Pediatric clear cell sarcoma of the kidney with cavoatrial thrombus

Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, India.
Journal of Pediatric Surgery (Impact Factor: 1.39). 12/2011; 46(12):2387-90. DOI: 10.1016/j.jpedsurg.2011.09.050
Source: PubMed


Clear cell sarcoma of the kidney (CCSK) is a rare renal tumor. Only 4 cases of CCSK with vascular thrombus have been reported, and 2 of these were pediatric cases. One of the children had an intraatrial thrombus as well. We describe a 3-year-old boy who was diagnosed as having a Wilms tumor but did not respond to preresection chemotherapy. He underwent complete resection of the tumor under cardiopulmonary bypass. Histologic examination indicated that the tumor was a CCSK. The patient was then managed with appropriate chemotherapy and radiation therapy and is well 16 months after diagnosis.

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    ABSTRACT: Clear cell sarcoma of the kidney (CCSK) is a rare renal tumour that is observed most often in children under 3years of age. Only a few large series of CCSK have been reported and patients with CCSK are often included among patients with other types of childhood renal tumours. The purpose of this paper is to review the published series and case reports of CCSK and to create an up-to-date overview of clinical and histological features, genetics, treatment, and outcome.
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    ABSTRACT: Clear cell sarcoma of the kidney (CCSK) is an uncommon neoplasm that accounts for almost 3 % of pediatric renal tumors. Cavoatrial tumor thrombosis is very rare and because of poor response to chemotherapy, invasive surgical interventions such as open heart surgery may be indicated. A 6-year-old girl with CCSK of right kidney was treated with neoadjuvant chemotherapy. According to poor chemosensitivity, surgical intervention was planned. Right atriotomy was done, but intra-atrial part of tumor was very firm and unsuctionable; so the procedure was completed by laparotomy and en bloc resection of tumoral kidney and its cavoatrial extension through a limited venotomy on inferior vena cava. Although radical resection of CCSK with intracaval involvement should be considered as a multidisciplinary approach and intensive care and supports should be provided, atriocaval tumor in growth in CCSK is firm, non friable and non-adherent, and tumor en bloc resection may be possible through a limited venotomy via primary abdominal approach.
    No preview · Article · Aug 2012 · Pediatric Surgery International