Journal article
Surgical management of an appendiceal neurofibroma in a neurofibromatosis-1 patient: A case report
Journal of pediatric surgery case reports, Vol.3(11), pp.492-495
11/01/2015
DOI: 10.1016/j.epsc.2015.09.011
Abstract
Neurofibromatosis-1 (NF1) is an autosomal dominant disease with an incidence of 1 in every 2500 to 3000 births [1]. Although neurofibromas that result from the NF1 gene mutation are typically benign, they may cause complications by compressing neighboring structures or undergoing malignant transformation. The various manifestations and symptoms of NF1 may cause a number of complications throughout childhood and into adulthood, and patients often require surgical management of these complications. A young adult with a previous NF1 diagnosis presented with symptoms concerning for appendicitis. Subsequent imaging and biopsy revealed a rare appendiceal neurofibroma. The patient underwent an ileocecectomy and pathology confirmed the diagnosis of appendiceal neurofibroma. The variety of symptoms and complications associated with NF1, such as vascular abnormalities and airway obstruction, create unique surgical risks for these patients. These risks should be understood and accounted for by surgeons who may encounter NF1 patients.
•We present a Neurofibromatosis-1 patient with an appendiceal neurofibroma.•Appendiceal neurofibromas are a rare manifestation of Neurofibromatosis-1.•The primary treatment for symptomatic Neurofibromatosis-1 is surgical excision.•Neurofibromatosis-1 patients present with a unique set of surgical risk factors.
Details
- Title: Subtitle
- Surgical management of an appendiceal neurofibroma in a neurofibromatosis-1 patient: A case report
- Creators
- Averi Wilson - The University of Texas MD Anderson Cancer CenterJohn Slopis - The University of Texas MD Anderson Cancer CenterRichard Andrassy - Department of Surgery, The University of Texas Medical School at Houston, Houston, TX, USASadhna Dhingra - The University of Texas MD Anderson Cancer CenterMary Austin - The University of Texas MD Anderson Cancer Center
- Resource Type
- Journal article
- Publication Details
- Journal of pediatric surgery case reports, Vol.3(11), pp.492-495
- DOI
- 10.1016/j.epsc.2015.09.011
- ISSN
- 2213-5766
- eISSN
- 2213-5766
- Publisher
- Elsevier Inc
- Number of pages
- 4
- Language
- English
- Date published
- 11/01/2015
- Academic Unit
- Stead Family Department of Pediatrics; Internal Medicine
- Record Identifier
- 9985123695102771
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