A cystic non-Wilms renal tumor in a Nepalese boy: A rare case of clear cell sarcoma.
Publication/Presentation Date
8-1-2023
Abstract
INTRODUCTION: Clear cell sarcoma of the kidney (CCSK) is an uncommon childhood malignancy known for its aggressive behavior and tendency to recur and metastasize to the brain and bones.
CASE PRESENTATION: We report a four-year-old boy evaluated for non-specific abdominal pain with an incidental radiological finding of a right lower pole cystic renal mass initially thought to be cystic Wilms' tumor. A pretherapy core biopsy of the mass suggested a clear cell tumor. An open transperitoneal radical nephroureterectomy with aortocaval lymph node sampling was done. Based on histopathological findings and immunohistochemical analysis, CCSK was diagnosed. Appropriate chemotherapy and radiotherapy were instituted postoperatively. At six years follow-up, he was tumor-free and doing well.
CLINICAL DISCUSSION: The workup for CCSK includes diagnostic and metastatic imaging, histopathology, and immunohistochemistry analysis. Diagnostic segregation of CCSK and Wilms' tumor is imperative to institute optimal oncological management and improve overall treatment outcomes.
CONCLUSION: Considering the age of presentation and clinico-radiological appearance, CCSK can be mistaken for Wilms' tumor, which is much more common in occurrence despite the distinguishable histopathological features, treatment modalities, and prognosis.
Volume
109
First Page
108582
Last Page
108582
ISSN
2210-2612
Published In/Presented At
Shrestha, A. L., Shrestha, P., Mishra, A., & Pandit, A. (2023). A cystic non-Wilms renal tumor in a Nepalese boy: A rare case of clear cell sarcoma. International journal of surgery case reports, 109, 108582. https://doi.org/10.1016/j.ijscr.2023.108582
Disciplines
Medicine and Health Sciences
PubMedID
37517259
Department(s)
Department of Medicine Fellows and Residents, Fellows and Residents
Document Type
Article