소장 신경내분비암종을 동반한 Peutz-Jeghers 증후군 1예
A Case of Peutz-Jeghers Syndrome with Small Bowel Neuroendocrine Carcinoma
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초록
PJS는 위장관의 다발성 과오종성 용종증과 피부 점막의색소 침착을 특징으로 하며, 상염색체 우성으로 유전하는 드문 질환으로, 위장관 출혈이나 장폐색, 장중첩증 등의 합병증을 야기할 수 있다. PJS에서는 위장관 및 타 장기에서의악성종양의 발생 빈도가 높으나, 위장관에서 보고된 종양의경우 대부분 선암이며, PJS가 신경내분비종양과 동반된 경우는 아직 국내에 보고된 바 없다. 저자들은 PJS 환자에서발생한 소장 기원의 소세포 신경내분비암종 1예를 경험하였기에 문헌고찰과 함께 보고하는 바이다.
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.
참고문헌 (10)
- Giardiello FM, "Very high risk of cancer in familial Peutz-Jeghers syndrome", Gastroenterology, 119, pp.1447~1453 (2000)
- Sun JM, "Gastrointestinal carcinoid tumor", Korean J Gastroenterol, 44, pp.59~65 (2004)
- Foley TR, "Peutz-Jeghers syndrome : a clinicopathologic survey of the"Harrisburg family"with a 49-year follow-up", Gastroenterology, 95, pp.1535~1540 (1998)
- Van Lier MG, "High cancer risk and increased mortality in patients with Peutz-Jeghers syndrome", Gut, 60, pp.141~147 (2011)
- Perzin KH, "Adenomatous and carcinomatous changes in hamartomatous polyps of the small intestine(Peutz-Jeghers syndrome) : report of a case and review of the literature", Cancer, 49, pp.971~983 (1982)
- Sim YS, "A case of multiple endocrine neoplasia type 1 with pulmonary large cell neuroendocrine carcinoma", Korean J Med, 72, pp.214~220 (2007)
- Lin CW, "Primary hepatic carcinoid tumor : a case report and review of the literature", Cases J, 2, pp.90~ (2009)
- 고평곤, "Peutz-Jeghers 증후군 환자에서 발생한 췌장 두부의 관내 유두상 점액성 악성 종양 1예", 대한소화기학회지, 55(1), pp.73~77 (2010), 대한소화기학회
- Wada K, "Rectal carcinoid tumor associated with the Peutz-Jeghers syndrome", J Gastroenterol, 33, pp.743~746 (1998)
- Chen XD, "Duodenal intussusception due to a giant neuroendocrine carcinoma in a patient with Peutz-Jeghers syndrome : case report and systematic review", Eur J Gastroenterol Hepatol, 24, pp.722~726 (2012)