Diffuse Anaplastic Wilms Tumor in a Child With LAMA2-related Muscular Dystrophy

J Pediatr Hematol Oncol. 2022 Jun 23. doi: 10.1097/MPH.0000000000002503. Online ahead of print.


Laminin alpha-2-related muscular dystrophy (LAMA2-MD), caused by mutations in the LAMA2 gene, is inherited in an autosomal recessive manner. There is no known association of LAMA2-MD with cancer predisposition. We present a 4-year-old female with LAMA2-MD and Children's Oncology Group stage III diffuse anaplastic Wilms tumor (DAWT). Given our patient's comorbidities, it was essential to tailor her adjuvant chemotherapy by omitting vincristine and doxorubicin to avoid the potential worsening of her neuromuscular dysfunction and cardiomyopathy. This report illustrates the sporadic occurrence of 2 rare events in our patient and highlights the successful risk-adapted management of DAWT based on the pathophysiology of LAMA2-MD.