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. 2017 Oct-Dec;38(4):447-451.
doi: 10.4103/ijmpo.ijmpo_188_16.

Sinusoidal Obstruction Syndrome during Treatment for Wilms' Tumor: A Life-threatening Complication

Sidharth Totadri  1 , Amita Trehan  1 , Deepak Bansal  1 , Richa Jain  1
Affiliations

Sinusoidal Obstruction Syndrome during Treatment for Wilms' Tumor: A Life-threatening Complication

Sidharth Totadri et al. Indian J Med Paediatr Oncol. 2017 Oct-Dec.

Abstract

Context: Survival rates exceed 90% in Wilms' tumor (WT). Actinomycin-D (ACT-D) which is indispensable in the management of WT is associated with the development of sinusoidal obstruction syndrome (SOS), a potentially fatal complication.

Aims: The aim is to study the presentation, management, and outcome of SOS complicating ACT-D administration in WT.

Settings and design: Retrospective file review conducted in a Pediatric Hematology-Oncology unit.

Materials and methods: Patients diagnosed and treated for WT from January 2012 to December 2015 were analyzed. SOS was diagnosed clinically, based on McDonalds criteria, requiring two of the following: jaundice, hepatomegaly and/or right upper quadrant pain, weight gain with or without ascites.

Results: Of 104 patients treated, SOS occurred in 5 (4.8%). Age: 6 months to 5 years, 3 were girls. Tumor involved left kidney in 3, right in 1 and a horseshoe kidney in 1. Histopathology was consistent with WT in 4 and clear cell sarcoma kidney in 1. One had pulmonary metastases. Three developed SOS preoperatively and two during adjuvant chemotherapy. None received radiotherapy. Clinical manifestations comprised of jaundice, hepatomegaly, ascites/weight gain, respiratory distress, hypotension, and encephalopathy. Laboratory findings included thrombocytopenia, elevated serum transaminases, and coagulopathy. Treatment included fluid restriction, broad spectrum antibiotics, and transfusional support. Two children received N-acetyl cysteine infusion. Defibrotide was administered to two patients. Four recovered and one succumbed to multi-organ failure. Two patients were safely re-challenged with 50% doses of ACT-D.

Conclusions: SOS is a clinical diagnosis. Systematic supportive care can enable complete recovery. Under close monitoring, re-challenge of ACT-D can be performed in gradually escalating doses.

Keywords: Developing country; liver failure; portal hypertension; renal malignancy; veno-occlusive disease.

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Conflict of interest statement

There are no conflicts of interest.

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