Yonsei Med J.  2007 Dec;48(6):1039-1042. 10.3349/ymj.2007.48.6.1039.

Two Separated Ileal Adenocarcinomas in Neurofibromatosis Type 1

• Hwangbo S
• Kim J
• Kim H
• Kim J
• Kang C
• Lee H

• KMID: 1786222
• DOI: http://doi.org/10.3349/ymj.2007.48.6.1039

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.

Keyword

Neurofibromatosis; ileum; adenocarcinoma

MeSH Terms

Adenocarcinoma/complications/*pathology
Adult
Humans
Ileal Neoplasms/complications/*pathology
Ileum/pathology
Intestinal Mucosa/pathology
Male
Neurofibromatosis 1/complications/*pathology

Figure

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  Fig. 1 Barium enema reveals a long segmental small bowel dilatation and multiple smooth nodular filling defects in the distal ileum.

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  Fig. 2 Coronal reformatted CT image reveals thickened mucosa, submucosa and muscle layer and serosa layer in the distal ileum. Two polypoid masses are seen, representing well enhanced thickened mucosa. Innumerable small nodular lesions are scattered in mesentery.

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  Fig. 3 Two ill-defined polypoid masses with diffuse edematous mucosal lesions in the ileum.

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  Fig. 4 Cross section of the intestinal wall revealed edematous submucosa and thick fibrotic muscle layer in the involved segment, which is contrasted with a continuous thin normal segment.

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  Fig. 5 (A) Diffuse transmural neurofibroma and overlying tubular adenoma of small bowel (hematoxylin-eosin, original magnification × 100). (B) Adenocarcinoma arising from tubular adenoma (hematoxylin-eosin, original magnification × 200).

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  Fig. 6 (A) Tubular adenoma glands resembling colitis cystica profunda (original magnification × 40). (B) Prominent ganglion cells in the mucosa (original magnification × 400).

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