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Clear Cell Sarcoma-Like Tumor of the Gastrointestinal Tract, Presenting as a Second Malignancy after Childhood Hepatoblastoma
Joint Authors
Judson, Ian R.
Thway, Khin
Fisher, Cyril
Source
Issue
Vol. 2014, Issue 2014 (31 Dec. 2014), pp.1-6, 6 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2014-02-17
Country of Publication
Egypt
No. of Pages
6
Main Subjects
Abstract EN
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.
American Psychological Association (APA)
Thway, Khin& Judson, Ian R.& Fisher, Cyril. 2014. Clear Cell Sarcoma-Like Tumor of the Gastrointestinal Tract, Presenting as a Second Malignancy after Childhood Hepatoblastoma. Case Reports in Medicine،Vol. 2014, no. 2014, pp.1-6.
https://search.emarefa.net/detail/BIM-513592
Modern Language Association (MLA)
Thway, Khin…[et al.]. Clear Cell Sarcoma-Like Tumor of the Gastrointestinal Tract, Presenting as a Second Malignancy after Childhood Hepatoblastoma. Case Reports in Medicine No. 2014 (2014), pp.1-6.
https://search.emarefa.net/detail/BIM-513592
American Medical Association (AMA)
Thway, Khin& Judson, Ian R.& Fisher, Cyril. Clear Cell Sarcoma-Like Tumor of the Gastrointestinal Tract, Presenting as a Second Malignancy after Childhood Hepatoblastoma. Case Reports in Medicine. 2014. Vol. 2014, no. 2014, pp.1-6.
https://search.emarefa.net/detail/BIM-513592
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-513592