Perineurioma: a distinctive and underrecognized peripheral nerve sheath neoplasm

Area of Science:

• Pathology
• Oncology
• Neurosurgery

Background:

• Perineuriomas are benign peripheral nerve sheath neoplasms originating from perineurial cells.
• They present diagnostic challenges due to overlapping histology with other tumors.
• Classification traditionally includes intraneural and extraneural types.

Purpose of the Study:

• To comprehensively review perineurioma aspects: clinical, morphologic, immunohistochemical, ultrastructural, cytogenetic, and molecular.
• To discuss clinicopathologic variants and differential diagnoses of perineurioma.

Main Methods:

• Systematic literature review of English-language publications from 1966 to 2005.
• Analysis of clinical, pathological, and diagnostic features from reviewed studies.

Main Results:

• Perineuriomas exhibit characteristic immunohistochemical and ultrastructural findings.
• Histologic diagnosis requires confirmation via immunohistochemistry (e.g., EMA, S100, claudin-1, GLUT1) or electron microscopy.
• Cytogenetic and molecular studies have limited diagnostic utility but aid in differential diagnosis and understanding neoplasm biology.

Conclusions:

• Accurate identification of perineuriomas is essential to avoid overtreatment.
• Immunohistochemical studies and electron microscopy are key for confirming histologic diagnosis.
• Further research into cytogenetic and molecular aspects may elucidate perineurioma biology and aid in differential diagnosis.