Abstract
To the Editor:Theprevalenceofneoplasmsinchildrenunder15years of age is low (14.1 per 100,000 individuals)[Harras, 1996]. However, overgrowth syndromes(OGS) have an increased frequency of tumors[Lapunzina, 2005a].Sotos syndrome (SoS) is an OGS caused by NSD1mutations. Manifestations include dolichocephaly,marked frontal bossing, frontoparietal balding,narrow temples, prominent mandible, acceleratedgrowth, dilatation of cerebral ventricles, delay inexpressive language and motor development, andnon-progressive neurological dysfunction mani-fested by unusual clumsiness.The risk of tumors in SoS patients has beenestimated to be about 2–3% [Cohen, 1999; Lapun-zina, 2005a]. At least 22 cases of malignant tumorshave been reported in this disorder [Lapunzina,2005b]. Also, three additional patients are known: agirl with retinoblastoma (personal observation) andtwo patients with leukemia/lymphoma (A. Fryer,personal communication, A. Barnicoat, personalcommunication).The overall male-to-female ratio in SoS patientswithcanceris2:1,whichissimilartothatobservedinthe general population of children. The mostfrequent tumor types are leukemia/lymphoma (11/25;44%ofSoScases).Accordingtodatastratifiedbyage and sex from the Surveillance, Epidemiology,and End Results Program [SEER, 2005], the overallprevalence of leukemia in the general populationyoungerthan20yearsofageisslightlyhigheramongmales than females (M:F¼1.2:1) and overall malepredominance for lymphoma in children youngerthan 15 years of age is the same (M:F¼2.1:1).However, among patients with SoS, 10 of 11leukemia/lymphoma cases have been observed inmales (M:F¼10:1) (Table I), a five- to eightfoldincrease from the expected frequency.Although these data are based on a small samplesize, a link between male gender and leukemia/lymphomainSoSseemsapparent.Becausethismaleshifttoleukemia/lymphomaisnotwellunderstood,reporting of all cases of SoS with neoplasms inthe future may help elucidate the cause of thisassociation.ACKNOWLEDGMENTSWeareindebtedtoProf.Dr.NazneenRahmanandDr.RichardScottforallowingaccesstotheirdataandto Angela Barnicoat, Alan Fryer, and Maurizio Aricofor providing data on their patients.REFERENCES
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