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CC BY-NC-ND 4.0 · Indian J Med Paediatr Oncol 2017; 38(04): 447-451
DOI: 10.4103/ijmpo.ijmpo_188_16
Original Article

Sinusoidal Obstruction Syndrome during Treatment for Wilms’ Tumor: A Life‑threatening Complication

Sidharth Totadri
Department of Pediatrics, Advanced Pediatrics Center, Pediatric Hematology-Oncology Unit, Postgraduate Institute of Medical Education and Research, Chandigarh, India
,
Amita Trehan
Department of Pediatrics, Advanced Pediatrics Center, Pediatric Hematology-Oncology Unit, Postgraduate Institute of Medical Education and Research, Chandigarh, India
,
Deepak Bansal
Department of Pediatrics, Advanced Pediatrics Center, Pediatric Hematology-Oncology Unit, Postgraduate Institute of Medical Education and Research, Chandigarh, India
,
Richa Jain
Department of Pediatrics, Advanced Pediatrics Center, Pediatric Hematology-Oncology Unit, Postgraduate Institute of Medical Education and Research, Chandigarh, India
› Author Affiliations Financial support and sponsorship Nil.
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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 fatalcomplication. Aims: The aim is to study the presentation, management, and outcome of SOScomplicating 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 manifestationscomprised 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 enablecomplete 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


Publication History

Article published online:
04 July 2021

© 2017. Indian Society of Medical and Paediatric Oncology. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used forcommercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/.)

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