Generalized neurofibromatosis is a relatively rare condition, and its occurrence in children in a fully developed and clinically recognizable form is even more rare. Intrathoracic tumor in association with neurofibromatosis has been reported only a few times, and to our knowledge there is no record of a case of neurofibromatosis and thoracic tumor in a child in which the tumor underwent malignant change with metastasis.
Neurofibromatosis, or Recklinghausen's disease, is to be distinguished both from solitary neurofibroma, a condition which is found frequently, and from fibrocystic disease of the bone (hyperparathyroid disease), which also bears Recklinghausen's name. The incidence of neurofibromatosis has been variously estimated. Preiser and Davenport,1 for example, gave the figure as 1 case in every 2,000 in which patients present themselves because of a cutaneous condition. This figure probably does not take into account the very mild, the very early or the so-called abortive form of
MAKSIM G, HENTHORNE JC, ALLEBACH HK. NEUROFIBROMATOSIS WITH MALIGNANT THORACIC TUMOR AND METASTASIS IN A CHILD. Am J Dis Child. 1939;57(2):381–390. doi:10.1001/archpedi.1939.01990020139015
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