Clear cell sarcoma of kidney (CCSK)--a clinicopathological study of 4 cases.

The clinical and histopathological features of four cases of clear cell sarcoma of kidney (CCSK) or Bone metastasising renal tumour of childhood (BMRTC) are presented. These cases were identified among 107 primary renal tumours in childhood over a period of 15 years (1973-1987) in the Trivandrum Medical College. Of the 107 cases 96 (89.7 percent) were nephroblastomas and 7 (6.6 percent) were Mesoblastic Nephromas. The incidence of Clear cell sarcoma was 3.7 percent. Abdominal mass and haematuria were the most common clinical features. All the four cases occurred in male children with no predilection for the right or the left kidney. At the time of presentation bone metastasis was not present in any of the four cases. Metastasis to scapula and skull was detected ten months after nephrectomy in one case. Of the four patients three were in stage I disease at the time of diagnosis. All the four cases showed the typical gross morphology and the classic microscopic pattern of Clear Cell sarcoma kidney. The treatment was similar in all the four cases with Surgery followed by radiotherapy and chemotherapy (Vincristine, Adriamycin Actinomycin D and cyclophosphamide). Only one of the four patients is alive and well 12 months after surgery. The literature is reviewed along with a discussion of the gross pathology, histology and histogenesis of clear cell sarcoma of kidney.