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The study goal was to determine the genetic (heritable) contribution to childhood brain tumors (CBT) which cause nearly one quarter of all childhood cancer deaths. Their etiology remains unknown, but previous studies have suggested a proportion of CBT may be heritable. In this study we collected family histories of 243 confirmed CBT patients referred to The University of Texas M. D. Anderson Cancer Center between the years 1944 and 1983, diagnosed before age 15, and residents of the United States or Canada. Family histories were obtained for all the probands' first degree relatives (parents, siblings, and offspring) and extended to include selected second degree relatives (aunts, uncles, grandparents) using sequential sampling. To determine if these CBT families exhibited excess cancer, we compared their cancer experience to age-, race-, sex-, and calendar-year specific rates from the Connecticut Tumor Registry. No cancer excess was observed among 1,099 first and second degree relatives [39 cancers observed (O) and 44 expected (E) for a standardized incidence ratio (SIR) of 0.88]. For colon cancer, although small numbers, five cases were observed among the probands' first degree relatives with 1.6 expected, for a significant SIR of 3.10. Segregation analysis demonstrated that chance alone could not account for the observed cancer distribution with a multifactorial model providing the best overall explanation of the data. Overall, heredity played a role in the etiology of CBT in 4% of the study families: four (1.7%) due to known hereditary syndromes (nevoid basal cell carcinoma syndrome and von Recklinghausens neurofibromatosis--NF-1), four (1.7%) with multifactorial inheritance, and two additional families with cancers aggregating similar to the clinical criteria described for the Li-Fraumeni cancer family syndrome.
M L Bondy, and
E D Lustbader, and
P A Buffler, and
W J Schull, and
R J Hardy, and
L C Strong
May 1996,
The Canadian journal of neurological sciences. Le journal canadien des sciences neurologiques,
M L Bondy, and
E D Lustbader, and
P A Buffler, and
W J Schull, and
R J Hardy, and
L C Strong
January 2000,
Zentralblatt fur Neurochirurgie,
M L Bondy, and
E D Lustbader, and
P A Buffler, and
W J Schull, and
R J Hardy, and
L C Strong
October 1999,
International journal of oncology,
M L Bondy, and
E D Lustbader, and
P A Buffler, and
W J Schull, and
R J Hardy, and
L C Strong
January 1992,
Pediatric hematology and oncology,
M L Bondy, and
E D Lustbader, and
P A Buffler, and
W J Schull, and
R J Hardy, and
L C Strong
September 2004,
Toxicology and applied pharmacology,
M L Bondy, and
E D Lustbader, and
P A Buffler, and
W J Schull, and
R J Hardy, and
L C Strong
June 1984,
Minerva medica,
M L Bondy, and
E D Lustbader, and
P A Buffler, and
W J Schull, and
R J Hardy, and
L C Strong
April 2018,
Zhong nan da xue xue bao. Yi xue ban = Journal of Central South University. Medical sciences,
M L Bondy, and
E D Lustbader, and
P A Buffler, and
W J Schull, and
R J Hardy, and
L C Strong
July 2011,
Nature reviews. Neurology,
M L Bondy, and
E D Lustbader, and
P A Buffler, and
W J Schull, and
R J Hardy, and
L C Strong
January 1992,
Journal of neuro-oncology,
M L Bondy, and
E D Lustbader, and
P A Buffler, and
W J Schull, and
R J Hardy, and
L C Strong
December 2013,
SpringerPlus,
