Neurofibroma of the scalp are mostly multiple as part of neurofibromatosis or other phakomatosis. De novo solitary types are less common and rarely erode the skull, unlike the intracranial counterpart. Skull erosion has been reported in adults with longstanding plexiform neurofibromas. We report a giant neurofibroma on the scalp of a five-year-old boy, managed in our center. Although this condition is a rare entity, it should be anticipated and the treatment strategy should include repair of the skull defect.
This technique is simple and provides a water-tight wound cover, with the pericranium giving reinforcement and better tensile strength, as well as a fairly good protection for the shunt valve. This is useful in preventing CSF leaks and exposure of the shunt with the associated morbidity and mortality.
Ganglioneuroma is a rare benign tumour that originates from the ganglion cells of the sympathetic nervous system. They are rare in cervical spine region and only 8 % of ganglioneuromas occur in the neck. The common sites of occurrence are in the posterior mediastinum, retroperitoneum and adrenal medulla, and as such, a cervical occurrence presenting with quadriplegia is a reportable event. We present a 26-year old young male with a two-year history of neck pain and progressive quadriplegia. He later became wheelchair-bound. Musculoskeletal examination revealed multiple generalized nodular skin swellings with café au lait macules. Magnetic resonance imaging showed a huge dumbbell tumour of the first two cervical vertebrae, to the right side of the spinal canal causing significant spinal cord compression. He had surgical intervention, aimed at complete tumour resection, postoperatively, power of the limbs improved to normal. Histological examination was consistent with ganglioneuroma. We present this report because the occurrence of ganglioneuroma is rare, secondly a cervical presentation is unusual and thirdly it presented as a rare cause of quadriplegia.
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