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Peer-reviewedCC BY-NCThe oncologist

Treatment of metastatic solid pseudopapillary neoplasms.

  1. 1Department of Gastrointestinal Oncology, Memorial Sloan Kettering Cancer Center, New York, NY 10065, United States.
  2. 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.

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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  -