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CT scan shows lymph node and peritoneal involvement. Given the extension, systemic therapy is started with partial response. It is decided to perform cytoreductive surgery (total gastrectomy, right hemicolectomy due to wall implants and resection of distal ileum due to larger than 10mm polyps) and large polyps mucosectomy in the proximal jejunum by anterograde enteroscopy, after which a second-line chemotherapy is started. Discussion PJS is a hamartomatous polyposis that is characterized by a high cumulative risk of cancer in different