Korean J Spine.  2015 Jun;12(2):91-94. 10.14245/kjs.2015.12.2.91.

Multiple Schwannomas of the Spine: Review of the Schwannomatosis or Congenital Neurilemmomatosis: A Case Report

• Lee SH
• Kim SH
• Kim BJ
• Lim DJ

• KMID: 1927566
• DOI: http://doi.org/10.14245/kjs.2015.12.2.91

Abstract

Schwannomas are the most common benign nerve sheath tumors originating in Schwann cells. With special conditions like neurofibromatosis type 2 or entity called schwannomatosis, patients develop multiple schwannomas. But in clinical setting, distinguishing schwannomatosis from neurofibromatosis type 2 is challengeable. We describe 58-year-old male who presented with severe neuropathic pain, from schwannomatosis featuring multiple schwannomas of spine and trunk, and underwent surgical treatment. We demonstrate his radiologic and clinical findings, and discuss about important clinical features of this condition. To confirm schwannomatosis, we performed brain magnetic resonance imaging, and took his familial history. Staged surgery was done for pathological confirmation and relief of the pain. Schwannomatosis and neurofibromatosis type 2 are similar but different disease. There are diagnostic hallmarks of these conditions, including familial history, pathology, and brain imaging. Because of different prognosis, the two diseases must be distinguished, so diagnostic tests that are mentioned above should be performed in caution.

Keyword

Neurofibromatosis type 2; Schwannoma; Schwannomatosis; Spine

MeSH Terms

Brain
Diagnostic Tests, Routine
Humans
Magnetic Resonance Imaging
Male
Middle Aged
Nerve Sheath Neoplasms
Neuralgia
Neurilemmoma*
Neurofibromatosis 2
Neuroimaging
Pathology
Prognosis
Schwann Cells
Spine*