Peutz-Jegher′s syndrome with gut maltoma: A rare presentation

Peutz-Jegher′s syndrome (PJS) is an autosomal dominant disorder characterized by numerous hamartomatous polyps in the gastrointestinal tract (GIT) and pigmented muco-cutaneous lesions. We present here a case of a maltoma associated with multiple hamartomatous polyps detected in a post-operative ileocolic specimen of a 28-year-old man. Prior to this, he had undergone surgery for intussusception when similar polyps were noted in the small bowel. Upper GIT endoscopy also confirmed the diffuse presence of such polyps. A clinico-pathological diagnosis of PJS was made, which by itself is rarely encountered. Furthermore, the detection of mucosa associated lymphoid tissue lymphoma/maltoma in a background of PJS is remarkably unique in this case, for which it has been reported.