Intraabdominal lesser sac metastasis from Ewing’s sarcoma: An exceptional localization

Ewing’s sarcoma/primitive neuroectodermal tumor is rare and aggressive with a poor prognosis. Intraabdominal metastases are an uncommon condition. Metastasis in the lesser sac is an exceptional occurrence. To the best of our knowledge, this location has not been described previously. We report a case of a 15-year-old patient treated for Ewing’s sarcoma of the left arm 6 years back. She had developed a suspicious mass in the lesser sac 6 years following her primary tumor. The histopathologic exam revealed a tumor with “small round cells” that were positive for CD99, confirming the relapse of Ewing’s sarcoma. The relapse was successfully managed with chemotherapy and surgery. Intraabdominal, extraintestinal masses in patients treated previously for Ewing’s sarcoma should be considered as Ewing’s sarcoma relapse in the differential diagnosis. We fully describe the management of this atypical relapse, with different components of clinical, radiological, and histological findings.

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Ewing's sarcoma and primitive neuroectodermal tumor in adults: are they different from Ewing's sarcoma and primitive neuroectodermal tumor in children?

Journal of Clinical Oncology ◽

10.1200/jco.1997.15.7.2611 ◽

1997 ◽

Vol 15 (7) ◽

pp. 2611-2621 ◽

Cited By ~ 71

Author(s):

M W Verrill ◽

I R Judson ◽

C L Harmer ◽

C Fisher ◽

J M Thomas ◽

...

Keyword(s):

Metastatic Disease ◽

Primary Tumor ◽

Primitive Neuroectodermal Tumor ◽

Ewing’S Sarcoma ◽

Ewing's Sarcoma ◽

Multidisciplinary Treatment ◽

Progression Free Survival ◽

Primary Treatment ◽

Neuroectodermal Tumor ◽

Primary Tumor Site

PURPOSE To determine whether age at diagnosis influences the behavior of Ewing's sarcoma and primitive neuroectodermal tumor (PNET). PATIENTS AND METHODS We reviewed the clinical features, treatment, and outcome of 59 consecutive patients with Ewing's sarcoma and PNET treated on the Adult Sarcoma Unit at our institution from 1980 to 1995. RESULTS The 37 male and 22 female patients had a median age of 24 years. Lower limb was the most common primary tumor site. Fifteen patients had nonmetastatic tumor less than 100-mL volume, 27 had nonmetastatic disease greater than 100-mL volume, and 17 had evidence of metastatic disease at presentation. The origin of the primary tumor was soft tissue in 28 cases, bone in 30, and uncertain in one. The Kaplan-Meier estimate of 5-year overall survival (OS) in all patients was 38% and of progression-free survival (PFS), 27%. When patients with metastatic disease at presentation were excluded, these figures increased to 52% and 34%, respectively. Bulk of disease at presentation and response to primary treatment were statistically highly significant predictors of both PFS and OS. Age and tissue of origin of the tumor did not influence outcome. CONCLUSION The behavior of Ewing's sarcoma and PNET in adults is no different from its behavior in children. We feel the way forward in the treatment of adults with Ewing's sarcoma and PNET is for them to be included in the current multicenter trials of multidisciplinary treatment directed at children.

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Faculty Opinions recommendation of Impact of EWS-ETS fusion type on disease progression in Ewing's sarcoma/peripheral primitive neuroectodermal tumor: prospective results from the cooperative Euro-E.W.I.N.G. 99 trial.

Faculty Opinions – Post-Publication Peer Review of the Biomedical Literature ◽

10.3410/f.2836957.2503055 ◽

2010 ◽

Author(s):

Katie Janeway

Keyword(s):

Disease Progression ◽

Primitive Neuroectodermal Tumor ◽

Ewing’S Sarcoma ◽

Ewing's Sarcoma ◽

Neuroectodermal Tumor ◽

Peripheral Primitive Neuroectodermal Tumor ◽

Fusion Type

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Extraosseous Ewing’s Sarcoma: Pictorial Review of Imaging Findings, Differential Diagnosis, and Pathologic Correlation

Indian Journal of Radiology and Imaging ◽

10.1055/s-0041-1729770 ◽

2021 ◽

Author(s):

Alan Alexander ◽

Kyle Hunter ◽

Michael Rubin ◽

Ambarish P. Bhat

Keyword(s):

Differential Diagnosis ◽

Ewing’S Sarcoma ◽

Ewing's Sarcoma ◽

Multimodality Imaging ◽

Imaging Features ◽

Older Children ◽

Radiologic Findings ◽

Pictorial Review ◽

Malignant Mesenchymal Tumor ◽

Extraosseous Ewing’S Sarcoma

AbstractExtraosseous Ewing’s sarcoma (EES), first described in 1969, is a malignant mesenchymal tumor just like its intraosseous counterpart. Although Ewing’s sarcomas are common bone tumors in young children, EESs are rarer and more commonly found in older children/adults, often carrying a poorer prognosis. We discuss the multimodality imaging features of EES and the differential diagnosis of an aggressive appearing mass in proximity to skeletal structures, with pathologic correlates. This review highlights the need to recognize the variability of radiologic findings in EES such as the presence of hemorrhage, rich vascularity, and cystic or necrotic regions and its imaging similarity to other neoplasms that are closely related pathologically.

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