Teratoid Wilms is a very rare histopathological variant of Wilms tumors that is characterized by the predominance of heterologous components, and is described by many to be a nonaggressive, nonmetastatic tumor with a favorable prognosis.
We report a case of a 4-year-old boy with a rare, aggressive metastatic variant of teratoid Wilms tumor. The boy presented with abdominal pain and a palpable abdominal mass. The computed tomography scan demonstrated a large, cystic, multiloculated left renal mass and a single left pulmonary metastasis. The patient had a 5-week course of neoadjuvant chemotherapy. As a result, the size of the metastatic lesion decreased, but there was no change in the size of the renal mass. Subsequently, a left radical nephrectomy and left pulmonary metastectomy were performed where the pathology report showed metastatic teratoid Wilms tumor. The patient received adjuvant chemotherapy and radiotherapy. One year following the initial surgery, the child developed bilateral pulmonary metastases where he is currently having an aggressive regimen of chemotherapy. Although teratoid Wilms has been described as a nonaggressive tumor with a favorable prognosis, it can present with more aggressive forms, with a tendency for metastasis.
KEYWORDS: Wilms tumor; Teratoid Wilms; Rare Wilms
CORRESPONDENCE: Jameel Hisham Bardesi, King Abdulaziz University, Jeddah 21589, Saudi Arabia ( ).
CITATION: UroToday Int J. 2012 Feb;5(1):art 90.
doi: http://dx.doi.org/10.3834/uij.1944-5784.2012.02.08