Pediatric clear cell sarcoma of the kidney with atriocaval thrombus.

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.

journal_name

Pediatr Surg Int

authors

Hiradfar M,Zabolinejad N,Shojaeian R,Saeedi P,Joodi M,Nazarzadeh R,Farhangi H,Abbasi Tashnizi M,Alamdaran SA

doi

10.1007/s00383-012-3160-0

subject

Has Abstract

pub_date

2012-11-01 00:00:00

pages

1141-5

issue

11

eissn

0179-0358

issn

1437-9813

journal_volume

28

pub_type

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