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KMID : 0882420130840050698
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Korean Journal of Medicine
2013 Volume.84 No. 5 p.698 ~ p.703
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A Case of Peutz-Jeghers Syndrome with Small Bowel Neuroendocrine Carcinoma
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Kim Seul-Ki
Jang Jin-Seok
Han Ji-Sun
Choi Seok-Reyol
Kwon Hee-Jin
Kim Soo-Jin
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Abstract
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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.
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KEYWORD
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Peutz-Jeghers syndrome, Neuroendocrine carcinoma
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