Surgical management and outcomes of intracranial metastatic Wilms' tumor in the pediatric population: a case series.

ABSTRACT

PURPOSE: Approximately 1 to 2% of patients with Wilms' tumor (WT), or nephroblastoma, will have metastasis to the brain. Due to the rarity of intracranial metastasis, the clinical characteristics, prognosis, and a standardized treatment approach to this occurrence remain poorly understood. Here we review the surgical management and treatment outcome of WT patients with intracranial metastasis at our institution. METHODS: A retrospective chart review of patients with WT at the Children's Hospital of Philadelphia was performed from 2007 to 2021. Clinical characteristics, operative details, radiographic studies, pathology, and patient outcomes were collected and analyzed. RESULTS: A total of 3 patients with histologically confirmed intracranial metastatic disease from WT were identified with a mean age of 5.7 years (range 3-10 years). 2 of the 3 patients were male. The mean time from diagnosis of primary WT to development of central nervous system metastasis was 15.3 months. Both supratentorial (n = 3) and infratentorial (n = 1) sites of metastasis were observed. Surgical resection was performed, and gross total resection was achieved in all 3 patients. All cases had favorable histology with no anaplasia and received whole-brain irradiation and chemotherapy. Two of 3 patients had a good neurologic function at postoperative follow-up. One patient died from their disease 4 months after resection of the brain metastasis. CONCLUSION: In WT patients with limited systemic disease burden, the combination of surgery, chemotherapy, and radiotherapy may play a role in enhancing survival when intracranial metastasis is present, despite the perioperative risk associated with surgery.