Hepatoblastoma With Tumor Extension Through the Inferior Vena Cava Into the Right Atrium

Huang, Yen-Lin MD*; Shih, Shin-Lin MD; Liu, Hsi-Che MD; Yeh, Ting-Chi MD

Journal of Pediatric Gastroenterology & Nutrition: June 2010 - Volume 50 - Issue 6 - p 577
doi: 10.1097/MPG.0b013e3181dcd277
Image of the Month

*Department of Radiology, Taiwan

Department of Pediatrics, Mackay Memorial Hospital, Taiwan

Department of Radiology, Taipei Medical University, Taipei, Taiwan.

Received 12 February, 2010

Accepted 5 March, 2010

Address correspondence and reprint requests to Shin-Lin Shih, MD, Department of Radiology, Taipei Medical University, 4F, No. 92, Zhong-Shan North Road, 104 Taipei, Taiwan (e-mail: b88401125@ntu.edu.tw).

The authors report no conflicts of interest.

Submissions for the Image of the Month should include high-quality TIF endoscopic images of unusual or informative findings. In addition, 1 or 2 other associated photographs, such as radiological or pathological images, can be submitted. A brief description of no more than 200 words should accompany the images. Submissions are to be made online at http://www.jpgn.org, and will undergo peer review by members of the NASPGHAN Endoscopy and Procedures Committee, as well as by the Journal.

Article Outline

A 4-year-old boy presented with abdominal fullness and edema of the lower legs for 1 month. Abdominal echography revealed a large hepatic tumor with ascites. Computed tomography showed a 10-cm × 8.5-cm enhancing mass in the right lobe of liver with extension through the inferior vena cava (IVC) into the right atrium (RA) and an abnormally engorged azygos vein (Figs. 1 and 2). Three metastatic lung nodules were noted (not shown). The patient's α-fetoprotein level was 950400.00 ng/mL (<10 ng/mL). Pathological findings after surgical biopsy confirmed the diagnosis of hepatoblastoma.

Secondary cardiac tumors in children that spread via IVC into RA are extremely rare. Wilms tumor, adrenal neuroblastoma, hepatocellular carcinoma, and hepatoblastoma were the tumor subtypes reported (1,2). Patients of malignant hepatic tumors with IVC and RA tumor thrombus had poor prognosis and a significant incidence (100%) of lung metastasis (2), which was also observed in our case.

Hepatoblastoma with IVC and RA extension makes the treatment approach more complex. Some authors suggest concomitant hepatic and cardiac surgery, whereas others suggest chemotherapy or coordinated use of both (3,4). Our patient was treated with chemotherapy for 2 months (cisplatin 90 mg/m2 + epirubicin 25 mg·m−2·day−1 for 3 days, then shifted to vincristine 1.5 mg·m−2·day−1 + 5-FU 300 mg·m−2·day−1) with initial response of mild shrinkage of the main tumor and the RA mass. Unfortunately, the boy died due to fungal meningitis.

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REFERENCES

1. Huh J, Noh CI, Kim YW, et al. Secondary cardiac tumors. Pediatr Cardiol 1999; 20:400–403.
2. Wang JN, Chen JS, Chuang HY, et al. Invasion of the cardiovascular system in childhood malignant hepatic tumors. J Pediatr Hematol Oncol 2002; 24:436–439.
3. Kesik V, Yozgat Y, Sari E, et al. Hepatoblastoma metastatic to the right atrium responding to chemotherapy alone. Pediatr Hematol Oncol 2009; 26:583–588.
4. Murakami T, Myojin K, Matano J, et al. Resection of hepatoblastoma with right atrial extension using cardiopulmonary bypass. J Cardiovasc Surg (Torino) 1995; 36:455–457.
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