Multiple neurofibrosarcoma in a patient with Neurofibromatosis-1 : a case report

ABSTRACT

Neurofibromatosis-1 is a multi-systemic; genetic and progressive disease. The patients have an increased risk of malignant transformation. Neurofibrosarcoma is rare but when it occurs; may pursue an aggressive course. This case report is of a 30-year-old female patient who first presented at the University of Calabar Teaching Hospital in November 2004 with a neurofibrosarcoma of the right foot complicating a neurofibroma of the same foot first noticed 27 years earlier. She had a right below knee amputation. Fourteen months later she presented with a huge tumour involving the right lower and middle lobes for which a right lower and middle lobectomy was done. The histology of the specimen confirmed neurofibrosarcoma. In the developing world late presentation is common with increased risk of malignant transformation. Early diagnosis and follow-up; genetic coun- selling of patients and relations on early presentation would improve outcome: