Orbital peripheral nerve sheath tumors

Area of Science:

• Ophthalmology
• Neuropathology
• Oncology

Background:

• Peripheral nerve sheath tumors (PNSTs) affecting the orbit and ocular adnexa are uncommon.
• These neoplasms often exhibit nonspecific clinical signs, diverse locations, and complex management, posing diagnostic difficulties.

Purpose of the Study:

• To provide a comprehensive overview of orbital and ocular adnexal PNSTs.
• To summarize current knowledge on their clinical, histopathologic, radiological, and molecular characteristics.

Main Methods:

• Literature review of PNSTs in the orbit and ocular adnexa.
• Synthesis of information regarding clinical presentation, histopathology, imaging findings, and molecular alterations.

Main Results:

• PNSTs in this region present with varied clinical manifestations and locations.
• Histopathologic, radiological, and emerging molecular features are crucial for accurate diagnosis and classification.

Conclusions:

• Understanding the multifaceted features of orbital PNSTs is essential for effective diagnosis and treatment.
• Further research into molecular aspects may offer novel therapeutic targets.