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Two Separated Ileal Adenocarcinomas in Neurofibromatosis Type 1

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초록

Patients with neurofibromatosis-1 (NF-1) have increased susceptibility to a variety of malignancies. Here, we document a rare case of two separated ileal adenocarcinomas in NF-1. The adenocarcinomas were surrounded by a diffuse tubular adenomatous lesion of the mucosa, and ganglion cells were scattered in the NF background. We found this case meaningful for several reasons: two separated adenocarcinomas arising in an unusual ileal segment, the association with precancerous tubular adenoma, and the presence of ganglion cells, which suggests ganglioneuromatosis in NF-1.

Abstract

Patients with neurofibromatosis-1 (NF-1) have increased susceptibility to a variety of malignancies. Here, we document a rare case of two separated ileal adenocarcinomas in NF-1. The adenocarcinomas were surrounded by a diffuse tubular adenomatous lesion of the mucosa, and ganglion cells were scattered in the NF background. We found this case meaningful for several reasons: two separated adenocarcinomas arising in an unusual ileal segment, the association with precancerous tubular adenoma, and the presence of ganglion cells, which suggests ganglioneuromatosis in NF-1.

참고문헌 (10)

  1. Korf BR, "Malignancy in neurofibromatosis type 1", 5, pp.477~485 (2000)
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  3. Hochberg FH, "Gastrointestinal involvement in von Recklinghausen's neurofibromatosis", pp.1144~51
  4. Nelson AM, "Small bowel adenocarcinoma associated with neurofibromatosis", pp.149~51
  5. Bridge MF, "Adenomas of the small intestine a clinicopathologic review of 51 cases and a study of their relationship to carcinoma", pp.799~819
  6. Sellner F, "Investigations on the significance of the adenoma-carcinoma sequence in the small bowel", pp.702~15
  7. Chambonniere ML, "Intestinal ganglioneuromatosis diagnosed in adult patients", 27, pp.219~224 (2003)
  8. Shekitka KM,Sobin LH, "Relation to Von Recklinghausen disease and other multiple tumor syndromes", 250-7
  9. Snover DC, "Diffuse mucosal ganglioneuromatosis of the colon associated with adenocarcinoma", pp.225~9
  10. Smith PA, "Report of a case in a patient with neurofibromatosis", pp.105~9

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