Systematic therapy for metastatic solid pseudopapillary neoplasm of the pancreas: A single-center experience.

person Yuting Sun National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital,Chinese Academy of Medical Science,Peking Union Medical College, Beijing, China info_outline Yuting Sun, Huijing Tan, Yihebali Chi

Authors person Yuting Sun National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital,Chinese Academy of Medical Science,Peking Union Medical College, Beijing, China info_outline Yuting Sun, Huijing Tan, Yihebali Chi Organizations National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital,Chinese Academy of Medical Science,Peking Union Medical College, Beijing, China, Cancer Hospital Chinese Academy of Medical Sciences, Beijing, China, National Cancer Center/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China Abstract Disclosures Research Funding No funding sources reported Background: Solid pseudopapillary neoplasm (SPN) of the pancreas is a rare tumor with low-grade malignancy. However, a small proportion of cases still exhibit more aggressive behavior and emerge as distant metastases. Limited data exist on systemic therapy for these entities. Methods: A retrospective analysis was performed on patients diagnosed with SPN of the pancreas who underwent medical treatment in our hospital between January 2020 and December 2023. The clinicopathological characteristics and therapeutic regimens of the patients were collected. The primary outcomes were disease control rate (DCR) and progression free survival (PFS). SPSS software V27.0 was used for data analysis. Results: Five patients were identified and 60.0% of patients were female . The median age was 34 years (range 16-53). All patients first underwent surgical resection. Four individuals had metachronous distant metastasis and one had synchronous distant metastasis. The most common metastatic site was peritoneum (3/5) and liver (3/5), followed by the distant lymph node (2/5). Progesterone receptor positive expression was found in four cases. Four patients received targeted therapy as first-line treatment (3 receiving anlotinib 12mg once daily on days 1-14 every 3 weeks and 1 receiving everolimus 10mg per day). All patients receiving targeted therapy had stable disease, with a DCR of 100%. The median PFS in the targeted therapy group was 8.1 months (95% CI, 6.7-9.5). Only one patient underwent first-line chemotherapy (gemcitabine plus S-1) and experienced disease progression at the initial efficacy evaluation. Data for second-line treatment was available for three patients. One patient got progressive disease (PD) after etoposide administration. Two individuals received everolimus 10mg daily, one of whom showed PD and the other achieved a partial response (PR). All treatment-related adverse events (AEs) were manageable. Conclusions: Metastatic SPN of the pancreas may be not chemosensitive. Targeted therapy, such as anlotinib and everolimus, appears to be a potential therapeutic option.