Comprehensive Analyses of Intraoral Benign and Malignant Nerve Sheath Tumors: The Rare Disease Entities Revisited

J Craniofac Surg. 2019 Jun;30(4):e317-e327. doi: 10.1097/SCS.0000000000005231.


Introduction: Intraoral benign and malignant nerve sheath tumors (BNST and MNST) are rare tumors with non-specific clinical presentations and represent diagnostic and therapeutic challenges. Current knowledge regarding their demographic, clinicopathological features and treatments remains fragmented.

Materials and methods: The original data about patients diagnosed as intraoral BNST and MNST were retrieved from our disease registry (2005-2017). Comprehensive reviews of English and Chinese literature were performed to collect and analyze the epidemiological, clinicopathological data and treatment outcomes about those published cases.

Results: Thirty-four intraoral BNSTs were found at our institution in the past 13 years. Literature reviews identified 354 intraoral BNSTs in 223 articles and 60 intraoral MNSTs in 50 articles. Most intraoral BNSTs and MNSTs were presented in the second to fifth decade of life. Males outnumbered females in MNSTs, while BNSTs displayed a slight female preponderance. The common sites for intraoral BNSTs were parapharyngeal space followed by tongue, whereas mandible was the most common site for MNSTs. Most intraoral BNSTs were presented as slow-growing, painless mass or swelling, while MNSTs usually appeared as painful and invasive mass with discomfort. Surgical excision was preferred for intraoral BNSTs with excellent prognosis. Complete resection was indicated for intraoral MNST with dismal prognosis as evidenced by much recurrence, metastasis, and death.

Conclusion: Intraoral BNST and MNST are rare diseases which should not be ignored when intraoral painless or painful mass/swelling is found. Surgical excision is indicated for intraoral BNST with favorable outcomes. However, further investigations are warranted to optimize the treatment for intraoral MNST to improve its prognosis.

Publication types

  • Review

MeSH terms

  • Adult
  • Female
  • Humans
  • Male
  • Middle Aged
  • Mouth Neoplasms*
  • Nerve Sheath Neoplasms*
  • Rare Diseases*