Low-grade astrocytoma of the spinal cord associated with neurofibromatosis type-1: report of a case with poor correlation between histopathology and prognosis

Neurofibromatosis (NF) is a genetic disorder, which primarily affects the skin and the central nervous system. NF Type 1 (NF-1), also known as von Recklinghausen’s disease, is the commoner subtype of NF and affects one in every 3000 individuals. The inheritance is autosomal dominant, although up to 50% of the cases may arise as somatic mutations. Genetic studies have mapped the responsible gene to chromosome 17. Diagnostic criteria for NF-1 include: FIG. 1. Sagittal (a,b) T1-weighted and (c,d) T2-weighted MRI demonstrates an intramedullary tumour between C5 and T5 that is hypointense on T1 and hyperintense on T2 images. Contrast-enhanced sagittal MR images (e, f) show striking enhancement at C5 – 6 levels. The periphery of the remaining tumour at C7 –T5 enhanced moderately while the core showed only mild but homogeneous enhancement.