RECTAL CARCINOID-TUMOR ASSOCIATED WITH THE PEUTZ-JEGHERS-SYNDROME

A 16-year-old man who had been diagnosed with the Peutz-Jeghers syndro me at the age of 8 years presented with crampy abdominal pain. Thoroug h examinations revealed a large jejunal polyp causing intussusception, as well as multiple polyps in the small and large intestines. Preoper ative proctoscopy demonstrated the coexistence of a submucosal tumor i n the rectum. Proctoscopic mucosal resection was performed and histolo gical and immunohistochemical examinations led to a diagnosis of carci noid tumor. Additional transanal resection of the rectal wall showed n o residual tumor and the patient has been well for 2 years to date. Al though malignant tumors are increasingly reported in association with the Peutz-Jeghers syndrome, to our knowledge, there have been no previ ous reports of such an association in the English-language and Japanes e literature.