J Korean Surg Soc.  2010 Dec;79(Suppl 1):S31-S36. 10.4174/jkss.2010.79.Suppl1.S31.

Gastric Outlet Obstruction due to Submucosal Neurofibromatous Proliferation of Duodenal Bulb in Neurofibromatosis Type 1 Patient

• Suh BS
• Shin DW
• Lee JS
• Kim SY
• Han EM
• Jang EJ

• KMID: 2040549
• DOI: http://doi.org/10.4174/jkss.2010.79.Suppl1.S31

Abstract

Neurofibromatosis type 1 (NF1; also known as von Recklinghausen's neurofibromatosis) is inherited in an autosomal dominant fashion, although it can also arise due to spontaneous mutation. Gastrointestinal involvement of NF1 is seen in 10% to 25% and causes symptoms in fewer than 5%. Histologically, the gastro intestinal (GI) manifestation of NF1 occurs in three forms: hyperplasia of the gut neural tissue, stromal tumors, and duodenal or periampullary endocrine tumors. A 31-year-old female, diagnosed with NF1, presented with poor oral intake and vomiting for 10 days prior to admission. Preoperative gastrofiberscopic finding was gastric outlet obstructing polypoid duodenal bulb lesion. The patient underwent hemigastrectomy with antecolic gastrojejunostomy due to gastric outlet obstruction. The final pathologic report was submucosal neurofibromatous proliferation with Brunner's gland hyperplasia located at the duodenal bulb in the NF1 patient. We report this case with a review of literatures.

Keyword

Neurofibromatosis type 1 (NF1); Gastric outlet obstruction

MeSH Terms

Adult
Female
Gastric Bypass
Gastric Outlet Obstruction
Humans
Hyperplasia
Neurofibromatoses
Neurofibromatosis 1
Vomiting

Figure

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  Fig. 1 Preoperative gastrofiberscopic findings of duodenal bulb polyp like lesion through the pyloric ring.

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  Fig. 2 Abdominopelvis MD-CT coronal scan revealed severely distended stomach due to gastric outlet obstruction.

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  Fig. 3 The arrow indicates an ill defined nodular elevated lesion in the duodenal bulb, measuring 3.6×3.2×1.0 cm (The gross specimen of resected stomach).

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  Fig. 4 (A) Low-power view reveals neurofibromatous proliferation and Brunner's gland hyperplasia in the submucosa (H&E, ×12.5). a = Brunner's gland hyperplasia in the submucosa; b = Myenteric plexus neural hyperplasia. (B) High-power view reveals interlacing bundles of neurons, schwann cells and fibroblasts (H&E, ×200). (C) The neural cells reveal positive immunoreactivity for S-100 protein (×200).

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