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Review
. 2018 Feb 23;16(1):38.
doi: 10.1186/s12957-018-1343-4.

Adult Wilms tumor with inferior vena cava thrombus and distal deep vein thrombosis - a case report and literature review

Krzysztof Ratajczyk  1 , Adrian Czekaj  2 , Joanna Rogala  3 , Pawel Kowal  2
Affiliations
Review

Adult Wilms tumor with inferior vena cava thrombus and distal deep vein thrombosis - a case report and literature review

Krzysztof Ratajczyk et al. World J Surg Oncol. .

Abstract

Background: Adult Wilms tumor (WT, nephroblastoma) is a rare, but well-described renal neoplasm. Although inferior vena cava tumor thrombosis is present in up to 10% of Wilms tumors in childhood, only few cases of this clinical manifestation in adults have been reported. To the best of our knowledge, this is the first case of adult WT infiltrating into inferior vena cava (IVC) with concomitant distal deep vein thrombosis.

Case presentation: A 28-year-old male patient with gross hematuria and right flank pain was diagnosed with right kidney tumor penetrating to IVC. Preoperatively, acute distal thrombosis in inferior vena cava and lower extremities veins occurred. Right radical nephrectomy with tumor thrombectomy via cavotomy was performed. In order to prevent pulmonary embolism, IVC was ligated below left renal vein level. Histopathological examination revealed a triphasic nephroblastoma without anaplastic features. Postoperatively, patient was diagnosed with metastatic liver disease, which was treated with two lines of chemotherapy followed by radiotherapy with achievement of complete response.

Conclusions: Adult WT occurs usually in young patients, under 40 years of age. Neoadjuvant chemotherapy proved to be effective in children, resulting with tumor shrinkage and venous tumor thrombus regression. Therefore, percutaneous biopsy should be always considered in young patients presenting with renal tumor invading venous system. IVC ligation is a safe treatment option in the event of complete inferior vena cava occlusion due to distal thrombosis concomitant to tumor thrombus, provided collateral venous pathways are well-developed.

Keywords: Adult Wilms tumor; Ligation; Vena cava inferior; Venous thrombosis.

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Written informed consent for publication of the relevant medical information was obtained from the patient. A copy of the consent form is available for review by the Editor of this journal.

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The authors declare that they have no competing interests.

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Figures

Fig. 1
Fig. 1
Abdominal CT scan at the time of diagnosis. Right kidney tumor with inferior vena cava tumor thrombus extending to the hepatic veins’ level. a Coronal plane. b Transverse plane
Fig. 2
Fig. 2
Triphasic pattern of Wilms tumor. a Predominant blastemal cells (H/E, 20×). b Stromal component (H/E, 10×). c Minor epithelioid component (H/E, 20×)
Fig. 3
Fig. 3
Abdominal CT scan at 3 months postoperatively-multiple liver metastases (white arrows)
See this image and copyright information in PMC

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