Neurofibromatosis and Malignant Childhood Cancers: A Survey in Italy, 1970–83

Abstract

Neural tumors, Wilms’ tumor, rhabdomyosarcoma and several types of leukemia have been previously described in association with neurofibromatosis (NF). In a nation-wide collection of cases in Italy, 15 children (0–14 years of age) with NF and cancer or leukemia were identified; 13 of them had been diagnosed with cancer between 1976–83. The expected number of children with cancer and NF in 1976–83 was 4.48. The distribution of tumor types was different from that found in the general population, with a higher proportion of tumors of neural crest origin as well as soft tissue sarcomas. In 7/15 the family history was positive for NF; in 5/7 the individuals affected included the mother and/or a maternal relative.