Malignant peripheral nerve sheath tumor of the orbit: a case report and review of the literature

ABSTRACT

Malignant peripheral nerve sheath tumor is a rare tumor which infrequently involves the orbit. They occur most often in the setting of neurofibromatosis 1 (NF1), and therefore the involvement of the orbit without a history of NF1 is even less common. Management of this tumor is fraught with a high rate of recurrences and metastases, with a high mortality rate. Primary surgical excision with tumor-free margins remains the primary treatment, while adjuvant modalities such as radiation and chemotherapy play a more minor role.

KEYWORDS:

• Malignant peripheral nerve sheath tumor
• neurofibromatosis
• orbital tumor

Disclosure statement

The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the article.

Meeting presentation

This material has not been previously presented at any meeting.