Sarcomas in three generations of a family with neurofibromatosis.
The development of sarcomas in three generations of a family with neurofibromatosis (NF) is described. A 7-month-old boy developed a prostatic rhabdomyosarcoma. Family history revealed that his maternal grandmother had died of a malignant peripheral nerve sheath tumor at age 37 years and had been affected with neurofibromatosis. His mother (aged 36 years) has also developed a malignant peripheral nerve sheath tumor and NF has been confirmed in both mother and son. This family illustrates a cluster of sarcomas associated with NF and shows the importance of thorough investigation of reported family illnesses.
Hartley
A L
AL
Department of Epidemiology and Social Oncology, Christie Hospital and Holt Radium Institute, Manchester, England.
Birch
J M
JM
Kelsey
A M
AM
Harris
M
M
Jones
P H
PH
D000328:Adult; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D009386:Neoplastic Syndromes, Hereditary / Q000235:genetics*; D009456:Neurofibromatosis 1 / Q000235:genetics*; D010375:Pedigree; D010524:Peripheral Nervous System Neoplasms / Q000235:genetics; D011471:Prostatic Neoplasms / Q000235:genetics; D012208:Rhabdomyosarcoma / Q000235:genetics; D012509:Sarcoma / Q000235:genetics*