A Case of Wilms Tumor with a Tumor Thrombus in a Boy with WAGR Syndrome

다국어 초록 (Multilingual Abstract)

Intravascular extension of Wilms tumor (WT) can occur in approximately 4-10% of patients. In general, it does not cause any clinical problems because most of these tumors are small. Although there is no standard treatment currently, preoperative chemotherapy and delayed nephrectomy is generally recommended for children with WT accompanied by tumor thrombus. We report a rare case of WT, aniridia, genitourinary anomalies, and mental retardation (WAGR) syndrome in a boy who also had a huge inferior vena cava thrombus, 7 cm length. The prevalence of bilateral WT and tumor thrombus in WAGR has not been identified. The patient was successfully treated with neoadjuvant chemotherapy to decrease the size of the tumor thrombus with WT and delayed nephrectomy following chemotherapy without any invasive intervention and did not show complications.

참고문헌 (Reference)

1 Lall A, "Wilms’ tumor with intracaval thrombus in the UK Children’s Cancer Study Group UKW3 trial" 41 : 382-387, 2006

2 Davidoff AM, "Wilms’ tumor" 21 : 357-364, 2009

3 Emir S, "Wilms tumor with intravascular tumor thrombus" 3 : 29-33, 2014

4 Fischbach BV, "WAGR syndrome : a clinical review of 54 cases" 116 : 984-988, 2005

5 Aldrink JH, "Update on Wilms tumor" 54 : 390-397, 2019

6 Clericuzio CL, "Recognition and management of childhood cancer syndromes : a systems approach" 89 : 81-90, 1999

7 Muto R, "Prediction by FISH analysis of the occurrence of Wilms tumor in aniridia patients" 108 : 285-289, 2002

8 Shamberger RC, "Intravascular extension of Wilms tumor" 234 : 116-121, 2001

9 Al Diab A, "Inferior vena cava involvement in children with Wilms tumor" 33 : 569-573, 2017

10 Clericuzio CL, "Clinical phenotypes and Wilms tumor" 21 : 182-187, 1993

11 Ayyathurai R, "Bland thrombus association with tumour thrombus in renal cell carcinoma : analysis of surgical significance and role of inferior vena caval interruption" 110 : E449-E455, 2012