The Korean Journal of Medicine

The Korean Association of Internal Medicine (대한내과학회)
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Two Cases of Neuroendocrine Carcinoma and GIST in a Patient with Neurofibromatosis Type 1

제1형 신경섬유종증 환자에서 신경내분비종양과 위장관기질종양 2예

  • Choi, Won-Young (Department of Internal Medicine, Chonnam National University Medical School) ;
  • Hong, Seung-Dok (Department of Internal Medicine, Chonnam National University Medical School) ;
  • Kim, Ha-Na (Department of Internal Medicine, Chonnam National University Medical School) ;
  • Choi, Yoo-Duk (Department of Pathology, Chonnam National University Medical School) ;
  • Hwang, Jun-Eul (Department of Internal Medicine, Chonnam National University Medical School) ;
  • Shim, Hyun-Jeong (Department of Internal Medicine, Chonnam National University Medical School) ;
  • Bae, Woo-Kyun (Department of Internal Medicine, Chonnam National University Medical School)
  • 최원영 (전남대학교 의과대학 내과학교실) ;
  • 홍승덕 (전남대학교 의과대학 내과학교실) ;
  • 김하나 (전남대학교 의과대학 내과학교실) ;
  • 최유덕 (전남대학교 의과대학 병리학교실) ;
  • 황준일 (전남대학교 의과대학 내과학교실) ;
  • 심현정 (전남대학교 의과대학 내과학교실) ;
  • 배우균 (전남대학교 의과대학 내과학교실)
  • Published : 2011.12.01
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Abstract

Patients with neurofibromatosis type 1 (NF1) are at increased risk of developing tumors throughout the gastrointestinal tract, including neuromas, gastrointestinal stromal tumors (GISTs), and periampullary somatostatin-rich carcinoids. The simultaneous occurrence of a GIST and a well-differentiated neuroendocrine carcinoma in a patient with NF1 is very rare. Here, we report two cases of the coexistence of a low-risk GIST in the jejunum with a well-differentiated neuroendocrine carcinoma in the duodenum in patients with NF1. These cases strengthen the known association of GIST with neuroendocrine carcinoma in patients with NF1.

제1형 신경섬유종증 환자에서 신경내분비종양과 위장관기질종양의 발생률이 일반인구에 비해 높다는 것은 잘 알려져 있지만 이들이 함께 발병한 경우는 전 세계적으로도 보고된 증례들이 매우 드물며 국내에서는 저자들이 알기에는 아직 보고된 바가 없다. 이에 저자 등은 제1형 신경섬유종증 환자에서 비기능성 고분화 신경내분비종양과 위장관기질종 양이 함께 발생한 2예를 경험하여 문헌고찰과 함께 보고하는 바이다.

Keywords

References

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