Peutz-Jeghers 综合征又称胃肠道多发性息肉综合症,是一种家族性遗传性疾病。本病国内外报道不多,伴恶变者更属少见。我们遇到两例典型的 P-J 综合征并伴有恶性变者,现报道如下。例1:男,24岁,患者自1982年6月起反复出现下腹部疼痛,有时伴恶心、呕吐或自觉下腹部有一肿块,能自行缓解。本次发病后未排气排便,以肠套叠收治。患者自3岁起在口唇等处出现色素斑,并随年龄增大 Peutz-Jeghers syndrome, also known as gastrointestinal multiple polyposis, is a familial inherited disease. There are not many reports of the disease at home and abroad, and those with malignant transformation are even rarer. We encountered two cases of typical P-J syndrome with malignant transformation, which are reported below. Example 1: Male, 24 years old. The patient experienced repeated lower abdominal pain since June 1982. Sometimes he was accompanied with nausea, vomiting, or perceived conscious mass in the lower abdomen. He was able to relieve himself. There was no defecation after the onset of the disease and intussusception was treated. The patient has pigmented spots on the lips, etc. since age 3, and increases with age