CASE REPORT: Extraskeletal Ewing sarcoma of the abdominal wall

Askri, A.; Farhat, Leïla Ben; Ghariani, Brahim; Rabeh, A; Dali, N.; Saïd, W.; Hendaoui, L.

doi:10.1102/1470-7330.2008.0023

Cited by 10 publications

(13 citation statements)

References 12 publications

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“…Poor prognosis indicators include extraskeletal involvement and metastatic disease. Primary extraskeletal Ewing's sarcoma is a rare soft tissue tumour 4–7 …”

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confidence: 99%

Intra-abdomen Ewing's sarcoma

Huang

Chiang

Jan

et al. 2011

ANZ Journal of Surgery

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An 18-year-old male adolescent presented with sudden onset, colicky epigastric pain. On physical examination, tenderness was over right upper quadrant and Murphy's sign was positive. No loco-regional swelling or tenderness was noted in the extremities. His medical history was otherwise unremarkable. Preliminary laboratory investigations revealed leukocytosis of 14.02 10 3 /mL (normal range, 5-10 10 3 /mL), serum lactate dehydrogenase of 235 U/L (normal range, 45-90 U/L), serum bilirubin of 1.5 mg/dL (normal range, 0.4-2.0 mg/dL) and alkaline phosphatase of 88 U/L (normal range, 60-120 U/L). Serum alanine transaminase and r-glutamyl transpeptidase values were normal. Viral serology results for hepatitis B and hepatitis C were negative. Transabdominal ultrasound revealed heterogenic hepatic tumour 10.4 ¥ 5.7 cm in size and moderate ascites. Contrast-enhanced computed tomography revealed a huge lobulated solid tumour 14 ¥ 11 ¥ 10 cm in size over segments 5 and 6 of the right lobe of liver. There is fluid accumulation in the subhepatic space. Normal appearance of gall bladder without stone formation and compression of the pancreatic head by the mass were noted as well (Fig. 1). Cystadenocarcinoma of gall bladder versus hepato-cellular carcinoma was the preoperative differential diagnosis. Intra-operative findings revealed hemoperitoneum and an intra-abdominal tumour with rupture over subhepatic space, connecting to the gall bladder and liver. During dissection, the tumour was found to be partially encapsulated and easily dissected from the retro-peritoneum, adrenal gland and kidney (Fig. 2). As the frozen section reported malignancy, the patient underwent cholecystectomy, total resection of the tumour, hepato-duodenal lymph node dissection and omentectomy. His postoperative recovery was uneventful and was discharged on the 7th post-operative day. Pathology report: grossly, the tumour measured 13 ¥ 8 ¥ 7 cm in size, grey and mildly firm in consistency, focal areas revealed massive necrosis (Fig. 3). On microscopic examination, the tumour composed of hypercellular small blue round cell tumour cells. Ewing's sarcoma was confirmed by periodic acid-schiff (PAS) staining (+), neuron-specific enolase (NSE) (+) (Fig. 4) and reverse transcription polymerase chain reaction (RT-PCR) result of translocation t (11; 22) (q24; q12). There was no extraskeletal lesion found on bone scan. Subsequent adjuvant combination chemotherapy was received at the oncology department. Fig. 1. Contrast-enhanced computed tomography showed fluid accumulation in the subhepatic space and normal appearance of gall bladder without stone formation (arrow) and compression of the pancreatic head by the mass (arrowhead).Fig. 2. A partially encapsulated intra-abdominal tumour with rupture over subhepatic space (arrow), connecting to the gall bladder (G) and liver (L). Fig. 3. The tumour measured 13 ¥ 8 ¥ 7 cm in size, grey and mildly firm in consistency (arrow); focal area revealed massive necrosis (arrowhead). G, gall bladder.

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“…Poor prognosis indicators include extraskeletal involvement and metastatic disease. Primary extraskeletal Ewing's sarcoma is a rare soft tissue tumour 4–7 …”

mentioning

confidence: 99%

Intra-abdomen Ewing's sarcoma

Huang

Chiang

Jan

et al. 2011

ANZ Journal of Surgery

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“…However, there are several case reports or small series of extraskeletal ES/PNET with primary location in the kidney, lung, pancreas, uterus, urinary bladder, ovary, testis, parotid gland, mesenterium, skin, and subcutaneous tissue [4,5]. After an extensive literature search on the PubMed, using keywords "Ewing sarcoma, " "primitive neuroectodermal tumor, " and "abdominal wall, " we found only three cases of neoplasms in this primary location [6,7,8]. Another three cases of extraskeletal ES in the abdominal wall location were reported, but all of them were described as tumors confined to the subcutaneous adipose tissue, so we consider that they should not be included in the group of abdominal wall neoplasms [4,9,10].…”

