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Case Reports in Medicine
Volume 2014, Article ID 984369, 6 pages
Case Report

Clear Cell Sarcoma-Like Tumor of the Gastrointestinal Tract, Presenting as a Second Malignancy after Childhood Hepatoblastoma

1Sarcoma Unit, Royal Marsden Hospital, London SW3 6JJ, UK
2Department of Histopathology, The Royal Marsden NHS Foundation Trust, 203 Fulham Road, London SW3 6JJ, UK

Received 11 September 2013; Accepted 29 December 2013; Published 17 February 2014

Academic Editor: Muhammad Wasif Saif

Copyright © 2014 Khin Thway et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


Clear cell sarcoma-like tumor of the gastrointestinal tract (CCSLGT) is a rare malignant neoplasm arising within the wall of the small bowel, stomach, or large bowel, predominantly in children and young adults. It is an aggressive tumor with a high rate of local recurrence, metastases, and early death from disease. Histologically, it is composed of relatively monomorphic ovoid or round cells with clear to eosinophilic cytoplasm, arranged in sheets and sometimes papillary or alveolar architectures, often with CD68-positive osteoclast-like giant cells in variable numbers, and is associated with EWSR1-CREB1 gene fusions. Its pathogenesis is unknown, and histologically it can be easily confused with a variety of intra-abdominal neoplasms. We describe a case of CCSLGT with molecular characterization, presenting as an acutely obstructing small bowel mass in a 33-year-old male, which occurred as a second malignant neoplasm 20 years after treatment with surgery, radiotherapy, and cisplatin and doxorubicin chemotherapy for childhood hepatoblastoma. This gives further insight into the clinical setting of this highly aggressive neoplasm and highlights the use of radiation therapy as a possible etiologic factor.