{"title":"Treatment of metastatic solid pseudopapillary neoplasms.","authors":"Courtney Porfido, Diane Reidy-Lagunes, Nitya Raj","doi":"10.1093/oncolo/oyag393","DOIUrl":null,"url":null,"abstract":"<p><strong>Background: </strong>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.</p><p><strong>Patients and methods: </strong>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.</p><p><strong>Results: </strong>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.</p><p><strong>Conclusion: </strong>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.</p>","PeriodicalId":54686,"journal":{"name":"Oncologist","volume":" ","pages":""},"PeriodicalIF":4.7000,"publicationDate":"2026-10-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13680103/pdf/","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Oncologist","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1093/oncolo/oyag393","RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"ONCOLOGY","Score":null,"Total":0}
引用次数: 0
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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