Section: Discussionmentioning

confidence: 91%

“…After the diagnostic incisional biopsy, neoadjuvant chemotherapy was started including vincristine, cyclophosphamide, and Adriamycin for three cycles. Because of poor response to chemotherapy, surgery was required, but authors did not report any data of the follow-up [7]. Roncati et al [8] reported a third case of small extraskeletal ES/PNET measuring 1.5 cm, located in the subcutaneous tissue with deep muscle infiltration, appearing in a 45-year-old man.…”

Section: Discussionmentioning

confidence: 99%

Extraskeletal Ewing sarcoma in the anterior abdominal wall

Savić

Djuricic²,

Milickovic

et al. 2018

Srp Arh Celok Lek

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Introduction Extraskeletal Ewing sarcoma (ES) is a highly malignant neoplasm occurring most commonly in the thoracic wall and the paravertebral region. ES belongs to the group of small round cell tumors and displays pathognomonic structural abnormalities of the EWS gene. The aim of this article was to present extraskeletal ES in an 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 hemorrhagic tumor tissue was discovered in the preperitoneal space. Histopathological and immunohistochemical analyses revealed a tumor resembling extraskeletal ES. A postoperative CT scan showed the tumor rest, which was completely removed in the second operation. Molecular genetic analysis confirmed extraskeletal ES by finding the EWSR1-FLI1 fusion gene. Chemotherapy and radiotherapy according to the 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 the molecular genetic finding, and the first case described in children at this anatomic site. Due to an extremely rare location, unusual clinical presentation, and needed genetic analysis, the tumor treatment strategy was modified with good short-term results.

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“…In our study of 10 patients (six males, four females), the median age at diagnosis was 36.5 years. The median age across different case reports of abdominal ES/PNET have been in similar age-groups (15–35 years) [ 8 , 10 , 12 – 15 , 20 , 21 ]. National Cancer Database (NCDB) and Surveillance, Epidemiology, and End Results (SEER) database studies show that the median age of patients at diagnosis of EES is older than its skeletal counterparts and has female predilection.…”

Section: Discussionmentioning

confidence: 99%

“…ES represents the least differentiated and PNET represents the most differentiated tumors [7]. Abdominal ES/ PNETs are rare with few case reports in literature [8][9][10][11][12][13][14][15][16][17][18][19][20]. Abdominal ES/PNETs are often confused with other SRCTs such as embryonal rhabdomyosarcomas, neuroblastomas, and lymphomas.…”

Section: Introductionmentioning

confidence: 99%

Management of Abdominal Ewing’s Sarcoma: A Single Institute Experience

Chatterjee

Patkar

Purandare

et al. 2021

Indian J Surg Oncol

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Ewing’s sarcoma (ES)/primitive neuroectodermal tumors (PNETs) are a rare group of tumors commonly arising from bones, uncommonly from soft tissues, and rarely from abdomen. The aim of the study was to analyze the outcome (recurrence-free survival[RFS]), patient characteristics, role of FDG-PET (fluorodeoxyglucose positron emission tomography) computerized scan, chemotherapy and radiation, and prognostic factors. We retrospectively studied patients diagnosed with abdominal ES/PNET and treated surgically between June 2005 and November 2019. Ten patients were included in the study, with a median age of 36.5 years (19–46 years). The median follow-up was 25 months (3–178 months). The site of origin was the retroperitoneum, small bowel, and abdominal wall in six, two, and two patients, respectively. 70% of patients were treated with induction chemotherapy. R0 resection was achieved in 90% of patients. With chemotherapy, there was significant reduction in tumor size (p = 0.034) with non-significant reduction in SUV max (p = 0.31). The 1- and 2-year RFS were 88.90% and 76.20%, respectively. Pathological peritoneal metastasis and ability to achieve R0 resection were prognostic factors affecting RFS. These patients must be offered multimodality treatment. Induction chemotherapy significantly reduces the tumor size. Pathological peritoneal metastasis and ability to achieving R0 resection significantly affect survival.

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CASE REPORT: Extraskeletal Ewing sarcoma of the abdominal wall

Cited by 10 publications

References 12 publications

Intra-abdomen Ewing's sarcoma

Intra-abdomen Ewing's sarcoma

Extraskeletal Ewing sarcoma in the anterior abdominal wall

Management of Abdominal Ewing’s Sarcoma: A Single Institute Experience

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