@article{7162a7eea86a4abea8cb28da68cbb13e,
title = "The Sarcoma, Breast Cancer, Lung Cancer, and Adrenocortical Carcinoma Syndrome Revisited: Childhood Cancer",
abstract = "We studied two children who had rhabdomyosarcoma and glioblastoma and who were from a family with a hereditary cancer syndrome that was characterized by sarcoma, breast cancer, brain tumors, lung cancer, laryngeal carcinoma, leukemia, and adrenocortical carcinoma. The deleterious genotype has now been expressed through the fourth generation of this large kindred. The pedigree emphasizes the need for an extended history of several generations to arrive at a hereditary-syndrome diagnosis. A limited pedigree may result in nonappreciation of the genetic component. The pedigree illustrates that, in certain circumstances, the highly specific varieties of cancer may occur in children before it is expressed in the parent who carries the putative gene. Pediatricians, in evaluating the causes of childhood cancer, must be cognizant of cancer among adult relatives, since this recognition may aid in the diagnosis of those hereditary cancer syndromes that are characterized by cancer occurrence in children as well as adults.",
author = "Lynch, {Henry T.} and Katz, {David A.} and Bogard, {Patrick J.} and Lynch, {Jane F.}",
note = "Funding Information: acancers ininthetheSBLAdirect Whenis parent geneticlineage unaffected,progeny 25%,becomesbutwhentheanywemustriskoftothehischildreninisa sibshipaffected, thenreassessa50%risktoeachofhis/ hersiblings.Thisisofmajorimpor¬ Itisthetanceforgeneticcounselling. to physician'sresponsibility provideempatheticandaccurateinfor¬ mationtomembersoffamilieswiththe SBLAsyndromeaswellasanyofthe morethan100mendelianinherited cancer-predisposingdiseases(more than50fulfillthecriteriaforcancer- associated genodermatoses).7,8 This can only beaccomplishedbymeticu¬ lousfamilystudy.Asaminimalpre¬ caution,werecommendextendingthe pedigreeandmaternaltoincludesiblings,parents, paternalgrandparents, andauntsanduncles(modifiednuclear pedigree).Becauseofthegenerally laterageattheonsetofcancer,older adultswill usually be more informative of thethantheprogeny,nephews,inandnieces proband.However, syn¬ dromeswherebothchildhood-and adult-onsetcancersarecharacteristic (asintheSBLA),onemustbekeenly awareofthenatural history and tumor patterntocapitalizeonthepotential significance of childhood cancer occur¬ renceforthefacilitationof hereditary cancersyndromeidentification. ThisstudywassupportedinpartbytheNe¬ braskaDivision-FraternalOrderofEaglesand grant 1297-AR2 from the Council for Tobacco Research,USA,Inc. References 1. LynchHT:Hereditaryfactorsinchildhood cancer.Paediatrician 1982;11:205-221. 2. DraperGJ,HeafMM,Kinnier-WilsonLM: Occurrenceofchildhoodcancersamongsibsand estimationoffamilialrisk.JMedGenet1977;14: 81-90. 3. BirchJM,HartleyAL,MarsdenHB,etal: Excessriskofbreastcancerinthemothersof childrenwithsofttissuesarcomas.BrJCancer 1984;49:325-331. 4. LynchHT,MulcahyGM,HarrisRE,etal: Geneticandpathologicfindingsinakindredwith hereditarysarcoma,breastcancer,braintumors, leukemia,lung,laryngeal,andadrenalcortical carcinoma.Cancer1978;14:2055-2064. 5. LiFP,FraumeniJF:Softtissuesarcomas, breastcancer,andotherneoplasms:afamilial syndrome?AnnInternMed1969;71:747-752. 6.LiFP,FraumeniJF:Prospectivestudy ofafamilycancersyndrome.JAMA1982;247: 2692-2694. 7.LynchHT,FusaroRM:Cancer-Associated Genodermatoses.NewYork,VanNostrandRein-holdCo,1982. 8.LynchHT:CancerGenetics. CharlesC ThomasPublisher,Springfield,Ill,1976.",
year = "1985",
month = feb,
doi = "10.1001/archpedi.1985.02140040032020",
language = "English (US)",
volume = "139",
pages = "134--136",
journal = "JAMA Pediatrics",
issn = "2168-6203",
publisher = "American Medical Association",
number = "2",
}