Gastrointestinal Cancers in a Peutz-Jeghers Syndrome Family: A Case Report
- Affiliations
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- 1Department of Internal Medicine, CHA Bundang Medical Center, CHA University, Seongnam, Korea. sphong@cha.ac.kr
Abstract
- A 17-year-old man was diagnosed as Peutz-Jeghers syndrome (PJS) because of pigmented lip and multiple gastrointestinal polyps. He had anemia and underwent polypectomy on the duodenum and colon. His maternal family members were patients with PJS. His mother used to be screened with endoscopy to remove large polyps. One and half years later, he underwent jejunal segmental resection due to intussusceptions. He underwent endoscopic polypectomy every 2 to 3 years. When he was 23 years old, high-grade dysplasia was found in colonic polyp and his mother underwent partial pancreatectomy due to intraductal papillary mucinous carcinoma. When he was 27 years old, diffuse gastric polyps on the greater curvature of corpus expanded and grew. Therefore, wide endoscopic polypectomy was done. Histological examination revealed focal intramucosal carcinoma and low-grade dysplasia in hamartomatous polyps. We report cases of cancers occurred in first-degree relatives with PJS.
MeSH Terms
Figure
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Fig. 1 The pedigree of the patient. The arrow indicates the patient. His two uncles died of renal failure and lung cancer.
Fig. 2 (A) Abdominal computed tomography scan. (B) Multiple variable sized and multiseptated cystic lesions are shown in the pancreas. Pedunculated polyps are found in the jejunum.
Fig. 3 Histopathologic findings of the resected pancreas from the patient's mother. The intraductal papillary mucinous neoplasm with irregular branching papillae and cribriform growth showing architectural and cytological atypia (A, H&E stain, ×12; B, H&E stain, ×200).
Fig. 4 (A) Endoscopic findings of the patient. (B) Diffuse nodular and papillary polyps covering the greater curvature of the body. Focal hyperemic large nodular polyps are shown.
Fig. 5 Histopathologic findings of the gastric polypectomy specimen. Gastric polyp shows villous morphology and branching multi-layered nucleus foci of carcinomatous change (A, H&E stain, ×12; B, H&E stain, ×200).
Reference
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