Extraskeletal Ewing sarcoma in children and adolescents: impact of narrow but negative surgical margin

Qureshi, Sajid; Laskar, Siddharth; Kembhavi, Seema; Talole, Sanjay; Chinnaswamy, Girish; Vora, Tushar; Ramadwar, Mukta; Desai, Saral; Khanna, Nehal; Muckaden, Mary Ann; Kurkure, Purna

doi:10.1007/s00383-013-3409-2

Cited by 26 publications

(21 citation statements)

References 17 publications

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“…Consistent with the available literature, the male preponderance of EES was also observed in our sample [14,[20][21][22]. Research suggestes that SES patients are younger than EES patients [1,14,17,18].…”

Section: Discussionsupporting

confidence: 92%

Extraskeletal Ewing’s sarcoma: outcomes and CT features of endoceliac lesions

Chen

Yang

Liu

et al. 2020

Preprint

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Background To analyse the clinicopathological features and outcomes of patients with extraskeletal Ewing’s sarcoma (EES). The basic imaging characteristics of endoceliac lesions, which are always misdiagnosed preoperatively, are also summarised.Methods This study involved EES patients admitted to our centre between January 2000 and January 2020. Clinical data from patients with EES (n = 25) and computed tomography (CT) data from patients with endoceliac lesions (n = 8) were retrospectively reviewed.Results The sample comprised 18 males and 7 females with a median age of 30 years (range 1–72 years). Seven patients had EES originating from surface sites and 18 had EES originating from endoceliac sites. The median tumour size was 8.0 cm (range 2.5–17.0 cm). In total, 20% of patients had distant metastasis at diagnosis. In the univariate analyses, tumour size > 8 cm, undergoing surgery, and regional lymph nodes metastasis were risk factors for poor prognosis of EES. In the multivariate analysis, patients undergoing surgery and regional lymph node metastasis were independent predictors of overall survival (OS). Endoceliac EES cases frequently exhibited lobulated contour (87.5%), absence of calcification (75%), severe necrosis or cystic degeneration (75%), heterogeneous enhancement (100%), moderate enhancement (75%), ill-defined borderline (62.5%), and organ invasion (75%). Half of the patients with endoceliac EES had CT features of lymphadenopathy.Conclusions Comprehensive understanding of the clinicopathological and imaging features of EES is beneficial for diagnosis, development of therapeutic strategies, and accurate prediction of prognosis for this rare malignant tumour. The results suggest that evaluation of the status of regional lymph nodes is important for the treatment of EES.

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Section: Discussionsupporting

confidence: 92%

Extraskeletal Ewing’s sarcoma: outcomes and CT features of endoceliac lesions

Chen

Yang

Liu

et al. 2020

Preprint

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“…The National Comprehensive Cancer Network guidelines recommend that any ES/PNET should be treated with local treatment (surgery and/or radiotherapy) plus chemotherapy [53]. Nevertheless, consistent with our findings in Table 1, others have suggested that complete surgery, if feasible, may be a better option for local disease considering the late side effects of high-dose radiotherapy especially for children [52, 54]. Because small bowel ES/PNET is extremely rare and difficult to cure, our case will contribute to the understanding of the prognosis and determination of optimal management.…”

Section: Discussionsupporting

confidence: 83%

Primary Ewing’s sarcoma/primitive neuroectodermal tumor of the ileum: case report of a 16-year-old Chinese female and literature review

Zhang

Cao

et al. 2017

Diagn Pathol

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BackgroundEwing’s sarcoma (ES) and primitive neuroectodermal tumors (PNET) are closely related tumors. Although soft tissue ES/PNET are common in clinical practice, they are rare in the small intestine. Because of the absence of characteristic clinical symptoms, they are easily misdiagnosed as other benign or malignant diseases.Case presentationHere, we present the case of a 16-year-old female who complained of anemia and interval hematochezia. Her serum test results showed only a slight elevation of CA-125 and a low level of hemoglobin. Computer tomography and magnetic resonance imaging revealed a cystic and solid mass in the lower abdominal quadrant and pelvic region, which prompted suspicion of a malignant gastrointestinal stromal tumor of the small intestine. After resection, the tumor’s histology and immunohistochemistry (positive for CD99, vimentin and synaptophysin) results suggested ES/PNET. Fluorescent in situ hybridization tests proved the breakpoint rearrangement of the EWSR1 gene in chr 22.Ultrastructural analysis revealed neurosecretory and glycogen granules in the tumor cell cytoplasm.ConclusionsTogether, these data supported the diagnosis of a rare case of localized ES/PNET in the small intestine without adjuvant chemo- or radiotherapy. To our knowledge, this is the first report from China of a primary small bowel ES/PNET in the English-language literature. In addition, on the basis of findings from previous publications and the current case, the optimal treatment for localized gastrointestinal ES/PNET is discussed.

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“…Neoadjuvant chemotherapy followed by aggressive surgical excision followed with or without RT is the preferred treatment. The goal of surgery should be a three-dimensional tumor-free margin [ 8 ]. Although EES is very radiosensitive, surgical advancements and the risks associated with radiation (secondary malignancies) have reduced the reliance upon radiation [ 9 ].…”

Section: Discussionmentioning

confidence: 99%

Extraosseus Ewing's Sarcoma of the Forearm

Bansal

Prasad

Shahi

et al. 2020

Cureus

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A 22-year-old female presented with painful, progressive swelling of the right forearm for six months. Physical examination revealed a 7 cm x 5 cm firm, tender soft-tissue swelling over the anterior aspect of the right proximal forearm with normal overlying temperature. Xrays showed increased soft tissue shadow but without any bony involvement. Serum alkaline phosphatase, serum lactate dehydrogenase, and leukocyte count were raised. MRI of the right forearm revealed enhancing soft tissue lesion with internal hemorrhagic and necrotic components involving the flexor carpi radialis muscle. Core biopsy confirmed the diagnosis of extraosseus Ewing's sarcoma. Neoadjuvant chemotherapy, wide local tumor excision, and adjuvant chemotherapy and radiotherapy were done. The patient then lost to follow-up and presented again after six months with a fungating mass and neurovascular involvement for which an above-elbow amputation was done. We, through this case report, aim to discuss the clinical and radiological findings, line of management, and the importance of early detection and treatment and a regular follow-up for extraosseus Ewing's sarcoma of the extremity.

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Extraskeletal Ewing sarcoma in children and adolescents: impact of narrow but negative surgical margin

Abstract: Optimal local control is feasible in children with EES regardless of the quantitative extent of negative margins. Achieving a three-dimensional tumor-free margin should be the goal of surgical resection.

Cited by 26 publications

References 17 publications

Extraskeletal Ewing’s sarcoma: outcomes and CT features of endoceliac lesions

Extraskeletal Ewing’s sarcoma: outcomes and CT features of endoceliac lesions

Primary Ewing’s sarcoma/primitive neuroectodermal tumor of the ileum: case report of a 16-year-old Chinese female and literature review

Extraosseus Ewing's Sarcoma of the Forearm

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