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|Year : 2018 | Volume
| Issue : 5 | Page : 1521-1522
Mesenteric plexiform neurofibroma
Li Yang1, Xia Tan1, Harrison X Bai2, Yuqian Huang3, John Wojcik4, Sharon Song4
1 Department of Neurology, The Second Xiangya Hospital, Central South University, Changsha, Hunan, China
2 Department of Radiology, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania, USA
3 Department of Neurology, The First Xiangya Hospital, Central South University, Changsha, Hunan, China
4 Department of Pathology and Laboratory Medicine, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania, USA
|Date of Web Publication||17-Sep-2018|
Dr. Li Yang
Department of Neurology, The Second Xiangya Hospital, Central South University, Changsha, Hunan
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Yang L, Tan X, Bai HX, Huang Y, Wojcik J, Song S. Mesenteric plexiform neurofibroma. Neurol India 2018;66:1521-2
A 17-year old female patient presented to the emergency department with intermittent crampy abdominal discomfort, most severe in the right lower quadrant for approximately a year and intermittent nausea and diarrhea for a week. Computed tomography showed a large mesenteric mass [Figure 1], which was determined, on pathology, to be a plexiform neurofibroma [Figure 2]., The parents of the patient reported that she underwent genetic testing around the age of 14–18 months because of multiple cafe au lait macules, but no diagnosis was made at that time. There was no known family history of neurofibromatosis type I (NF-1), but a paternal aunt has Birt–Hogg–Dubé syndrome. Her physical exam demonstrated axillary skinfold freckling. Genetic testing revealed an E17: c2851-4 T>G mutation in the NF1 gene. The nerves of the mesenteric plexuses give rise to plexiform neurofibromas of the mesentery, which can manifest as infiltrating lesions that extend from the root of the mesentery to the wall of the intestine. The extensive involvement can make surgical excision difficult. Our patient received no treatment. At follow-up, the patient continued to have intermittent but controllable abdominal pain.
|Figure 1: Coronal (a) and axial (b) CT images demonstrate an extensive mesenteric infiltrative process with extrinsic compression on, but with no involvement, of the small bowel. Arrows point to the extensive mesenteric process|
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|Figure 2: Histologic sections illustrate a proliferation of bland, loosely arranged spindled cells with round and wavy nuclei in a variably myxoid and collageneous matrix. Individual collagen fibers demonstrate a characteristic “shredded carrot” appearance often seen in neurofibromas. Hematoxylin and eosin sections (a and b), and immunohistochemical stain for S100 (c) are presented|
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[Figure 1], [Figure 2]