Shrestha Ashish Lal, Shrestha Prinsa, Mishra Aakash, Pandit Anjali
Department of Pediatric and Neonatal Surgery, Kathmandu Medical College Teaching Hospital, Kathmandu, Nepal.
Kathmandu Medical College Teaching Hospital, Kathmandu, Nepal.
Int J Surg Case Rep. 2023 Aug;109:108582. doi: 10.1016/j.ijscr.2023.108582. Epub 2023 Jul 28.
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.
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.
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.
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.
肾透明细胞肉瘤（CCSK）是一种罕见的儿童恶性肿瘤，以其侵袭性、复发倾向以及转移至脑和骨骼的趋势而闻名。
我们报告一名4岁男孩，因非特异性腹痛接受评估，偶然通过影像学检查发现右肾下极有一个囊性肿块，最初被认为是囊性威尔姆斯瘤。对该肿块进行的治疗前核心活检提示为透明细胞瘤。实施了开放性经腹根治性肾输尿管切除术并进行主动脉腔静脉淋巴结采样。根据组织病理学发现和免疫组化分析，诊断为CCSK。术后进行了适当的化疗和放疗。随访6年时，他无肿瘤且情况良好。
CCSK的检查包括诊断性和转移性影像学检查、组织病理学以及免疫组化分析。区分CCSK和威尔姆斯瘤对于制定最佳肿瘤治疗方案和改善总体治疗结果至关重要。
考虑到发病年龄和临床影像学表现，CCSK可能会被误诊为威尔姆斯瘤，尽管两者在组织病理学特征、治疗方式和预后方面存在明显差异，但威尔姆斯瘤更为常见。
