Clinical Features, Natural History, and Outcomes of Intraneural Perineuriomas: A Systematic Review of the Literature

Background: Intraneural perineurioma is a rare peripheral nerve sheath tumor characterized by localized proliferation of perineurial cells. The literature consists predominantly of case reports and institutional series, with inconsistent and confusing nomenclature. We present a pooled analysis of all reported cases of intraneural perineurioma in the literature.

Methods: A systematic search of PubMed, MEDLINE, Embase, and Scopus was performed according to PRISMA guidelines to identify all reported cases of intraneural perineurioma in the literature. Individual cases were pooled and analyzed for demographics, clinical features, and outcomes.

Results: A total of 172 cases were identified across 72 studies, of which 149 were found in major peripheral nerves and their branches. Median age of patients at diagnosis and onset of symptoms was 18 years (interquartile range [IQR], 12-34 years) years and 13.5 years (IQR, 8-26 years), respectively, with 54.4% (81/149) being female. The most common sites were the sciatic nerve or its branches (41.9%), median nerve (13.5%), radial nerve (12.2%), and brachial plexus (12.2%). Most patients were managed conservatively (52.9%; 72/136). Among those managed conservatively with available follow-up (n = 31), median follow-up was 11 months, and most (67.7%, n = 21) reported no change in neurologic status, although worsening was noted in 29.0% (9/31). Among surgically managed cases (n = 64), the most common intervention was resection with or without repair (62.5%; 40/64), neurolysis (25%; 16/64), or tendon transfers without resection (12.5%; 8/64). No lesion recurred after surgical resection.

Conclusions: Intraneural perineurioma represents a benign focal lesion presenting with weakness and atrophy affecting adolescents and young adults. Most cases are managed nonoperatively and surgical treatment strategies are varied.