Extraskeletal Ewing sarcoma in the anterior abdominal wall
Introduction. Extraskeletal Ewing sarcoma (ES), is highly malignant neoplasm occurring most commonly in the thoracic wall and paravertebral region. ES belongs to the group of small round cell tumors and displays pathognomonic structural abnormalities of EWS gene. The aims of this article wa...
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| Format: | Article |
| Language: | English |
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Serbian Medical Society
2018-01-01
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| Series: | Srpski Arhiv za Celokupno Lekarstvo |
| Subjects: | |
| Online Access: | http://www.doiserbia.nb.rs/img/doi/0370-8179/2018/0370-81791700126S.pdf |
| _version_ | 1818385878251208704 |
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| author | Savić Đorđe Đuričić Slaviša M. Miličković Maja Đokić Dragomir Grujić Blagoje Vukadin Miroslav Samardžija Gordana |
| author_facet | Savić Đorđe Đuričić Slaviša M. Miličković Maja Đokić Dragomir Grujić Blagoje Vukadin Miroslav Samardžija Gordana |
| author_sort | Savić Đorđe |
| collection | DOAJ |
| description | Introduction. Extraskeletal Ewing sarcoma (ES), is highly malignant neoplasm
occurring most commonly in the thoracic wall and paravertebral region. ES
belongs to the group of small round cell tumors and displays pathognomonic
structural abnormalities of EWS gene. The aims of this article was to present
extraskeletal ES in extremely rare anatomic location, an unusual clinical
presentation, and modified treatment strategy. Case outline. A 15-year-old
boy was admitted to the hospital with acute abdominal pain in the right iliac
region. During urgent operation, because of suspected appendicitis with
periappendicular infiltrate, partly haemorrhagic tumor tissue was discovered
in the preperitoneal space. Histopathological and immunohistochemical
analyses revealed tumor resembling extraskeletal ES. Postoperative CT scan
has shown the tumor rest, which was completely removed in the second
operation. The molecular genetic analysis confirmed extraskeletal ES by
finding of the EWSR1-FLI1 fusion gene. Chemotherapy and radiotherapy
according to VAC protocol were started, and the patient is free of the
disease eight months after the first operation. Conclusion. Our case is the
fourth case of extraskeletal ES located in the abdominal wall, the second
case confirmed by molecular genetic finding, and the first case described in
children at this anatomic location. Due to extremely rare location, unusual
clinical presentation, and needed genetic analysis, the strategy of tumor
treatment was modified with the good short-term result. |
| first_indexed | 2024-12-14T03:45:09Z |
| format | Article |
| id | doaj.art-85e117effa634dc5a3c3a7020d0e0384 |
| institution | Directory Open Access Journal |
| issn | 0370-8179 2406-0895 |
| language | English |
| last_indexed | 2024-12-14T03:45:09Z |
| publishDate | 2018-01-01 |
| publisher | Serbian Medical Society |
| record_format | Article |
| series | Srpski Arhiv za Celokupno Lekarstvo |
| spelling | doaj.art-85e117effa634dc5a3c3a7020d0e03842022-12-21T23:18:22ZengSerbian Medical SocietySrpski Arhiv za Celokupno Lekarstvo0370-81792406-08952018-01-011463-420721010.2298/SARH170323126S0370-81791700126SExtraskeletal Ewing sarcoma in the anterior abdominal wallSavić Đorđe0Đuričić Slaviša M.1Miličković Maja2Đokić Dragomir3Grujić Blagoje4Vukadin Miroslav5Samardžija Gordana6Mother and Child Health Care Institute of Serbia “Dr Vukan Čupić”, Belgrade + School of Medicine, BelgradeMother and Child Health Care Institute of Serbia “Dr Vukan Čupić”, Belgrade + University of Banjaluka, School of Medicine, Banjaluka, Bosnia and HerzegovinaMother and Child Health Care Institute of Serbia “Dr Vukan Čupić”, Belgrade + School of Medicine, BelgradeMother and Child Health Care Institute of Serbia “Dr Vukan Čupić”, BelgradeMother and Child Health Care Institute of Serbia “Dr Vukan Čupić”, Belgrade + School of Medicine, BelgradeMother and Child Health Care Institute of Serbia “Dr Vukan Čupić”, BelgradeMother and Child Health Care Institute of Serbia “Dr Vukan Čupić”, BelgradeIntroduction. Extraskeletal Ewing sarcoma (ES), is highly malignant neoplasm occurring most commonly in the thoracic wall and paravertebral region. ES belongs to the group of small round cell tumors and displays pathognomonic structural abnormalities of EWS gene. The aims of this article was to present extraskeletal ES in extremely rare anatomic location, an unusual clinical presentation, and modified treatment strategy. Case outline. A 15-year-old boy was admitted to the hospital with acute abdominal pain in the right iliac region. During urgent operation, because of suspected appendicitis with periappendicular infiltrate, partly haemorrhagic tumor tissue was discovered in the preperitoneal space. Histopathological and immunohistochemical analyses revealed tumor resembling extraskeletal ES. Postoperative CT scan has shown the tumor rest, which was completely removed in the second operation. The molecular genetic analysis confirmed extraskeletal ES by finding of the EWSR1-FLI1 fusion gene. Chemotherapy and radiotherapy according to VAC protocol were started, and the patient is free of the disease eight months after the first operation. Conclusion. Our case is the fourth case of extraskeletal ES located in the abdominal wall, the second case confirmed by molecular genetic finding, and the first case described in children at this anatomic location. Due to extremely rare location, unusual clinical presentation, and needed genetic analysis, the strategy of tumor treatment was modified with the good short-term result.http://www.doiserbia.nb.rs/img/doi/0370-8179/2018/0370-81791700126S.pdfextraskeletal Ewing sarcomaprimitive neuroectodermal tumorsoft tissueabdominal wallsurgery |
| spellingShingle | Savić Đorđe Đuričić Slaviša M. Miličković Maja Đokić Dragomir Grujić Blagoje Vukadin Miroslav Samardžija Gordana Extraskeletal Ewing sarcoma in the anterior abdominal wall Srpski Arhiv za Celokupno Lekarstvo extraskeletal Ewing sarcoma primitive neuroectodermal tumor soft tissue abdominal wall surgery |
| title | Extraskeletal Ewing sarcoma in the anterior abdominal wall |
| title_full | Extraskeletal Ewing sarcoma in the anterior abdominal wall |
| title_fullStr | Extraskeletal Ewing sarcoma in the anterior abdominal wall |
| title_full_unstemmed | Extraskeletal Ewing sarcoma in the anterior abdominal wall |
| title_short | Extraskeletal Ewing sarcoma in the anterior abdominal wall |
| title_sort | extraskeletal ewing sarcoma in the anterior abdominal wall |
| topic | extraskeletal Ewing sarcoma primitive neuroectodermal tumor soft tissue abdominal wall surgery |
| url | http://www.doiserbia.nb.rs/img/doi/0370-8179/2018/0370-81791700126S.pdf |
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