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Clear cell sarcoma of kidney: a clinicopathological study of eight cases from Malaysia
1Department of Pathology, University Hospital, University of Malaya, Kuala Lumpur.
Abstract:
Eight cases of clear cell sarcoma of kidney were seen in the Department of Pathology, University Hospital, Kuala Lumpur, Malaysia over the 16-year period from 1973 to 1989. Five of the patients were males. Six patients were Malay, one Chinese and one Indian. The patients' ages ranged from 8 months to 3 years. Clear cell sarcoma was the original diagnosis in two patients while six were diagnosed as blastemal-predominant Wilms' tumours at presentation. Metastases developed in five patients. Metastatic sites included the thoracic vertebra, skull, orbit, humerus, radius, ulna, shoulder, lung and liver. The prolonged survival, of 9 years and 9 months, seen in one patient despite omission of Adriamycin (doxorubicin) from the chemotherapeutic protocol is highlighted. We also emphasise the histological factors which are of help in differentiating clear cell sarcoma from Wilms' tumour.
Insights
Clear cell sarcoma of the kidney is a rare childhood cancer. This study highlights its distinct histological features and potential for prolonged survival, even without certain chemotherapy drugs.
Area of Science:
- Pediatric Oncology
- Renal Pathology
- Cancer Histology
Background:
- Clear cell sarcoma of the kidney (CCSK) is a rare and aggressive renal tumor in children.
- Distinguishing CCSK from Wilms' tumor, particularly the blastemal-predominant subtype, is critical for appropriate management and prognosis.
Purpose of the Study:
- To report on a series of pediatric clear cell sarcoma of the kidney cases.
- To identify key histological features differentiating CCSK from Wilms' tumor.
- To discuss prognostic factors and treatment outcomes, including prolonged survival.
Main Methods:
- Retrospective review of eight cases of clear cell sarcoma of the kidney diagnosed between 1973 and 1989.
- Analysis of patient demographics, clinical presentation, treatment protocols, and survival data.
- Histopathological examination to identify distinguishing features between CCSK and Wilms' tumor.
Main Results:
- Eight cases of CCSK were identified over a 16-year period.
- Five patients (62.5%) developed metastases to various sites, including bone and lung.
- One patient achieved long-term survival (9 years, 9 months) despite incomplete chemotherapy, underscoring the importance of histological diagnosis.
Conclusions:
- Clear cell sarcoma of the kidney requires careful histological evaluation to differentiate it from Wilms' tumor.
- Metastatic potential is high, necessitating vigilant monitoring.
- Long-term survival is possible, even with modified treatment protocols, emphasizing the need for accurate diagnosis and potentially tailored therapeutic approaches.