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Perineuroma. A frequently unrecognized entity with emphasis on a plexiform variant
B Zelger1, G Weinlich, B Zelger
1Department of Pathology, University of Innsbruck, Austria.
Advances in Clinical Pathology : the Official Journal of Adriatic Society of Pathology
|August 11, 2000
Summary
Perineurioma, a rare skin tumor, is often misdiagnosed due to varied appearances, including a plexiform pattern. Accurate diagnosis is crucial for proper management of these nerve sheath tumors.
Area of Science:
- Dermatopathology
- Surgical Pathology
- Oncology
Background:
- Perineurioma is a rare tumor originating from peripheral nerve sheath cells.
- These tumors are frequently misdiagnosed due to their varied histological presentations.
- Recognition of perineurioma is important for accurate diagnosis and patient management.
Purpose of the Study:
- To describe the clinicopathologic features of six perineurioma cases.
- To highlight the diagnostic challenges and unusual presentations of perineurioma.
- To emphasize the importance of correct terminology for perineurioma.
Main Methods:
- Retrospective analysis of six surgically resected perineurioma specimens.
- Detailed histopathological examination including immunohistochemistry.
- Ultrastructural analysis in one case to confirm perineurial differentiation.
Main Results:
- All six perineuriomas occurred in adults on the upper extremities or shoulders, predominantly in females.
- Histological features varied, with common misdiagnoses including dermatofibroma and neurofibroma.
- Characteristic findings included "onion bulbs" and a variable mix of cellularity, nuclear shape, and stromal changes; one case showed a plexiform pattern.
Conclusions:
- Cutaneous and subcutaneous perineurioma is often unrecognized or misinterpreted.
- Plexiform perineurioma can mimic other plexiform lesions, necessitating careful differential diagnosis.
- The term perineurioma should be used consistently, avoiding imprecise historical terms.