Uncommon Presentation Of Neurofibromatosis Focal Gigantism In The Upper Extremity
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Abstract
Neurofibromatosis with the uncommon presentation of focal gigantism of the left forearm in a 15 year old school boy is presented. There was no evidence of the disease elsewhere in the body. The radiographic features of a huge lobulated soft tissue mass and the
markedly thinned over tubulated and bowed bones of the forearm with cortical defects were suggestive of the disease and this was confirmed on histology. Angiographic features however raised suspicion of asarcomatous transformation. Extensive surgical
excision produced a good result with restoration of function. The clinical and radiological features are discussed.
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