Abstract
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.
Highlights
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
We describe a case arising in the small bowel wall and harboring EWSR1-CREB1 gene fusion by reverse transcription polymerase chain reaction, which occurred in a 33-year-old male who had previously had surgery, irradiation, and chemotherapy for childhood hepatoblastoma
We describe a case of clear cell sarcoma-like tumor of the gastrointestinal tract, arising in the small bowel of an adult male, which harbored EWSR1-CREB1 gene fusion and occurred 20 years after chemotherapy, resection, and radiation treatment for childhood hepatoblastoma
Summary
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 associated with high rates of local recurrence, metastasis, and early deaths from disease. We describe a case arising in the small bowel wall and harboring EWSR1-CREB1 gene fusion by reverse transcription polymerase chain reaction, which occurred in a 33-year-old male who had previously had surgery, irradiation, and chemotherapy for childhood hepatoblastoma. This expands the clinical spectrum of this highly aggressive neoplasm and highlights the possibility of radiation therapy as a predisposing cause
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