Child plexiform neurofibroma of the lumbar region. Case report

Abstract

Tumors of the peripheral nerves are not well described. Plexiform neurofibromas are common NF1 benign tumors that carry a low malignant risk of transformation. In some area where the conventional therapies (clinical trial) are not available, surgery remains the gold standard although this one is not radical. There is not a lot of publications related to this subject in Africa. The aim of study is to share our experience and show the difficulties of managing this pathology in children.We report a case of a 4 year old boy without any family history of neurofibromatosis. He presented to our department of neurosurgery at renaissance hospital of Ndjamena (Chad) with primary skin concern about a right lumbar swelling which was progressive increase in size over the last few years. Physical examination revealed the presence of several cafe-au-lait macules (CALM) more than forty, freckling, hypertrichosis, hyperpigmentation, and thoraco-lumbar scoliosis without other associated lesions. Ultrasonography, CT scan and Magnetic resonance imaging (MRI) of the swelling was done, and gave features description of this lesion. The patient underwent surgery and the diagnosis of plexiform neurofibromatosis was made at the histological examination. After a follow-up of 16 months there is no tumor recurrence. A regular follow-up is necessary for this young patient.The surgery has an aesthetic purpose and it delays the progression of the disease.

Keywords

• Neurofibromatosis
• Plexiform
• Lumbar mass
• Surgery
• Child