Grand Rounds

Wilm`s Tumor

Medical Sciences Department, Pediatric Oncall, Mumbai, India

Address for Correspondence: Dr Ira Shah, 1, B Saguna, 271, B St Francis Road, Vile Parle {W}, Mumbai 400056

Clinical Problem :
A 9 month old boy was referred for jaundice. He was diagnosed to have right sided Wilm`s tumor at 6 months of age and underwent right sided nephrectomy. He was subsequently on chemotherapy of Vincristine and daptomycin. But since past 1 month he had jaundice and chemotherapy had to be stopped. His bilirubin was 3.7 mg, dl {direct = 1.6 mg, dl}, SGOT = 88 IU, L, SGPT = 42 IU, L, Total proteins = 7.1 gm, dl, Albumin = 4.2 gm, dl, alkaline phosphatase = 604 IU, L, GGTP = 59 IU, L.

What is the likely cause of jaundice in this child_?

Discussion :
Common causes of jaundice in a patient with Wilm`s tumor are veno-occlusive disease {VOD}, hepatotoxicity due to chemotherapy agents, obstruction of the inferior vena cava {IVC} due to tumor mass and infection related such as Hepatitis B and Hepatitis C.
VOD is a well- known complication in patients undergoing high-dose chemotherapy and bone marrow transplantation. The clinical signs of the disease are hepatomegaly, sudden weight gain with or without ascites, and jaundice. {1} Liver biopsy can confirm the clinical diagnosis of VOD by showing the small intrahepatic venules narrowed by an edematous concentric subendothelial zone containing fragmented red cells, debris, and fibrillar material` surrounding sinusoids are engorged and centrilobular hepatocytes are damaged. These changes lead to intrahepatic hypertension, hepatic enlargement, hyperbilirubinemia, peripheral edema, and ascites. {2} Ultrasound {USG} abdomen with doppler studies show reversal of flow in portal veins.
Hepatotoxicity may be observed during conventional chemotherapy. A syndrome characterized by ascites and hyperbilirubinemia has been reported following the administration of several antineoplastic drugs. These patients usually increased transaminases. {1} Both Vincristine and actinomycin are known to cause severe hepatotoxicity. {3}
In our patient, USG abdomen showed recurrence of tumor and invasion of IVC that was causing the jaundice.

References :
  1. Bisogno G, de Kraker J, Weirich A, Masiero L, Ludwig R, Tournade M-F, Carli M. Veno-occlusive disease of the liver in children treated for Wilms tumor. Downloaded from UvA-DARE, the institutional repository of the University of Amsterdam (UvA). Available at website:, 11245, 2.3285. Accessed on 23rd December 2014
  2. Mc Donald GB: Veno-occlusive disease of the liver following marrow transplantation. Marrow Transpl Rev. 1993; 3:49–56
  3. McVeagh P, Ekert H. Hepatotoxicity of chemotherapy following nephrectomy and radiation therapy for right-sided Wilms tumor. J Pediatr. 1975; 87:627-8

Correct Answers :  yes 31%
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