Srpski arhiv za celokupno lekarstvo 2018 Volume 146, Issue 3-4, Pages: 207-210
https://doi.org/10.2298/SARH170323126S
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Extraskeletal Ewing sarcoma in the anterior abdominal wall
Savić Đorđe (Mother and Child Health Care Institute of Serbia “Dr Vukan Čupić”, Belgrade + School of Medicine, Belgrade)
Đuričić Slaviša M. (Mother and Child Health Care Institute of Serbia “Dr Vukan Čupić”, Belgrade + University of Banjaluka, School of Medicine, Banjaluka, Bosnia and Herzegovina)
Miličković Maja (Mother and Child Health Care Institute of Serbia “Dr Vukan Čupić”, Belgrade + School of Medicine, Belgrade)
Đokić Dragomir (Mother and Child Health Care Institute of Serbia “Dr Vukan Čupić”, Belgrade)
Grujić Blagoje (Mother and Child Health Care Institute of Serbia “Dr Vukan Čupić”, Belgrade + School of Medicine, Belgrade)
Vukadin Miroslav (Mother and Child Health Care Institute of Serbia “Dr Vukan Čupić”, Belgrade)
Samardžija Gordana (Mother and Child Health Care Institute of Serbia “Dr Vukan Čupić”, Belgrade)
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.
Keywords: extraskeletal Ewing sarcoma, primitive neuroectodermal tumor, soft tissue, abdominal wall, surgery