Abstract
BACKGROUND: Intraneural perineurioma is a rare, benign slow-growing lesion that usually involves a single main trunk nerve during childhood and young adulthood. The treatment of intraneural perineurioma is still a subject of controversy, especially in fast-growing children. To date, there was no systemic analysis of intraneural perineurioma in children. METHOD: A case of Intraneural perineurioma affecting the left sciatic nerve with 2 years of follow-up was presented. A systematic review was performed on literature published before June 2023, focusing on intraneural perineurioma diagnosed at no older than 18 years old. RESULT: A 9-year-old boy presented with progressive left foot-drop and abnormal gait for 2 years. The electromyography and magnetic resonance neurography study confirmed neuropathy involving the left sciatic nerves and its branches. Pathological investigation of the left sural nerve confirmed the diagnosis of intraneural perineurioma. The boy received physical therapy, and the disease was stable during the 2 years of follow-up. Fifty-seven childhood cases were identified in literature. Five patients with oral intraneural perineurioma underwent excision of the mass with good outcomes. In the other 52 patients with peripheral nerve involvement, 25 of them received surgical treatment, with different outcomes according to different operations. Out of 33 cases with precise lesion sizes, the length of the lesion in patients without nerve resection was significantly longer than that in patients with nerve resection (12.86 ± 7.44 cm vs 4.57 ± 4.5 cm. p < 0.05). CONCLUSIONS: Intraneural perineuriomas are rare benign tumors with slow progression. The options for surgery should be cautiously considered in childhood patients with long segmental peripheral nerve involvement.