{"entity":{"id":"solid-pseudopapillary-neoplasm","kind":"cancer","name":"Solid pseudopapillary neoplasm of the pancreas","aka":["Solid pseudopapillary neoplasm (young women; CTNNB1; low-grade malignant; cured by resection in about 95 percent)","Solid pseudopapillary tumour","SPN","SPT","Frantz tumour","Hamoudi tumour","Papillary cystic neoplasm of the pancreas","Solid and papillary epithelial neoplasm"],"tldr":"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.","summary":"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).\n\nHow 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.\n\nHow 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.\n\nHow 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.","asOf":"2026-09-24","wikipedia":"https://en.wikipedia.org/wiki/Solid_pseudopapillary_tumour","links":[{"label":"Law, Pancreas 2014: systematic review of 2,744 solid-pseudopapillary neoplasms","url":"https://doi.org/10.1097/mpa.0000000000000061"},{"label":"Abraham, Am J Pathol 2002: solid-pseudopapillary tumours are genetically distinct and almost always carry beta-catenin mutations","url":"https://doi.org/10.1016/s0002-9440(10)62563-1"},{"label":"Nagtegaal, Histopathology 2020: the 2019 WHO classification of tumours of the digestive system","url":"https://doi.org/10.1111/his.13975"},{"label":"CRUK: types of pancreatic cancer","url":"https://www.cancerresearchuk.org/about-cancer/pancreatic-cancer/stages-types-grades/types"}],"tags":["gi","pancreatic","subtype-page"],"related":["pancreatic","pancreatoblastoma","pancreatic-acinar-cell-carcinoma","pancreatic-net","ipmn-cystic-precursors","mcn-associated-carcinoma"],"cancers":[],"sections":[],"technologies":["robotic-surgery","histopathology-ihc","mri","ct"],"targets":["kras"],"drugs":[],"companies":[],"institutions":[],"pathways":["wnt","pancreatic-cancer-signalling"],"terms":["distal-pancreatectomy","whipple","tumour-grade","oligometastatic"],"trials":[],"people":[],"bottlenecks":[],"keyPapers":[],"journals":[],"dependsOn":[],"notes":[],"group":"gastrointestinal","burden":"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.","subtypes":["Classical solid pseudopapillary neoplasm (young women; resected; disease-free in 95.6 percent)","Solid pseudopapillary neoplasm with metastases or recurrence (liver most often; resected where possible)","Solid pseudopapillary neoplasm in men or older patients (a minority; distinguished from pancreatic NET and from ductal PDAC on biopsy)"],"biomarkers":["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)"],"standardOfCare":[{"setting":"Localised","approach":"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.","refs":["distal-pancreatectomy","whipple","robotic-surgery","mri"],"guideline":{"version":"NCCN Guidelines: Pancreatic Adenocarcinoma","url":"https://www.nccn.org/guidelines/guidelines-detail?category=1&id=1455"}},{"setting":"Recurrent or metastatic","approach":"Resection of recurrences and liver metastases where feasible; no established systemic therapy; case reports only.","refs":["oligometastatic","distal-pancreatectomy"],"guideline":{"version":"NCCN Guidelines: Pancreatic Adenocarcinoma","url":"https://www.nccn.org/guidelines/guidelines-detail?category=1&id=1455"}}],"stateOfArt":["Recognised as a beta-catenin-driven, low-grade cancer distinct from ductal adenocarcinoma, with excellent long-term outcomes after surgery."],"history":[{"year":1959,"title":"Frantz describes the tumour (papillary tumour of the pancreas)","refs":["tumour-grade"]},{"year":2002,"title":"Beta-catenin mutations found in almost all solid pseudopapillary tumours","refs":["wnt"]},{"year":2014,"title":"Systematic review of 2,744 cases: 95.6 percent disease-free after resection","refs":["distal-pancreatectomy"]}],"pipeline":[],"openProblems":["No systemic therapy is established for the few patients with unresectable recurrence.","The optimal length of follow-up is unknown because recurrences are late and rare.","Small biopsies confuse it with neuroendocrine tumours; beta-catenin staining is the key."],"parent":"pancreatic"},"route":"/cancers/solid-pseudopapillary-neoplasm/","neighbours":{"cancer":[{"id":"ipmn-cystic-precursors","kind":"cancer","name":"Intraductal papillary mucinous neoplasm and other pancreatic cystic precursors","route":"/cancers/ipmn-cystic-precursors/"},{"id":"mcn-associated-carcinoma","kind":"cancer","name":"Mucinous cystic neoplasm of the pancreas with associated invasive carcinoma (MCN-associated carcinoma)","route":"/cancers/mcn-associated-carcinoma/"},{"id":"pancreatic-acinar-cell-carcinoma","kind":"cancer","name":"Pancreatic acinar cell carcinoma","route":"/cancers/pancreatic-acinar-cell-carcinoma/"},{"id":"pancreatic","kind":"cancer","name":"Pancreatic ductal adenocarcinoma","route":"/cancers/pancreatic/"},{"id":"pancreatic-net","kind":"cancer","name":"Pancreatic neuroendocrine tumours","route":"/cancers/pancreatic-net/"},{"id":"pancreatoblastoma","kind":"cancer","name":"Pancreatoblastoma","route":"/cancers/pancreatoblastoma/"}],"technology":[{"id":"ct","kind":"technology","name":"CT (computed tomography)","route":"/technologies/ct/"},{"id":"histopathology-ihc","kind":"technology","name":"Histopathology & immunohistochemistry","route":"/technologies/histopathology-ihc/"},{"id":"mri","kind":"technology","name":"MRI","route":"/technologies/mri/"},{"id":"robotic-surgery","kind":"technology","name":"Robotic & minimally invasive surgery","route":"/technologies/robotic-surgery/"}],"target":[{"id":"kras","kind":"target","name":"KRAS","route":"/targets/kras/"}],"pathway":[{"id":"pancreatic-cancer-signalling","kind":"pathway","name":"Pancreatic cancer (KEGG map)","route":"/pathways/pancreatic-cancer-signalling/"},{"id":"wnt","kind":"pathway","name":"Wnt / β-catenin","route":"/pathways/wnt/"}],"term":[{"id":"distal-pancreatectomy","kind":"term","name":"Distal pancreatectomy (removal of the body and tail of the pancreas, usually with the spleen)","route":"/terms/distal-pancreatectomy/"},{"id":"tumour-grade","kind":"term","name":"Grade","route":"/terms/tumour-grade/"},{"id":"oligometastatic","kind":"term","name":"Oligometastatic disease","route":"/terms/oligometastatic/"},{"id":"whipple","kind":"term","name":"Whipple procedure (pancreaticoduodenectomy)","route":"/terms/whipple/"}]}}