; low-grade malignant; cured by resection in about 95 percent" OR TITLE:"Solid pseudopapillary tumour" OR ABSTRACT:"Solid pseudopapillary tumour" OR TITLE:"SPN" OR ABSTRACT:"SPN" OR TITLE:"SPT" OR ABSTRACT:"SPT" OR TITLE:"Frantz tumour" OR ABSTRACT:"Frantz tumour") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Solid pseudopapillary neoplasm of the pancreas, not a curated reading list.
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