Key result
Localized tumor resection followed by chemotherapy and chest radiotherapy resulted in no evidence of tumor recurrence or metastasis at 18 months in a 21-year-old female with esophageal ES/PNET.
Why the study?
Ewing sarcoma and primitive neuroectodermal tumors are rare neuroectodermally derived tumors whose optimal treatment remains a major challenge.
Case Report (n=1)
Answers are grounded in this paper and cite their sources.
A multimodal approach including surgery, chemotherapy, and radiotherapy was effective in treating a rare case of esophageal Ewing sarcoma/primitive neuroectodermal tumor.
Ewing sarcoma and primitive neuroectodermal tumors (ES/PNETs) are rare tumors that belong to a family of round-cell neuroectodermally derived tumors, and their optimal treatment remains a great challenge. This study presented a case of ES/PNET, arising in the esophagus of a 21-year-old female patient presented with progressive dysphagia. Computed tomography and endoscopic ultrasonography showed a well-defined, submucosal solid mass in the superthoracic esophagus. The accurate diagnosis after surgery was obtained through immunohistochemistry and genetic studies, namely the CD99 immunopositivity as well as the EWSR1/FLI1 gene rearrangement associated with t(11;22)(q24;q12) in tumor cells. The patient underwent localized tumor resection followed by chemotherapy and chest radiotherapy. The patient is doing well with no evidence of tumor recurrence or metastasis 18 months after surgery. Although the esophagus is a rare site for ES/pPNET, we can speculate that the treatment protocol of ES/pPNET should include multi-agent chemotherapy, surgery, and local radiotherapy in order to improve the prognosis based on our report.
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Li et al. (2022) conducted a case report in Ewing sarcoma and primitive neuroectodermal tumor (ES/PNET) of the thoracic esophagus (n=1). Localized tumor resection followed by chemotherapy and chest radiotherapy was evaluated on Tumor recurrence or metastasis. Localized tumor resection followed by chemotherapy and chest radiotherapy resulted in no evidence of tumor recurrence or metastasis at 18 months in a 21-year-old female with esophageal ES/PNET.
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