PMC4532108_01
Multiple, 1-2 cm sized, flesh colored neurofibromas on the back (a) and cafe-au-lait spots on the chest wall (b).
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There were multiple 1-2 cm sized neurofibromas on the whole body, especially on the back (Fig. 1).
Small bowel series showing no abnormal mucosal changes or filling defect.
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The UGI series and small bowel series (Fig. 2) revealed no specific abnormal findings.
Radionuclide angiogram using 99mTc pertechnate-labelled RBC 20 mCi intravenously and taken 30 minutes after injecion showing an abnormal accumulation of radioactivity in the pelvic cavity (arrow).
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The radionuclide scanning, using 99mTc pertechnate-labelled RBC, revealed abnormal radioactivity in the right lower abdomen and increased activity by the time sequence, which was suggestive of lower gastrointestinal bleeding from the right hypogastric arterial branch (Fig. 3).
Superior mesenteric angiogram showing abnormal contrast media extravasation from ileal artery (arrow).
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The superior mesenteric and ileal arteriogram showed bleeding from the left ileal arterial branches (Fig. 4).
A polypoid mass of the jejunum is protruded into the serosa and bowel lumen. It is covered by the serosa and intact mucosa.
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The pathologic diagnosis was neurofibromas of the jejunum (Fig. 5-, 8) and the skin (Fig. 9).
The neurofibroma of the jejunal mass showing dark staining axons and Schwann cell cylinders separated by mucinous matrix. The stroma is dotted with occasional mast cells and lymphocytes (H & E, x400).
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The pathologic diagnosis was neurofibromas of the jejunum (Fig. 5-, 8) and the skin (Fig. 9).