Teratoid Wilms Tumor with Tumor Embolus in Inferior Vena Cava and Right Atrium: Experience from Single Tertiary Hospital

Shihao Huang, Yeming Wu and Xiaojun Yuan

Department of Pediatric Hematology/Oncology, Xinhua Hospital, Shanghai Jiao Tong University School of Medicine, China
Department of Pediatric Surgery, Xinhua Hospital, Shanghai Jiao Tong University School of Medicine, China

^*Correspondance to: Xiaojun Yuan 

 PDF  Full Text DOI: 10.25107/2474-1647.2647

Abstract

Teratoid Wilms Tumor (TWT) with tumor embolus in inferior vena cava and right atrium is extremely rare. These patients have risks of cardiac arrest and need emergency treatment after diagnosis. However, pathologic diagnosis may be difficult at the initial diagnosis due to the limited tumor tissue by needle biopsy and uncommon unique pathological characteristics in TWT. This makes it hard to decide the appropriate initial treatment. We reported a three-year-old boy presented with abdominal pain and a huge mass with tumor embolus in inferior vena cava and right atrium. Pathologic diagnosis by needle biopsy for this patient was uncertain because the tumor tissue was composed of immature rhabdomyomatous elements but free of stromal, blastemal, and epithelial elements. After discussion by multidisciplinary team, two courses of chemotherapy were given according to clinically diagnosed Wilms tumor. But the tumor enlarged. Then surgical operation was immediately performed by pediatric surgeon combined with cardiothoracic surgeon. Both the primary tumor (include left kidney) and the tumor embolus were completely resected. The final pathology revealed the diagnosis as TWT. Further eight cycles of chemotherapy and local postoperative radiotherapy were given. The child has been followed up for 27 months and still with disease-free survival. The cooperation of multidisciplinary team during the whole diagnosis and treatment was an important factor for the long-term survival of this case.

Keywords

Teratoid; Wilms tumor; Tumor embolus; Multidisciplinary team

Cite the article

Huang S, Wu Y, Yuan X. Teratoid Wilms Tumor with Tumor Embolus in Inferior Vena Cava and Right Atrium: Experience from Single Tertiary Hospital. Clin Surg. 2019; 4: 2647..