Jejunal carcinoma in a patient with Peutz-Jeghers syndrome

Peutz–Jegher syndrome (PJS) is a rare autosomal dominant disorder characterized by typical pigmented perioral macules, pigmented spots in the buccal mucosa and digits along with hamartomatous polyps in the gastrointestinal tract.1 The characteristic clinical course includes recurrent episodes of polyp-induced bowel obstruction, abdominal pain and gastrointestinal bleeding. In addition to polyposis, the risk of gastrointestinal and extra-gastrointestinal malignancies is significantly increased in patients with PJS. The relative risk of dying from a gastrointestinal cancer is 13 times greater in patients with PJS than in patients without the condition. The risk of other cancers, especially of the reproductive organs, breasts, pancreas and lungs is 9 times greater among these patients than the general population.2 The Johns Hopkins University reported a 48% incidence of cancer, with 73% of these arising in the gastrointestinal tract.1 However, the occurrence of jejunal carcinoma is very rare. We present the unusual case of a patient with PJS and carcinoma of the jejunum.