Case Report: First report of a Wilms tumor in an individual with Dias-Logan Syndrome (BCL11A-related intellectual disability)

Alexandre Guilherme Troullioud Lucas^1*Elise Fiala¹Ahmed Razeq²Talia Sauerhaft¹Anita P Price¹Juan Miguel Mosquera³Jeremy Miyauchi⁴Ming Gao¹Michael F. Walsh⁵Michael Vincent Ortiz^1*

• ¹Memorial Sloan Kettering Cancer Center, New York, United States
• ²Children's Hospital of San Antonio, San Antonio, Texas, United States
• ³Weill Cornell Medical Center, NewYork-Presbyterian, New York City, New York, United States
• ⁴Northern Westchester Hospital, Mount Kisco, New York, United States
• ⁵Regeneron Pharmaceuticals, Inc., Tarrytown, New York, United States

Dias-Logan Syndrome (DLS) is a rare condition caused by heterozygous germline BCL11A pathogenic variants associated with global developmental delay, distinctive facial features, and asymptomatic persistence of fetal hemoglobin. There has been no evidence of an association between DLS and increased risk of cancer. We report the first instance of a child with DLS diagnosed with cancer, a Wilms tumor (WT), notably much older than typical onset. Although this case alone is insufficient to warrant routine WT screening in DLS, given the extreme rarity, we cannot rule out an association with DLS and WT predisposition.