Brain Infarcts: Description of a New Paraneoplastic Phenomenon in the Setting of Wilms Tumor



We present the case of a 23-month-old male with a one-year history of intermittent abdominal pain who presented for a routine well-child visit and was incidentally noted to have a large abdominal mass and regression of developmental milestones. Upon further investigation, the child was diagnosed with Wilms Tumor (WT) with metastases to the periaortic lymph nodes and bilateral lungs. Initial management included left radical nephrectomy, lymphadenectomy, and mediport placement for chemotherapy infusions. Tumor cytogenetic report was positive for loss of heterozygosity at 11p15 region and pathology demonstrated blastema predominant WT. Treatment decision was based on a therapeutic clinical trial (AREN0534) outlined by the Children’s Oncology Group and the patient was placed on a 28-week chemotherapy regimen, including vincristine, dactinomycin, doxorubicin, cyclophosphamide, carboplatin, and etoposide in conjunction with radiation. MRI of the head showed no evidence of metastasis, however it demonstrated multiple areas of infarction in the distribution of the posterior cerebral artery (PCA). Coagulation studies were performed to evaluate possible underlying causes and the patient was found to be heterozygous for Factor V Leiden (FVL) mutation. While FVL is associated with an increased risk of venous thromboembolic (VTE) events, arterial thromboembolic events are generally rare. In this report, we explore potential etiologies for the unique presentation of brain infarction in a pediatric patient with WT.

Keywords: Wilms Tumor, brain infarction, coagulopathies, thromboses, paraneoplastic phenomenon, metastases

Citation: Kalahasti S, Mariano SB, Burjonrappa S. “Brain Infarcts: Description of a New Paraneoplastic Phenomenon in the Setting of Wilms Tumor”. SVOA Paediatrics 1:1 (2022) Pages 10-14.