Ewing Sarcoma and Primitive Neuroectodermal Tumor of the Thoracic Esophagus: Case Report and Comprehensive Literature Review
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 patien...
| Main Authors: | , , , , , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Karger Publishers
2022-03-01
|
| Series: | Case Reports in Oncology |
| Subjects: | |
| Online Access: | https://www.karger.com/Article/FullText/522152 |
| _version_ | 1818273807635316736 |
|---|---|
| author | Jie Li Pengfei Sun Li Ma Xianhua Min Binqiang Ye Yao Zhang Weiwei Ta Jiyun Deng Xiangrong Cao Chi Dong |
| author_facet | Jie Li Pengfei Sun Li Ma Xianhua Min Binqiang Ye Yao Zhang Weiwei Ta Jiyun Deng Xiangrong Cao Chi Dong |
| author_sort | Jie Li |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-12-12T22:03:50Z |
| format | Article |
| id | doaj.art-359d076a7f984ac6a4363e1eb0868b2f |
| institution | Directory Open Access Journal |
| issn | 1662-6575 |
| language | English |
| last_indexed | 2024-12-12T22:03:50Z |
| publishDate | 2022-03-01 |
| publisher | Karger Publishers |
| record_format | Article |
| series | Case Reports in Oncology |
| spelling | doaj.art-359d076a7f984ac6a4363e1eb0868b2f2022-12-22T00:10:26ZengKarger PublishersCase Reports in Oncology1662-65752022-03-0115126727610.1159/000522152522152Ewing Sarcoma and Primitive Neuroectodermal Tumor of the Thoracic Esophagus: Case Report and Comprehensive Literature ReviewJie Li0https://orcid.org/0000-0002-9299-4385Pengfei Sun1https://orcid.org/0000-0001-6864-9584Li Ma2Xianhua Min3Binqiang Ye4Yao Zhang5Weiwei Ta6Jiyun Deng7Xiangrong Cao8Chi Dong9Department of Radiotherapy, Second Hospital Affiliated to Lanzhou University, Lanzhou, ChinaDepartment of Radiotherapy, Second Hospital Affiliated to Lanzhou University, Lanzhou, ChinaDepartment of Radiotherapy, Second Hospital Affiliated to Lanzhou University, Lanzhou, ChinaDepartment of Radiotherapy, Second Hospital Affiliated to Lanzhou University, Lanzhou, ChinaDepartment of Radiotherapy, Second Hospital Affiliated to Lanzhou University, Lanzhou, ChinaDepartment of Radiotherapy, Second Hospital Affiliated to Lanzhou University, Lanzhou, ChinaDepartment of Radiotherapy, Second Hospital Affiliated to Lanzhou University, Lanzhou, ChinaDepartment of Radiotherapy, Second Hospital Affiliated to Lanzhou University, Lanzhou, ChinaDepartment of Radiotherapy, Second Hospital Affiliated to Lanzhou University, Lanzhou, ChinaDepartment of Pathology, Second Hospital Affiliated to Lanzhou University, Lanzhou, ChinaEwing 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.https://www.karger.com/Article/FullText/522152extraosseous ewing sarcomaprimitive neuroectodermal tumoresophagusdiagnosistreatment |
| spellingShingle | Jie Li Pengfei Sun Li Ma Xianhua Min Binqiang Ye Yao Zhang Weiwei Ta Jiyun Deng Xiangrong Cao Chi Dong Ewing Sarcoma and Primitive Neuroectodermal Tumor of the Thoracic Esophagus: Case Report and Comprehensive Literature Review Case Reports in Oncology extraosseous ewing sarcoma primitive neuroectodermal tumor esophagus diagnosis treatment |
| title | Ewing Sarcoma and Primitive Neuroectodermal Tumor of the Thoracic Esophagus: Case Report and Comprehensive Literature Review |
| title_full | Ewing Sarcoma and Primitive Neuroectodermal Tumor of the Thoracic Esophagus: Case Report and Comprehensive Literature Review |
| title_fullStr | Ewing Sarcoma and Primitive Neuroectodermal Tumor of the Thoracic Esophagus: Case Report and Comprehensive Literature Review |
| title_full_unstemmed | Ewing Sarcoma and Primitive Neuroectodermal Tumor of the Thoracic Esophagus: Case Report and Comprehensive Literature Review |
| title_short | Ewing Sarcoma and Primitive Neuroectodermal Tumor of the Thoracic Esophagus: Case Report and Comprehensive Literature Review |
| title_sort | ewing sarcoma and primitive neuroectodermal tumor of the thoracic esophagus case report and comprehensive literature review |
| topic | extraosseous ewing sarcoma primitive neuroectodermal tumor esophagus diagnosis treatment |
| url | https://www.karger.com/Article/FullText/522152 |
| work_keys_str_mv | AT jieli ewingsarcomaandprimitiveneuroectodermaltumorofthethoracicesophaguscasereportandcomprehensiveliteraturereview AT pengfeisun ewingsarcomaandprimitiveneuroectodermaltumorofthethoracicesophaguscasereportandcomprehensiveliteraturereview AT lima ewingsarcomaandprimitiveneuroectodermaltumorofthethoracicesophaguscasereportandcomprehensiveliteraturereview AT xianhuamin ewingsarcomaandprimitiveneuroectodermaltumorofthethoracicesophaguscasereportandcomprehensiveliteraturereview AT binqiangye ewingsarcomaandprimitiveneuroectodermaltumorofthethoracicesophaguscasereportandcomprehensiveliteraturereview AT yaozhang ewingsarcomaandprimitiveneuroectodermaltumorofthethoracicesophaguscasereportandcomprehensiveliteraturereview AT weiweita ewingsarcomaandprimitiveneuroectodermaltumorofthethoracicesophaguscasereportandcomprehensiveliteraturereview AT jiyundeng ewingsarcomaandprimitiveneuroectodermaltumorofthethoracicesophaguscasereportandcomprehensiveliteraturereview AT xiangrongcao ewingsarcomaandprimitiveneuroectodermaltumorofthethoracicesophaguscasereportandcomprehensiveliteraturereview AT chidong ewingsarcomaandprimitiveneuroectodermaltumorofthethoracicesophaguscasereportandcomprehensiveliteraturereview |