Rare case of multiple neurofibromas of the scalp and trunk in association with intradural extramedullary spinal tumor: a case report

Abstract

Introduction and importance: Neurofibromas are tumors of neural connective tissue composed of Schwann cells and fibroblasts. They can occur anywhere in the body, primarily as a solitary mass or as a component of neurofibromatosis. Only 1/4 of the intradural extramedullary spinal tumors are neurofibromas. While most of the neurofibromas are asymptomatic, the authors report a patient with symptomatic multiple neurofibromas of the scalp and trunk that lacked the salient features of neurofibromatosis-1. Case presentation: A 63-year-old male from Saptari, Nepal, with multiple insidious swellings in the scalp and trunk region, presented with complaints of weakness of the bilateral lower limb for 6–7 years resulting in difficulties in walking and bleeding from the most significant swelling that was in the occipital area. Clinical findings and investigations: Masses were present all over the scalp, averaging about 4×4 cm, with active bleeding from the two swellings. The authors performed the neurological assessment and histopathological and radiological investigations. Interventions and outcomes: A confirmed diagnosis of neurofibroma was made with myelography revealing an intradural extramedullary spinal tumor. A laminectomy with total excision of the tumor was performed. Relevance and impact: Meningiomas and nerve sheath tumors (schwannomas and neurofibromas) can both develop in the intradural extramedullary spinal compartment. Contrast-enhanced MRI is the most sensitive and specific imaging modality to evaluate possible spinal column lesions. Surgical excision, partial or complete, is the hallmark treatment of neurofibroma.