ABSTRACT
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.
