Abstract
Objective Intraneural perineurioma is a rare tumor entity. It is a benign, very slow growing
peripheral nerve sheath tumor that typically occurs in children and young adults.
Motor deficits and muscle atrophy are classic presenting symptoms, while sensory deficits
are rare at the onset of the disease. Recommended treatment strategies are lacking.
We have evaluated the clinical follow-up and our experience with treatment of this
rare entity.
Methods A total of 30 patients with intraneural perineuriomas were assessed retrospectively.
Demographic data, clinical symptoms, diagnostic examinations, therapy strategies,
and clinical outcome were analyzed. Descriptive statistical methods were used for
evaluation.
Results The mean age was 22 years. Eleven women and 19 men were affected. The lesion occurred
in the area of the upper extremity in 16 patients and in the area of the lower extremity
in 14 patients. The most frequently affected nerve was the sciatic nerve, followed
by the radial nerve. All patients showed a motor deficit to some extent. Seventy percent
(n = 21) revealed atrophy, 43.3% (n = 13) had sensitive deficits, and 17% (n = 5) suffered of pain. Fascicle biopsies were performed in 26 patients (87%). In
four patients (13%), the tumor was completely resected and then reconstructed via
nerve grafts. Seventy percent of the patients (n = 21) received a magnetic resonance imaging (MRI) within 5 years postoperatively,
in which no progress was shown.
Conclusions To diagnose perineurioma, it is essential to take a biopsy of an enlarged, nonfunctional
fascicle. Furthermore, a long-distance epineuriotomy to decompress the hypertrophic
fascicle is reasonable. To preserve the nerves' residual function, a complete resection
is not recommended. Results after grafting are poor. One reason for this might be
residual tumor cells along the nerve that cannot be visualized. Malignant transformation
is not yet reported and tumor growth is stable for years.
Keywords
perineurioma - peripheral nerve sheath tumor - epineuriotomy - epithelial membrane
antigen
