e-ISSN 1309-2545      ISSN 1301-062X
TR    ENG
 

Download Current Issue.

Volume : 26 Issue : 2 Year : 2020

Current Issue Archive Popular Articles Ahead of Print Submit Your Article Login
Turkish Journal of Neurology Indexed By
  Neurofibromatosis Type 1-related Multiple Plexiform Neurofibromas: A Case Report [Turk J Neurol]
Turk J Neurol. 2020; 26(1): 42-46 | DOI: 10.4274/tnd.2019.44520  

Neurofibromatosis Type 1-related Multiple Plexiform Neurofibromas: A Case Report

Tamer Bayram1, Derya Bayram2, Hülya Tireli3
1Beykoz State Hospital, Clinic of Neurology, Istanbul, Turkey
2University of Health Sciences Turkey, Sancaktepe Şehit Prof. Dr. İlhan Varank Training and Research Hospital, Clinic of Neurology, Istanbul, Turkey
3İstanbul Haydarpaşa Numune Training and Research Hospital, Clinic of Neurology, Istanbul, Turkey

Neurofibromatosis type 1 (NF1) and type 2 (NF2) are autosomal dominant diseases. Patients with NF1 or NF2 have the potential to develop benign and malignant tumors. We present a 17-year-old patient with undiagnosed NF1-related multiple plexiform neurofibromas (PNs). The patient was admitted to our department for his left limb paresthesia. He had café-au-lait spots spread over his body and his cervical, thoracic, and lumbosacral magnetic resonance imaging (MRI) of the spine showed multiple PNs. Among his family members, only his father had café-au-lait spots. He refused to have genetic analysis and biopsy of the lesions. Even though clinical symptoms of neurofibromas vary according to the localization of lesions, it is known that PNs could be asymptomatic throughout life. Surgical treatment for PNs is essential. Phase 2 clinical trials have been performed for the medical treatment of PNs and further research on this subject is ongoing. Extensive growth of the tumors and invasion of surrounding tissues made resection of PNs difficult in the patient. Therefore, he had annual MRI follow-ups to assess the growth rate of the neurofibromas and transformation to malignant peripheral nerve-sheath tumors. This case was demonstrative to show multiple PNs. The presence of as much multiple PNs as in our patient is rare.

Keywords: Neurofibromatosis type 1, plexiform neurofibroma, spinal tumor


Tamer Bayram, Derya Bayram, Hülya Tireli. Neurofibromatosis Type 1-related Multiple Plexiform Neurofibromas: A Case Report. Turk J Neurol. 2020; 26(1): 42-46

Corresponding Author: Tamer Bayram, Türkiye


TOOLS
English Full Text
Print
Download citation
RIS
EndNote
BibTex
Medlars
Procite
Reference Manager
Share with email
Share
Send email to author

Similar articles
PubMed
Google Scholar




 
© Copyright 2020 Turkish Journal of Neurology
Home        |        Contact
LookUs & OnlineMakale