Zhang Deying
Children's Hospital of Chongqing Medical University, P.R.China
Title: Diagnosis and treatment of CCSK in children: 12-years experience with 31 cases
Biography
Biography: Zhang Deying
Abstract
Objective: To summarize the experience of diagnosis and treatment of clear small sarcoma of the kidney (CCSK) in children and analyze the prognostic factors.
METHODS: A total of 31 cases of pathological diagnosis of renal clear cell sarcoma were collected from March 2006 to March 2018 in Department of Urology, Children's Hospital of Chongqing Medical University. All cases were diagnosed by pathology, imaging, etc. Preoperative or/and postoperative chemotherapy, selective radiotherapy and other comprehensive treatment measures were adopted. Retrospective study of clinical features, long-term prognosis and prognosis-related factors.
Results: From March 2006 to March 2018, a total of 308 renal tumors were collected from Department of Urology, Children’s Hospital of Chongqing Medical University. Among them, 31 cases (10%) of CCSK after operation were included in this report. There were 22 males and 9 females (male: female = 2.44:1). The age of onset was 2.6 (0.25 to 11.5) years. There were 22 cases on the left side and 9 cases on the right side. 10 cases were stage I, 7 cases were stageII, 10 cases were stage III, and 5 caseswere stage IVat first diagnosis. There were 11 cases with "abdominal mass" as the main complaint, 8 cases with "macrohematuria", 3 cases with "abdominal pain", 3 cases with "physical examination found mass", and 6 casesrecievedchemotherapy before operation, all patients underwent surgical resection of the tumor and affected side kidney, the tumor weight were between 400-3500g, diameter were between 6-17cm. Until March 2018, the follow-up time was 53.3 (5.9 to 142.3) months. 4 cases were reoperated after reccurence. As of the last follow-up, 5 patients died, 3 were lost follow-up or abandoned treatment. The common metastatic sites were lung, liver, and vertebral bodies. Older children, distant lung metastases, and bilateral metastases have a poor prognosis. The longest recurrence interval is 6 years and 5 months.
Conclusions: In children with CCSK, there can be 70% or more of long-term EFS with a combination of surgery and radiotherapy and chemotherapy. The standardized comprehensive treatment model based on pathology and clinical stage will help improve the survival rate of CCSK.
Key words: Renal clear cell sarcoma, comprehensive treatment, survival rate