A 19-year old male Caucasian patient visited the emergency department of our hospital because of melena. He was previously healthy and used no medication but had noticed black stools one week before his visit. There had been no abdominal discomfort, nausea or vomiting. None of his family members had gastrointestinal diseases, and he used no alcohol, did not smoke or use elicit drugs. His occupation is unknown. On physical examination the abdomen was nontender and without palpable masses. Rectal examination revealed black stools. His weight was 62 kg. and his length 1,87 m. Laboratory tests showed an iron deficiency anemia with a hemoglobin concentration of 4.7 mmol/L (normal > 8,5 mmol/L) and a serum ferritin concentration of 4 μg/L (normal > 30 μg/L). Liver function tests, serum creatinine, albumin, CRP and glucose were all normal. Duodenoscopy showed a normal esophageal, gastric and duodenal mucosa without evidence of recent bleeding. A Meckel scan showed no evidence for a Meckel's diverticulum. A small bowel series however, demonstrated a large polyp in the proximal jejunum and possibly a smaller polyp in the distal jejunum (Figure ).\nAs this polyp was the probable cause of the patient's bleeding, a laparotomy with peroperative small bowel endoscopy was performed. Approximately 50 cm beyond Treitz' ligament, a multilobular, 40 mm large polyp was seen, which was subsequently surgically resected (Figure ). There were no other small bowel polyps; the suspected smaller polyp seen on the small bowel series was therefore most likely an artefact.\nPathologic examination of this polyp demonstrated a hamartomatous polyp with arborization of smooth muscle compatible with a PJP (Figure ). No signs of dysplasia were seen and the surrounding tissue had a normal histological appearance. Immunohistochemistry was negative for p53, focally positive for cyclin D1 and the basal parts of the cryps were positive for Ki67/MIB1.\nThe postoperative course has been unremarkable and no further gastrointestinal bleeding has occurred. Total colonoscopy did not show any additional polyps. A repeated, extensive family history, performed by a clinical genetics consultant, did not reveal clues (including pigmentation, nasal or gastrointestinal polyps or typical secondary tumors) to the occurrence of PJS in family members.
Our case was a 2-year-old boy who was incidentally diagnosed with anemia during the evaluation of an upper respiratory tract infection. There was no history of hematemesis, melena, or abdominal pain. The child was evaluated by the pediatric gastroenterologist with an upper gastrointestinal endoscopy and was found to have a large sessile gastric polyp that was not amenable to endoscopic resection. Physical examination was insignificant except for a few melanotic spots over the lower lip []. Radiological imaging revealed a large hyper-enhancing mass lesion in the lumen of the stomach, measuring about 8 cm in greatest dimension along with multiple small intestinal polyps. Laparotomy revealed a large sessile polyp involving the greater curvature and posterior wall of the stomach measuring about 10 cm × 8 cm along with multiple small sessile polyps involving the corpus of the stomach sparing the antrum and pylorus, requiring partial gastrectomy []. There were two more polyps, one each in the duodenum and distal jejunum in their antimesenteric border and they could be excised by enterotomy. The child had an uneventful postoperative course. The histopathology revealed the features of tubulovillous adenoma in all the resected polyps. At 36 months of follow-up, the child is asymptomatic and disease-free.