Treatment of metastatic solid pseudopapillary neoplasms.
- 1Department of Gastrointestinal Oncology, Memorial Sloan Kettering Cancer Center, New York, NY 10065, United States.
- 2Division of Medical Oncology, Duke Cancer Institute, Durham, NC 27710, United States.
Abstract
Background
Surgical resection is the primary treatment for solid pseudopapillary neoplasms (SPN). Systemic treatment options are limited; no prospective trials or consensus guidelines exist given the rarity of SPN.Patients and methods
We retrospectively reviewed patients treated for SPN at Memorial Sloan Kettering Cancer Center from 2009-2024, summarizing demographics, treatments, outcomes, and genetic testing results when available.Results
Of 45 patients, 42 (93%) underwent surgical resection and 8 (18%) received systemic or locoregional treatment (average 3.25 therapies per patient). Genetic testing was available for 10 patients (22%), 9 of whom had Wnt/β-catenin pathway alterations. One patient whose tumor harbored a CTNNB1 mutation was treated with an investigational therapy inhibiting β-catenin, achieving disease control for 7 months.Conclusion
This is the largest single-institution series of treatment of advanced SPN, demonstrating that select therapies can provide disease control. Molecular characterization of SPN frequently identifies Wnt/β-catenin pathway alterations, demonstrating a potential clinical benefit for drugs targeting this pathway.
Related
- Differentiating pancreatic acinar cell carcinoma from solid pseudopapillary neoplasm using contrast-enhanced computed tomography
- Solid Pseudopapillary Epithelial Neoplasm of the Pancreas: Two Cases and a Review of the Literature
- Solid Pseudopapillary Neoplasm of the Pancreatic Tail With Splenic Hilar Involvement: A Case Report.
- Pancreatic head solid-pseudopapillary neoplasm in a man treated by robot-assisted laparoscopic partial pancreatectomy: a case report.
- Surgical and functional outcomes of pediatric pancreatic solid pseudopapillary neoplasm: A retrospective study
BibTeX
@article{porfido2026treatment,
title = {{Treatment of metastatic solid pseudopapillary neoplasms.}},
author = {Courtney Porfido and Diane Reidy-Lagunes and Nitya Raj},
journal = {The oncologist},
year = {2026},
month = oct,
doi = {10.1093/oncolo/oyag393},
url = {https://europepmc.org/article/MED/42841746},
note = {CC BY-NC},
}
RIS
TY - JOUR
TI - Treatment of metastatic solid pseudopapillary neoplasms.
AU - Courtney Porfido
AU - Diane Reidy-Lagunes
AU - Nitya Raj
PY - 2026
DA - 2026/10/01
JO - The oncologist
DO - 10.1093/oncolo/oyag393
UR - https://europepmc.org/article/MED/42841746
AB - Background Surgical resection is the primary treatment for solid pseudopapillary neoplasms (SPN). Systemic treatment options are limited; no prospective trials or consensus guidelines exist given the rarity of SPN.Patients and methods We retrospectively reviewed patients treated for SPN at Memorial Sloan Kettering Cancer Center from 2009-2024, summarizing demographics, treatments, outcomes, and genetic testing results when available.Results Of 45 patients, 42 (93%) underwent surgical resection and 8 (18%) received systemic or locoregional treatment (average 3.25 therapies per patient). Genetic testing was available for 10 patients (22%), 9 of whom had Wnt/β-catenin pathway alterations. One patient whose tumor harbored a CTNNB1 mutation was treated with an investigational therapy inhibiting β-catenin, achieving disease control for 7 months.Conclusion This is the largest single-institution series of treatment of advanced SPN, demonstrating that select therapies can provide disease control. Molecular characterization of SPN frequently identifies Wnt/β-catenin pathway alterations, demonstrating a potential clinical benefit for drugs targeting this pathway.
SN - 1083-7159
SN - 1549-490X
ER -