소장 신경내분비암종을 동반한 Peutz-Jeghers 증후군 1예 = A Case of Peutz-Jeghers Syndrome with Small Bowel Neuroendocrine Carcinoma

다국어 초록 (Multilingual Abstract)

Peutz-Jeghers syndrome (PJS) is an autosomal dominant inherited disorder characterized by hamartomatous polyps in the gastro-intestinal tract and mucocutaneous melanin pigmentation. Hamartomas are not generally regarded as premalignant, although pa-tients 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. (Korean J Med 2013; 84:698-703)

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