Abstract

Appendiceal neurofibromas are exceedingly rare, with neither experimental nor observational data to support evidence-based diagnosis or treatment. We describe the case of a 52-year-old woman with neurofibromatosis 1 (NF1) complicated by aqueductal stenosis and resultant hydrocephalus needing a ventriculoperitoneal shunt (VPS). She presented to the emergency department with abdominal pain and was found to have abnormalities in the right hemiabdomen on cross-section imaging, also a Staphylococcus epidermidis growth at the distal portion of the VPS. She was initially treated with two rounds of intravenous antibiotics and VPS removal without improvement. She ultimately underwent an appendectomy, which revealed pathologic evidence of NF. The appendectomy was key to ruling out malignancy, addressing further symptoms and preventing future malignant transformation. This case highlights the importance of including appendiceal neurofibromas in the differential diagnoses of abdominal pain in patients with NF1.

Details

Title
Appendiceal neurofibroma in a patient with neurofibromatosis 1 and recurrent abdominal infections from ventriculoperitoneal shunt: a case report
Author
Greenberg, Anya L 1 ; Choi, Won-Tak 2 ; Shaked, Oren 3 ; Lee, Anthony T 4 ; Berrahou, Iman K 5 ; Jacques, Line G 4 ; Lebares, Carter C 3 

 School of Medicine , University of California, San Francisco, CA, USA 
 Department of Pathology , University of California, San Francisco, CA, USA 
 Department of Surgery , University of California, San Francisco, CA, USA 
 Department of Neurological Surgery , University of California, San Francisco, CA, USA 
 Department of Obstetrics & Gynecology , University of California, San Francisco, CA, USA 
Publication year
2021
Publication date
Apr 2021
Publisher
Oxford University Press
e-ISSN
20428812
Source type
Scholarly Journal
Language of publication
English
ProQuest document ID
3170796998
Copyright
Published by Oxford University Press and JSCR Publishing Ltd. All rights reserved. © The Author(s) 2021. This work is published under http://creativecommons.org/licenses/by-nc/4.0/ (the “License”). Notwithstanding the ProQuest Terms and Conditions, you may use this content in accordance with the terms of the License.