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Updated: Aug 11, 2026
Comparative Lesions Analysis Through a Targeted Sequencing Approach
Published on: November 5, 2019
Case Report: Targeted Therapy for Metastatic Solid Pseudopapillary Neoplasm of the Pancreas With CTNNB1 and PTEN
Xinbo Wang1, Daojun Zhu1, Wei Bao2
1Research Institute of General Surgery, Jinling Hospital, Nanjing University Medical School, Nanjing, China.
Background:
Solid pseudopapillary neoplasm (SPN) of the pancreas shows an indolent clinical behavior in cases undergoing surgical resection. The efficacy of combination therapy in the metastatic extrapancreatic SPN treatment remains largely unknown and a clinical challenge.
Case Presentation:
We report a case of a metastatic pancreatic SPN in a 45-year-old woman who presented with an aggressive peritoneal dissemination and hepatic metastases and still showed an indolent clinical course with combination therapy with repeated surgery and targeted therapy. Although the follow-up effect remains to be seen, this is the first report of practical experience of the targeted agents sunitinib and everolimus in metastatic SPN tumors based on the mutation status of PTEN (c.379G>A; p.G127R) and CTNNB1 (c.98C>G; p.S33C). To our knowledge, the PTEN variant identified in this case has not been previously reported in SPN.
Conclusion:
Evidence on variant genetics indicates that future molecular studies may not only help to explain the mechanism of SPN occurrence and development but are also more likely to direct to future precision treatments.
Insights
This case study explores combination therapy for metastatic pancreatic solid pseudopapillary neoplasm (SPN), highlighting the potential of targeted agents like sunitinib and everolimus in managing aggressive disease.
Area of Science:
- Oncology
- Gastroenterology
Background:
- Solid pseudopapillary neoplasm (SPN) of the pancreas typically exhibits indolent behavior after surgical resection.
- The effectiveness of combination therapy for metastatic extrapancreatic SPN is not well-established.
Observation:
- A 45-year-old woman with metastatic pancreatic SPN presented with aggressive peritoneal dissemination and hepatic metastases.
- The patient maintained an indolent clinical course despite extensive disease, utilizing combination therapy including repeated surgery and targeted agents.
Findings:
- This report details the first documented use of sunitinib and everolimus in metastatic SPN, guided by specific genetic mutations (PTEN c.379G>A; p.G127R and CTNNB1 c.98C>G; p.S33C).
- A novel PTEN variant (p.G127R) was identified in this patient's SPN, previously unreported in this neoplasm.
Implications:
- Genetic variant analysis in SPN may elucidate disease mechanisms and guide future precision medicine strategies.
- Targeted therapies, in conjunction with surgery, show promise for managing aggressive metastatic pancreatic SPN.
