Sporadic Schwannomatosis: A Systematic Review Following the 2005 Consensus Statement

J Neurol Surg A Cent Eur Neurosurg. 2018 Sep;79(5):408-415. doi: 10.1055/s-0038-1655548. Epub 2018 Jun 14.


Aims: To identify the frequency of reports of sporadic schwannomatosis, the types of patients affected, and the nerves affected.

Patients and methods: We identified all case reports and case series that reported on patients with sporadic schwannomatosis according to established criteria.

Results: The initial search yielded 1,597 studies, of which 15 were included. A total of 38 of 55 individuals met the inclusion criteria. The mean age of the patients was 48 years; 41% were male. Thirty-three patients had peripheral nerve tumors, and 17 had spinal tumors. Twelve had tumors in both locations. Tumor distribution was unilateral in 25 of 30 cases (83.3%) and segmental (limited to one limb or five or fewer contiguous segments of the spine) in 28 of 38 cases (73.7%).

Conclusion: This systematic review quantified the number of individuals who meet the criteria for sporadic schwannomatosis and better described this population to facilitate the classification of neurofibromatosis in regard to the 2005 consensus statement. Unilateral or segmental distribution of nerve tumors are key aspects when dealing with multiple noncutaneous schwannomas without involvement of the vestibular nerve.

Publication types

  • Review
  • Systematic Review

MeSH terms

  • Adult
  • Female
  • Humans
  • Male
  • Middle Aged
  • Neurilemmoma / classification
  • Neurilemmoma / diagnosis*
  • Neurofibromatoses / classification
  • Neurofibromatoses / diagnosis*
  • Skin Neoplasms / classification
  • Skin Neoplasms / diagnosis*

Supplementary concepts

  • Schwannomatosis