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Table of Contents    
NEUROIMAGES
Year : 2018  |  Volume : 66  |  Issue : 5  |  Page : 1521-1522

Mesenteric plexiform neurofibroma


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 Publication17-Sep-2018

Correspondence Address:
Dr. Li Yang
Department of Neurology, The Second Xiangya Hospital, Central South University, Changsha, Hunan
China
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.241333

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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

How to cite this URL:
Yang L, Tan X, Bai HX, Huang Y, Wojcik J, Song S. Mesenteric plexiform neurofibroma. Neurol India [serial online] 2018 [cited 2018 Oct 23];66:1521-2. Available from: http://www.neurologyindia.com/text.asp?2018/66/5/1521/241333




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].[1],[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.[3] The extensive involvement can make surgical excision difficult.[4] 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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Financial support and sponsorship

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Conflicts of interest

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 » References Top

1.
Singh SK, Mankotia DS, Borkar SA, Gupta UD. Multiple mirror image cervical neurofibromas in neurofibromatosis type 1. Neurol India 2017;65:428-9  Back to cited text no. 1
    
2.
Yeung CY, Li TY. An unexpected manifestation of extensive plexiform neurofibroma. Neurol India 2017;65:916  Back to cited text no. 2
    
3.
Levy AD, Patel N, Dow N, Abbott RM, Miettinen M, Sobin LH. From the archives of the AFIP: Abdominal neoplasms in patients with neurofibromatosis type 1: Radiologic-pathologic correlation. Radiographics 2005;25:455-80.  Back to cited text no. 3
    
4.
Park J. Mesenteric plexiform neurofibroma in an 11-year-old boy with von Recklinghausen disease. J Pediatr Surg 2007;42:E15-8.  Back to cited text no. 4
    


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