Stewart DR, et al. - Researchers studied the combined data from three large cohorts of patients who carry germline pathogenic variation in DICER1, to quantify risk, hazard rates, and the probability of neoplasm incidence accounting for competing risks (“cumulative incidence”) of neoplasms (benign and malignant) and standardized incidence ratios for malignant tumors. For the first time, site-specific neoplasm risk and excess malignancy risk was quantified in 102 (female and male) systematically characterized nonproband DICER1 carriers. They found the development of a neoplasm (females, 4.0%; males, 6.6%) in 5.3% of nonproband DICER1 carriers by age 10 years. Development of a neoplasm was seen in 19.3% of nonprobands by age 50 years (females, 26.5%; males, 10.2%). An increased risk was seen among females vs males after age 10 years. Significant cancer excesses overall, particularly of gynecologic and thyroid cancers, was observed in standardized cancer incidence ratio analysis, which represented 3,344 person-years of observation.