Intraneural perineurioma: a retrospective study of 19 patients

Abstract

Intraneural perineurioma is a benign neoplasm of peripheral nerve sheath with perineurial cell origin that typically affects teenagers and young adults and tends to result in a motor-predominant neuropathy. The aim of our study is to present the epidemiology, clinical presentation, way of diagnosis and management plan in a consecutive patient series. Ninteen patients diagnosed as having intraneural perineurioma were retrospectively chart reviewed. Diagnosis was done by MRI and/or biopsy with morphological, immunohistochemical staining study confirmation. Patients assessments included gender, age, symptoms, tumor localization, radiological aspect, management and pathological confirmation.Sex ratio was 10 males to 9 females with mean age of 31.2 (15-64). All the patients presented with motor deficit, ten with sensory deficit. Upper limb was involved in 11 cases (among them 4 lesions of brachial plexus), the lower limb in 8 cases. On magnetic resonance imaging, 16 patients showed a nerve enlargement whereas 5 presented with atypical fusiform tumor. Eighteen patients were operated for excision biopsy and/or palliative treatment for their motor deficit. Anatomopathological analysis confirmed the diagnosis in seventeen cases with a morphological pseudo-onion bulb shape and/or specific immunohistochemical assay. One patient had only palliative treatment without excision biopsy. Our data confirmed the equal penetration of intraneural perineurioma to both sex and affected limb. Because of the benignity of the tumor, the surgical treatment focused on optimizing the functional outcome. A prospective study with long term follow-up is required for this under-diagnosed tumor.

Keywords

• intraneural perineurioma
• hypertrophic neuropathy
• pseudo-onion bulbs