A 45-year-old woman with a 3-year history of chronic anemia was followed up at the Gastroenterology outpatient department of another hospital, with a 6-month history of abdominal pain. The pain was intermittent, worse in the mornings, and localized to the epigastric region. The patient noticed that her abdomen became distended after meals and distension was relieved after a bowel movement. The patient noticed a 12-kg weight loss over the last 6 months. There was a history of nausea but no vomiting. The patient denied any history of loss of appetite, diarrhea or constipation, and upper or lower gastrointestinal bleeding. There was no significant past medical history other than anemia and multiple blood transfusions. The patient denied any history of tobacco smoking, alcohol consumption, drug abuse, Helicobacter pylori (H. pylori) infection, chronic renal failure, peptic ulcer disease, and chronic pancreatitis. Her family history was unremarkable. The patient did not have any prior surgeries or history of malignancy. Workup for her chronic anemia included CT scan of the abdomen and pelvis with intravenous and oral contrast, which revealed duodenal intussusception. The patient was booked for upper gastrointestinal endoscopy in the referral hospital but missed her appointment and asked for a referral to another hospital.\nUpon arrival at our hospital, she appeared underweight (height, 162 cm; weight, 43 kg). Her vitals were stable, and she was afebrile (blood pressure, 118/67 mm/Hg; heart rate, 96 bpm; respiratory rate, 23 bpm; oxygen saturation, 98% in room air; temperature: 36.9 °C). On examination, her abdomen was soft and laxative, with no tenderness, or organomegaly. A complete blood count on the day of admission revealed the following: low hemoglobin level, 7.2 g/dL (12.0–17.0 g/dL); mean corpuscular volume (MCV), 78.0 fL (80.0–100.0 fL); low hematocrit, 24.8% (40.0%–51.0%); leukocyte count, 8.2 × 109/L (4.5-11.0 × 109/L); and platelet count, 323 × 109/L (150-450 × 109/L). Other laboratory profiles were unremarkable. Contrast CT of the abdomen and pelvis revealed significant dilation of the stomach with no detectable gastric lesion. The third part of the duodenum was distended by a sausage-shaped intraluminal structure with alternating fat planes and dilated vessels reaching up to the first part of the duodenum, suggesting retrograde jejuno-duodenal intussusception. There was no detectable soft tissue lesion, and the rest of the bowel was unremarkable (A–C). Endoscopic examination of the upper digestive tract revealed a large, long, mobile submucosal longitudinal and tortuous bulge extending from the first to the second part of the duodenum that moved freely with bowel contraction. The papilla of Vater was normal with no neoplastic process.\nThe patient underwent laparoscopic exploration, which revealed antiperistaltic intussusception in the proximal jejunum (). A jejunostomy was performed, and the mass was pulled through in the proximal jejunum (A).\nA cholecystectomy was performed, and a pediatric feeding tube was passed through the cystic duct to locate the ampulla of Vater. The mass was not resectable without affecting the ampulla, so the decision was made to perform a pancreas-sparing duodenal resection. The excised duodenum with mass was sent for histopathological examination (B).\nPathological findings showed a large polypoid mass with the mucosa pedunculated from the bowel lumen, measuring 12 × 7.5 × 2.0 cm3. Microscopic examination revealed a bowel mucosa with underlying submucosa showing lobules of benign normal-appearing Brunner's glands separated by fibrous stroma. The nodular proliferation of Brunner glands was accompanied by cystically dilated ducts and adipose tissue with no microscopic features of malignancy (A–C). The patient was discharged postoperatively in good condition, given an appointment after 1 month.
Case 2 is a 76-year-old Japanese man who had been treated for prostate, rectal and lung cancer, with no mucocutaneous pigmentation or family history of PJS. An upper gastrointestinal endoscopy revealed a duodenal polyp measuring 15 mm in diameter in the second part of his duodenum (Figure ). Endoscopic mucosal resection was performed, and histological examination showed findings suggestive of a hamartomatous polyp (Figure ). A colonoscopy and small-intestinal follow-through showed no other polyps. After the endoscopic treatment, concomitant liver and thyroid cancers were found.