PMC9479501_01
A 5-year-old male patient with NF-1 presented with abdominal pain and distention. (A) Abdominal X-rays on the first day admitted to the hospital showed a lot of air-fluid levels (white arrow) suggesting a small bowel obstruction. (B) There was an elimination in the quantity of air-fluid levels (white arrow) after one week compared to the first day. No subdiaphragmatic air crescent was seen.
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F1
His abdominal X-ray image showed a lot of air-fluid levels, >2.5 cm in width [Figure 1].
A 5-year-old male patient with NF-1 presented with abdominal pain and distention. Portal venous phase CT images: (A, B, C) Diffuse lesions of small bowel mesentery (white arrow), slightly enhancing and encasing the mesenteric vessels without invasion or causing vessel stenosis/obstruction. (D) Similar lesions surrounding the rectum (white arrow).
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F2
On the enhanced CT images, there were diffuse lesions of small bowel mesentery and transverse mesocolon, encasing the mesenteric vessels without invasion or causing vessel stenosis/obstruction [Figure 2].
A 5-year-old male patient with NF-1 presented with abdominal pain and distention. Intraoperative findings: (A) Dilated small bowel (white arrow); (B, C, D) The entire small bowel mesentery and the right half of transverse mesocolon had many yellow rigid nodules (white arrow), along the vessels.
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F3
The entire small bowel mesentery and the right haft of the transverse mesocolon were thickened with yellow nodular cord-like structures along the vessels [Figure 3].
A 5-year-old male patient with NF-1 presented with abdominal pain and distention. Pathologic images of the mesenteric lesions: H&E stain x40 (A), x200 (B, C) showed tumor tissue structure consisting of clusters of long slender spindle cells with wavy cytoplasm, slightly mucoid basal substances, blood vessels proliferation without malignant cells. (D) The immunohistochemical stain showed S100 (+).
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F4
The immunohistochemical staining results showed S100 (++), SOX10 (-), CD117 (-) [Figure 4].