# Solid pseudopapillary neoplasm of the pancreas

Source: https://onco.cc/cancers/solid-pseudopapillary-neoplasm/  
OnCo record `solid-pseudopapillary-neoplasm` (Cancer). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

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).

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.

## Fields

- Kind: Cancer
- Last checked: 2026-09-24
- Also known as: 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
- Tags: gi; pancreatic; subtype-page
- 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)

## Sections of this record

The page is a hub with ten sections in reading order; large sections have their own page. The same plan as JSON: https://onco.cc/api/v1/cancers/solid-pseudopapillary-neoplasm/sections.json

- Overview (on the hub): The TL;DR, the family this cancer belongs to, the organ, who gets it and what the state of the art is. https://onco.cc/cancers/solid-pseudopapillary-neoplasm/#overview [1 state-of-the-art points]
- What it is (on the hub): Anatomy, the subtypes and how they differ, how it is staged, and where advanced disease spreads. https://onco.cc/cancers/solid-pseudopapillary-neoplasm/#what-it-is [3 subtypes]
- Finding it (on the hub): How it shows itself, how it is confirmed, what screening exists, and the biomarkers clinicians test for. https://onco.cc/cancers/solid-pseudopapillary-neoplasm/#finding-it [3 biomarkers]
- Treating it (on the hub): The standard of care by setting, the medicines, surgery and radiotherapy named in it, and the regimens behind them. https://onco.cc/cancers/solid-pseudopapillary-neoplasm/#treating-it [2 settings, 1 decision with options]
- Evidence (on the hub): Trials recruiting now, the landmark trials, the key papers and what they mean, the latest literature, and the milestones year by year. https://onco.cc/cancers/solid-pseudopapillary-neoplasm/#evidence [3 milestones]
- The science (on the hub): The molecular landscape: the targets and how often each appears, the pathways, the mechanics stages and the preclinical models. https://onco.cc/cancers/solid-pseudopapillary-neoplasm/#science [1 target, 2 pathways]
- Where you are (own page): Cases by country, the UK and NHS pathway and other country lenses, and the expert centres with trials on record. https://onco.cc/cancers/solid-pseudopapillary-neoplasm/where-you-are/
- Living with it (on the hub): The decisions you may face, the aids that walk through them, the warnings on record, the first sixty days and the questions to ask. https://onco.cc/cancers/solid-pseudopapillary-neoplasm/#living-with-it [10 questions]
- What is coming (own page): Everything in development, the open problems and what is being done about them, the roadmaps, and what changed on this record. https://onco.cc/cancers/solid-pseudopapillary-neoplasm/coming/ [3 open problems]
- Data (own page): Every connected record, the notes, the JSON, Markdown and RDF twins, and where the record came from and when it was checked. https://onco.cc/cancers/solid-pseudopapillary-neoplasm/data/ [11 connected records]

## Standard of care

- 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. ([Distal pancreatectomy (removal of the body and tail of the pancreas, usually with the spleen)](https://onco.cc/terms/distal-pancreatectomy/), [Whipple procedure (pancreaticoduodenectomy)](https://onco.cc/terms/whipple/), [Robotic & minimally invasive surgery](https://onco.cc/technologies/robotic-surgery/), [MRI](https://onco.cc/technologies/mri/))
- Recurrent or metastatic: Resection of recurrences and liver metastases where feasible; no established systemic therapy; case reports only. ([Oligometastatic disease](https://onco.cc/terms/oligometastatic/), [Distal pancreatectomy (removal of the body and tail of the pancreas, usually with the spleen)](https://onco.cc/terms/distal-pancreatectomy/))

## State of the art

- Recognised as a beta-catenin-driven, low-grade cancer distinct from ductal adenocarcinoma, with excellent long-term outcomes after surgery.

## Open problems

- 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.

## Sources

- Wikipedia: https://en.wikipedia.org/wiki/Solid_pseudopapillary_tumour
- Law, Pancreas 2014: systematic review of 2,744 solid-pseudopapillary neoplasms: https://doi.org/10.1097/mpa.0000000000000061
- Abraham, Am J Pathol 2002: solid-pseudopapillary tumours are genetically distinct and almost always carry beta-catenin mutations: https://doi.org/10.1016/s0002-9440(10)62563-1
- Nagtegaal, Histopathology 2020: the 2019 WHO classification of tumours of the digestive system: https://doi.org/10.1111/his.13975
- CRUK: types of pancreatic cancer: https://www.cancerresearchuk.org/about-cancer/pancreatic-cancer/stages-types-grades/types

## Connected records

- cancers: [Intraductal papillary mucinous neoplasm and other pancreatic cystic precursors](https://onco.cc/cancers/ipmn-cystic-precursors/), [Mucinous cystic neoplasm of the pancreas with associated invasive carcinoma (MCN-associated carcinoma)](https://onco.cc/cancers/mcn-associated-carcinoma/), [Pancreatic acinar cell carcinoma](https://onco.cc/cancers/pancreatic-acinar-cell-carcinoma/), [Pancreatic ductal adenocarcinoma](https://onco.cc/cancers/pancreatic/), [Pancreatic neuroendocrine tumours](https://onco.cc/cancers/pancreatic-net/), [Pancreatoblastoma](https://onco.cc/cancers/pancreatoblastoma/)
- technologies: [CT (computed tomography)](https://onco.cc/technologies/ct/), [Histopathology & immunohistochemistry](https://onco.cc/technologies/histopathology-ihc/), [MRI](https://onco.cc/technologies/mri/), [Robotic & minimally invasive surgery](https://onco.cc/technologies/robotic-surgery/)
- targets: [KRAS](https://onco.cc/targets/kras/)
- pathways: [Pancreatic cancer (KEGG map)](https://onco.cc/pathways/pancreatic-cancer-signalling/), [Wnt / β-catenin](https://onco.cc/pathways/wnt/)
- terms: [Distal pancreatectomy (removal of the body and tail of the pancreas, usually with the spleen)](https://onco.cc/terms/distal-pancreatectomy/), [Grade](https://onco.cc/terms/tumour-grade/), [Oligometastatic disease](https://onco.cc/terms/oligometastatic/), [Whipple procedure (pancreaticoduodenectomy)](https://onco.cc/terms/whipple/)

---
JSON: https://onco.cc/api/v1/entities/solid-pseudopapillary-neoplasm.json