Gastric Ewing Sarcoma

Young, Richard A.; Dishop, Megan K.; Eshun, Francis; Gage, Sandra; Williams, Dana I.

doi:10.1097/mpg.0000000000003279

Cited by 2 publications

(3 citation statements)

References 8 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…A brief search of PubMed revealed that only 55 cases of GI ES had been reported in the literature, most of which were single case reports. [2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17] To date, no systematic study has been performed to investigate the clinical and pathologic characteristics of GI ES. The purpose of this study is to summarize the clinical and pathologic features of GI ES by reporting a series of 25 cases with a review of the literature.…”

Section: Discussionmentioning

confidence: 99%

“…A brief search of PubMed revealed that only 55 cases of GI ES had been reported in the literature, most of which were single case reports 2–17 . To date, no systematic study has been performed to investigate the clinical and pathologic characteristics of GI ES.…”

Section: Discussionmentioning

confidence: 99%

“…Misdiagnosis may in turn lead to inappropriate treatment and incorrect assessment of the prognosis. To date, most of the studies regarding GI ES are brief case reports 2–17 . Here, we report 25 cases of ES arising primarily in the GI tract, focusing on the clinicopathological and molecular features, differential diagnosis, and biological behavior.…”

mentioning

confidence: 99%

See 2 more Smart Citations

Gastrointestinal Ewing Sarcoma

Yin,

Shao,

Sun

et al. 2023

American Journal of Surgical Pathology

View full text Add to dashboard Cite

Occurrence of extraskeletal Ewing sarcoma (ES) in the gastrointestinal (GI) tract is extremely rare. Here, we report 25 cases of ES arising primarily in the GI tract with a focus on the clinicopathological and molecular features, differential diagnosis, and biological behavior. Thirteen patients (52%) were male, and 12 (48%) were female with age ranging from 9 to 59 years (mean: 36.2 years; median: 38 years). Twenty-one tumors (84%) occurred in the small intestine, 3 (12%) in the stomach, and 1 (4%) in the anal canal. At operation, 8/18 (44.4%) patients presented with abdominopelvic disseminated disease. Tumor size measured from 2 to 25 cm (mean: 8.2 cm; median: 6 cm) in maximum size. Microscopically, the tumors were composed of infiltrative small round, ovoid, or short spindle cells arranged mostly in lobular and solid sheet-like patterns with a rich capillary vasculature. Focal formation of Homer Wright–type rosettes and pseudoalveolar architecture was noted each in 2 (8%) cases and 3 (12%) cases. Besides CD99 (25/25; 100%), Fli-1 (15/15, 100%), and NKX2.2 (14/16; 87.5%), the tumor cells also showed variable staining of CD117 (14/17; 82.4%). Of 25 cases, 23 (92%) demonstrated EWSR1 rearrangement by fluorescence in situ hybridization analysis. The 2 cases with negative fluorescence in situ hybridization results were found to harbor EWSR1::ERG and EWSR1::FLI1 fusion by further RNA sequencing, respectively, with a median follow-up of 12 months (range: 1 to 42 months), 5/19 (26.3%) patients developed visceral metastasis and 12/19 (63.2%) patients died of the disease (range:1 to 33 months; median: 9 months). This study showed that GI ES had a predilection for the small intestine, although other sites of the GI tract could also be involved. GI ES had a poor prognosis with a high rate of mortality, particularly in patients with abdominopelvic disseminated disease. In light of appropriate therapeutic strategies and prognostic considerations, it is essential not to misdiagnose GI ES as gastrointestinal stromal tumor owing to the expression of aberrant CD117.

show abstract

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Gastrointestinal Ewing Sarcoma

Yin,

Shao,

Sun

et al. 2023

American Journal of Surgical Pathology

View full text Add to dashboard Cite

show abstract

Extraskeletal Ewing Sarcoma of the Gastrointestinal and Hepatobiliary Tract

Shiyanbola,

Nigdelioglu,

Dhall

et al. 2024

American Journal of Surgical Pathology

View full text Add to dashboard Cite

Ewing sarcoma (ES) is an uncommon mesenchymal neoplasm that typically develops as a bone mass, although up to 30% arise in extraskeletal sites. ES of the gastrointestinal (GI) and hepatobiliary tract is rare and may be misdiagnosed as other, more common neoplasms that occur in these sites. However, the correct classification of extraskeletal ES is important for timely clinical management and prognostication. We reviewed our experience of ES in the GI and hepatobiliary tract in order to further highlight the clinicopathologic features of these neoplasms and document the potential for misdiagnosis in this setting. The archives and consultation files of 6 academic institutions were retrospectively queried for cases of ES occurring in the GI and hepatobiliary tract. The histologic slides and ancillary studies were reviewed and clinical data were retrieved for each case through the electronic medical records, when available. Twenty-three patients with ES in the GI and/or hepatobiliary tract were identified from 2000 to 2022. Of these, 11 were women and 12 were men with a median age of 38 years (range, 2 to 64). Tumor locations included the pancreas (n=5), liver (n=2), stomach (n=3), colorectum (n=3), and small intestine (n=5), as well as tumors involving multiple organs, pelvis and retroperitoneum (n=5). Tumor size varied between 2 cm and 18 cm. Twenty were primary and 3 were metastases. Of the 23 cases, only 17% were initially diagnosed as ES. The most common misdiagnoses involved various forms of neuroendocrine neoplasia due to expression of synaptophysin and other neuroendocrine markers (22%). A wide variety of diagnoses including GI stromal tumor was considered due to aberrant CD117 expression (4%). The diagnosis of ES was ultimately confirmed by detection of the EWSR1 rearrangement in 22 cases. The remaining case was diagnosed using traditional immunohistochemistry. Follow-up information was available in 20 cases, with follow-up time varying between 2 and 256 months. Six patients with follow-up died of disease between 6 and 60 months following initial presentation. Our data indicate ES in the GI and hepatobiliary tract is commonly misdiagnosed leading to a delay in therapy. In light of the attendant therapeutic and prognostic implications, ES should be considered in the differential diagnosis of any GI or hepatobiliary tumor with epithelioid and/or small round cell morphology.

show abstract

Gastric Ewing Sarcoma

Abstract: We report the youngest case of a patient with ulcerative colitis (UC) and primary sclerosing cholangitis (PSC) with Menetrier disease (MD).

Cited by 2 publications

References 8 publications

Gastrointestinal Ewing Sarcoma

Gastrointestinal Ewing Sarcoma

Extraskeletal Ewing Sarcoma of the Gastrointestinal and Hepatobiliary Tract

Contact Info

Product

Resources

About