Management and outcome of pediatric Wilms tumor with malignant inferior Vena cava thrombus: largest cohort of single-center experience.

Abstract

BACKGROUND:

Wilms tumor (WT) with an inferior Vena cava (IVC) malignant thrombus comprises 4-10% of all WT cases.

METHODS:

This retrospective analysis included 51 pediatric patients presenting at Children Cancer Hospital Egypt-57357 from July 2007 to December 2016 with the diagnosis of WT with malignant IVC thrombus.

RESULTS:

Median age at presentation = 4.4 years and 28 cases (55%) were females. Twenty-five patients (49%) were metastatic and 4 patients (7.8%) had bilateral disease. Forty-seven cases (92.2%) had favorable histology with no evidence of anaplasia. Level of thrombus extension at presentation was classified as infra-hepatic, retro-hepatic, supra-hepatic and intra-cardiac in 33, 9, 6 and 3 patients, respectively. Fifty patients started neoadjuvant chemotherapy (CTH) with 16 patients showing complete resolution of thrombus after 6 weeks of CTH. None of the patients developed thrombus progression after neoadjuvant CTH; one patient had stationary intra-cardiac thrombus, while remaining patients showed partial regression of their thrombus and had nephrectomy with en-bloc thrombectomy. The mean cranio-caudal dimension of IVC thrombi at initial presentation was 6.5 cm, and 3.6 cm post 6th week of CTH. The 5-year OS and EFS were 75.9% and 71.1%, respectively. There was no significant correlation of initial levels of thrombus extension with survival.

CONCLUSION:

Neoadjuvant chemotherapy followed by radical nephrectomy with en-bloc thrombectomy and radiotherapy seems a successful approach for management of patients with WT and IVC tumor thrombus. Measurement of the cranio-caudal dimension of thrombus and its response to treatment should be considered in the surgical planning.

• Authors: Moatasem Elayadi, Mahmoud Hammad, Kareem Sallam, Gehad Ahmed, Soha Ahmed, Ahmed Ibrahim, Amal Refaat, Naglaa Elkinaai, Alaa Younes, Norbert Graf, Wael Zekri
• Journal: International journal of clinical oncology (Int J Clin Oncol) Vol. 25 Issue 7 Pg. 1425-1431 (Jul 2020) ISSN: 1437-7772 [Electronic] Japan
• PMID: 32249334 (Publication Type: Journal Article)

Topics

• Child
• Child, Preschool
• Cohort Studies
• Female
• Humans
• Kidney Neoplasms (pathology, surgery, therapy)
• Male
• Neoadjuvant Therapy
• Nephrectomy (methods)
• Retrospective Studies
• Thrombectomy (methods)
• Thrombosis (pathology)
• Treatment Outcome
• Vena Cava, Inferior (pathology)
• Venous Thrombosis (pathology)
• Wilms Tumor (pathology, surgery, therapy)