Neurofibromatosis and development of cancer in childhood

Neurofibromatosis takes two major forms; classical or peripheral neurofibromatosis as described by von Recklinghausen, which accounts for more than 90% of the cases, and central or bilateral acoustic neurofibromatosis. The diagnosis is often postponed until adulthood, since the classical signs gradu...

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Bibliographic Details
Published inTidsskrift for den Norske Lægeforening Vol. 109; no. 12; p. 1297
Main Authors Kolmannskog, S, Stokland, T, Anke, I M
Format Journal Article
LanguageNorwegian
Published Norway 30.04.1989
Subjects
Adolescent
Brain Neoplasms - etiology
Child
Child, Preschool
Eye Neoplasms - etiology
Female
Follow-Up Studies
Humans
Infant
Male
Neurofibromatosis 1 - complications
Retroperitoneal Neoplasms - etiology
Tomography, X-Ray Computed
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Summary:Neurofibromatosis takes two major forms; classical or peripheral neurofibromatosis as described by von Recklinghausen, which accounts for more than 90% of the cases, and central or bilateral acoustic neurofibromatosis. The diagnosis is often postponed until adulthood, since the classical signs gradually appear during childhood and adolescence. It is a relatively common autosomal dominant disorder affecting about one in 3,000. At least 20% of patients will develop one or more complications associated with neurofibromatosis. One of the complications is the development of malignancies. Four children at our hospital developed different forms of malignant tumours arising from neurofibromatosis. We recommend that all patients suffering from this disease are evaluated in detail after the diagnosis has been confirmed and are followed up every six to 12 months. In this way complications may be discovered early and the necessary steps taken.
ISSN:0029-2001
0807-7096