Korean J Intern Med.  2010 Jun;25(2):221-223. 10.3904/kjim.2010.25.2.221.

A Large Intrathoracic Meningocele in a Patient with Neurofibromatosis-1

Affiliations
  • 1Department of Internal Medicine, Korea Cancer Center Hospital, Seoul, Korea. jclee@kcch.re.kr
  • 2Department of Neurosurgery, Korea Cancer Center Hospital, Seoul, Korea.
  • 3Department of Radiology, Korea Cancer Center Hospital, Seoul, Korea.

Abstract

A large intrathoracic meningocele, a saccular protrusion of the meninges through a dilated intervertebral foramen or a bony defect of the vertebral column, was diagnosed in a 41-year-old female patient showing clinical features of neurofibromatosis-1 (NF-1), including cafe-au-lait spots, cutaneous neurofibromas, and axillary frecklings and Lisch nodules on the iris. Her daughter and son also had similar manifestations of NF-1. Regular follow-up with periodic imaging was recommended without surgical treatment because there were no signs or symptoms. Meningocele should be differentiated from posterior mediastinal tumors such as neurofibroma, neuroblastoma, and ganglioneuroma because NF-1 has a high risk of tumor formation. We report on this case with a brief review of the literature.

Keyword

Meningocele; Neurofibromatosis 1

MeSH Terms

Adult
Diagnosis, Differential
Female
Humans
Incidental Findings
Meningocele/*complications/*pathology
Neurofibromatosis 1/*complications/genetics/*pathology
Pedigree
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