A 22-year-old male patient was admitted in the surgery ward with symptoms of bowel obstruction. He had a history of vomiting and abdominal distention since 10 days. The patient had experienced similar complaints on and off for the past year. On examination, the patient's abdomen was distended and a vague swelling was noted in the umbilical region. The baseline investigations were normal with only a rise noted in the patient's erythrocyte sedimentation rate which was 70 mm at 30 minutes and 118 mm at the end of 1hour. His Mantoux tested negative. An X-ray abdomen showed multiple fluid levels and a contrast-enhanced CT scan revealed ileal wall thickening and mesenteric nodes. The surgeon proceeded with a laparotomy and resected a segment of ileum with omental adhesions and sent for histopathological examination.\nThe cut open small intestinal segment measured 34 × 6 cm. A polyp of 1.5 cm diameter was noted 18 cm from the proximal end. Two ulcers were identified, the first measuring 2 × 2.5 cm was 14 cm from the proximal end and the second, measuring 2 × 1.5 cm was 20cm from the proximal end. Both ulcers were shallow, situated along the horizontal plane, with undermined edges and a fibrotic base. A stricture was present 9cm from the distal end with dilatation of the proximal segment. The serosa showed large lymph nodes measuring 4 × 5 cm, whose cut section was whitish and creamy in consistency. The areas adjacent to the ulcers showed diffuse thickening of the wall and on cut section revealed a whitish mass extending from the submucosa upto the serosa. Representative sections from each of the grossly abnormal areas and the resected margins were submitted for processing.\nSections from the polyp showed a polypoidal mass with a core containing smooth muscle fibers and was identified as a Peutz-Jeghers polyp. Sections from the ulcerated areas showed edematous lamina propria with a dense infiltration of eosinophils and lymphocytes with congested blood vessels in the submucosa and a dense lymphocytic infiltration in the muscularis propria [] and serosa. Sections from the lymph node showed partial loss of architecture, areas of necrosis and fibrosis with epithelioid cell aggregates but no definite granuloma was made out. Multiple sections from the thickened areas showed diffuse sheets of lymphocytes ulcerating the mucosa and infiltrating transmurally upto the serosa and periserosal fat. Rest of the mucosa showed focal erosions, increased eosinophilic and lymphocytic infiltrates in the lamina propria and increased intra-epithelial lymphocytes with focal shortening of villi. In view of the high suspicion of tuberculosis and lymphoma, further diagnostic modalities were sought after such as acid fast staining, polymerase chain reaction (PCR) and immunohistochemistry.\nZiehl–Neilson acid fast staining revealed numerous acid fast bacilli. The PCR was positive for tubercle bacilli. A lymphoma panel of immunohistochemical markers showed CD 45 and CD 20 positivity [] with weak positivity for Bcl2. The rest of the IHC markers such as cytokeratin, chromogranin, CD 3 and CD 10 were negative.\nThus, a final diagnosis of primary small intestinal B-cell lymphoma, tuberculosis and Peutz-Jeghers polyp was made.
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.