Adult Wilm’s tumor (Nephroblastoma), with pulmonary and liver metastasis: Case report of a rare tumor and a brief review of the literature

Wilms' tumor (WT) (nephroblastoma) is a childhood renal neoplasm encountered rarely in adults. Although radiological imaging is an excellent tool for diagnosis in most cases, histomorphologic diagnosis can be challenging, especially with limited sampling, which may result in the potential absence of one of the three histological components. In addition, the rare occurrence in adults makes it less likely to be included in the differential diagnosis of an adult presenting with a renal mass. We report a case of a 21-year-old woman who presented with a left abdominal palpable mass, eventually diagnosed with an adult Wilms tumor (AWT). Following the Children's Oncology Group (COG) protocol, the patient underwent total left nephrectomy. The histological features were classic triphasic, with blastemal (40%), epithelial (45%), and stromal (15%) components, and occasional foci of anaplasia, which were not sufficient to classify it as an anaplastic subtype. The patient had adjuvant chemotherapy with Dactinomycin and Doxorubicin and initially responded well to treatment. 

Nine months after nephrectomy, pulmonary and liver metastases were found. Surgical treatment was not possible due to the high tumor burden in the lung and the liver. Chemotherapy, including Vincristine, Dactinomycin, and Doxorubicin, was initiated, resulting in a favorable clinical response. The patient was disease-free at the last follow-up, 26 months after treatment. This case highlights the diagnostic and therapeutic challenges associated with AWTs. We provide a brief review of the literature, including history, clinical presentation, diagnosis, and treatment. We also discuss the potential benefits of diagnosing and treating AWTs with current technological advancements.