Peutz-Jeghers syndrome(PJS) is a rare autosomal dominant inherited disorder, manifested as multiple hamartomatous polyps of gastrointestinal tract, mucocutaneous pigmentations and increased risk of cancers. In this manuscript, we reported five cases of small intestinal carcinoma associated with the PJS. All the five patients have a history of PJS and postoperative pathological examination confirmed the diagnosis of small intestinal carcinoma. Histopathological features and recommended surveillance were additionally discussed.
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