Pediatric peripheral nerve tumors: clinical and surgical aspects

Purpose Pediatric peripheral nerve tumors (PNTs) are rare. Most are related to neurofibromatosis type 1 (NF1) with the potential for malignancy. An ongoing debate occurs about the best approach to such patients. This study describes a cohort of pediatric patients with PNTs and discusses clinical characteristics and surgical treatment. Methods We retrospectively reviewed the charts of seven pediatric patients with eight PNTs surgically treated from 2007 to 2018. Information concerning patient demographics, clinical presentation, PNTs characteristics, treatment choice, and outcome were recorded. Results All children presented with intense pain and a palpable mass. Three of the eight tumors were associated with a neurological deficit. Among the four patients with NF1, two had a neurofibroma and two a malignant peripheral nerve sheath tumor (MPNST). Histologically, three of the lesions were a benign peripheral nerve sheath tumor (BPNST), three a MPNST, and one each a desmoid tumor and Ewing's sarcoma. Two of the eight tumors underwent partial tumor excision and six gross total excisions. Conclusions Intense pain at rest, day, and/or night, preventing normal activities; a palpable, hard, immobile mass; an intense Tinel's sign related to the lump; clinical evidence of NF1; and high-speed growth of a tumor in the trajectory of the nerve or plexus should alert the clinician to the potential for malignancy. Preoperative biopsy is not indicated when clinical and imaging findings suggest a benign tumor. The surgical management of PNTs must be to achieve total resection, including wide margins with malignant tumors, though this is not always possible.