Occurrence of renal cell carcinoma and hematologic malignancies (predominantly lymphoid) in individuals and in families - Beyond the Abstract
The most limited estimates, with the smallest number of first degree relatives (1-2 sibs, 2 children) showed an incidence of 15.5 - 18.6% with an O/E ratio of 8.85 and 7.74 respectively. In a more extreme estimate with larger numbers of first degree relatives (4 sibs and 4 children), the incidence was 9.3% and the O/E ratio was 4.45. In all of these scenarios, the risk far exceeds the general population lifetime risk of developing NHL or other, more rare, hematologic malignancies.
Additionally, since the probands were the RCC patients, and we noted anticipation among parent child pairs with RCC/HM or HM/RCC, there could be ascertainment bias. Therefore, we evaluated age-of-onset anticipation among the families with multiple HM. Of those with multiple HM, there were 9 in which the age-of-onset was known, among multi-generational subjects. Age-of-onset anticipation was observed in all 9 families. Seven pairs were parent child, and 1 each was uncle/niece and aunt/nephew. The median age of generation one is 71 years, and of generation two is 47 years, with a median anticipation of 24 years.
Thus, in the 10% of RCC patients with family history of HM, age-of-onset anticipation was noted among the first generation RCC/HM pairs and the multiple HM pairs. Additionally, the O/E ratios far exceed the expected association. We anticipate that these observations will lead to additional molecular genetic investigations of such families.
Written By: Janice P. Dutcher, Peter H. Wiernik
Our Lady of Mercy Cancer Center, New York Medical College, 750 Kappock Street, # 511, Bronx, NY, 10463, USA.
Reference:
Dutcher JP, Wiernik PH, Varella L, Chintapatla R: Occurrence of renal cell carcinoma and hematologic malignancies (predominantly lymphoid) in individuals and in families. Familial Cancer, () 1-11; doi 10.1007/s10689-016-9911-7
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