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http://dx.doi.org/10.3904/kjm.2013.84.5.698

A Case of Peutz-Jeghers Syndrome with Small Bowel Neuroendocrine Carcinoma  

Kim, Seul Ki (Department of Gastroenterology, Internal Medicine, Dong-A University College of Medicine)
Jang, Jin Seok (Department of Gastroenterology, Internal Medicine, Dong-A University College of Medicine)
Han, Ji Sun (Department of Gastroenterology, Internal Medicine, Dong-A University College of Medicine)
Choi, Seok Reyol (Department of Gastroenterology, Internal Medicine, Dong-A University College of Medicine)
Kwon, Hee Jin (Department of Radiology, Dong-A University College of Medicine)
Kim, Su Jin (Department of Pathology, Dong-A University College of Medicine)
Publication Information
The Korean Journal of Medicine / v.84, no.5, 2013 , pp. 698-703 More about this Journal
Abstract
Peutz-Jeghers syndrome (PJS) is an autosomal dominant inherited disorder characterized by hamartomatous polyps in the gastrointestinal tract and mucocutaneous melanin pigmentation. Hamartomas are not generally regarded as premalignant, although patients with PJS are at increased risk for common and unusual types of gastrointestinal and non-gastrointestinal malignancies. However, most of the reported gastrointestinal malignancies have been adenocarcinomas, and few reports of an association of this syndrome with a neuroendocrine tumor (NET) have been published. Moreover, no case of this syndrome with NET has been reported in Korea. Here, we report a 21-year old male with PJS who had a small bowel neuroendocrine carcinoma.
Keywords
Peutz-Jeghers syndrome; Neuroendocrine carcinoma;
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