{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,23]],"date-time":"2026-04-23T16:40:35Z","timestamp":1776962435014,"version":"3.51.4"},"reference-count":15,"publisher":"Oxford University Press (OUP)","issue":"4","license":[{"start":{"date-parts":[[2026,4,23]],"date-time":"2026-04-23T00:00:00Z","timestamp":1776902400000},"content-version":"vor","delay-in-days":22,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2026,4,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Primary pancreatic Ewing sarcoma (ES) is an exceedingly rare malignancy, with fewer than 100 confirmed cases reported worldwide. We report the case of a young adult woman presenting with a large solid-cystic pancreatic body-tail mass, initially diagnosed as solid pseudopapillary neoplasm and subsequently misinterpreted as metastatic endometrial stromal sarcoma following resection. Lack of adjuvant therapy and loss to follow-up preceded metastatic recurrence, whereupon targeted immunohistochemistry and molecular analysis established the definitive diagnosis of extraosseous ES with t(11;22)(q24;q12) translocation. Palliative chemotherapy yielded transient disease control before rapid progression and death. This case illustrates the diagnostic challenges posed by this rare entity due to overlapping clinicoradiologic and histologic features with more common pancreatic tumors, emphasizing the critical role of early inclusion of CD99, NKX2.2, and EWSR1 testing to facilitate appropriate multimodal management and improve outcomes.<\/jats:p>","DOI":"10.1093\/jscr\/rjag295","type":"journal-article","created":{"date-parts":[[2026,4,6]],"date-time":"2026-04-06T11:19:20Z","timestamp":1775474360000},"source":"Crossref","is-referenced-by-count":0,"title":["Extraosseous Ewing sarcoma of the distal pancreas: diagnostic pitfalls and the critical role of molecular testing"],"prefix":"10.1093","volume":"2026","author":[{"given":"Rakesh Kumar","family":"Singh","sequence":"first","affiliation":[{"name":"Department of Surgical Gastroenterology, Indira Gandhi Institute of Medical Sciences , Bailey Road, Raja Bazar, Patna District, Patna 800014, Bihar ,","place":["India"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tushar","family":"Saini","sequence":"additional","affiliation":[{"name":"Department of General Surgery (GI Surgery Unit), Rohilkhand Medical College and Hospital , Pilibhit Bypass Road (Pawan Vihar \/ Mahanagar area), Bareilly District, Bareilly 243006, Uttar Pradesh ,","place":["India"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sanjay","family":"Kumar","sequence":"additional","affiliation":[{"name":"Department of Surgical Gastroenterology, Indira Gandhi Institute of Medical Sciences , Bailey Road, Raja Bazar, Patna District, Patna 800014, Bihar ,","place":["India"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0009-0007-3693-3362","authenticated-orcid":false,"given":"Kumar","family":"Kaushik","sequence":"additional","affiliation":[{"name":"Department of General Surgery, Rohilkhand Medical College and Hospital , Pilibhit Bypass Road (Pawan Vihar \/ Mahanagar area), Bareilly District, Bareilly 243006, Uttar Pradesh ,","place":["India"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Vinaysheel","family":"Priyadarshi","sequence":"additional","affiliation":[{"name":"Department of General Surgery, Rohilkhand Medical College and Hospital , Pilibhit Bypass Road (Pawan Vihar \/ Mahanagar area), Bareilly District, Bareilly 243006, Uttar Pradesh ,","place":["India"]}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[2026,4,23]]},"reference":[{"key":"2026042311540726200_ref1","volume-title":"Ewing sarcoma","author":"Durer","year":"2024"},{"key":"2026042311540726200_ref2","volume-title":"Atlas Genet Cytogenet Oncol Haematol","author":"Giordano","year":"2025"},{"key":"2026042311540726200_ref3","doi-asserted-by":"publisher","first-page":"e17391","DOI":"10.7759\/cureus.17391","article-title":"Ewing sarcoma and Ewing-like sarcoma and the role of NKX2.2 immunoreactivity","volume":"13","author":"Ullah","year":"2021","journal-title":"Cureus"},{"key":"2026042311540726200_ref4","doi-asserted-by":"publisher","first-page":"2694","DOI":"10.3390\/diagnostics14232694","article-title":"Primary and metastatic pancreatic Ewing sarcomas: a case report and review of the literature","volume":"14","author":"Koufopoulos","year":"2024","journal-title":"Diagnostics (Basel)"},{"key":"2026042311540726200_ref5","doi-asserted-by":"publisher","first-page":"85","DOI":"10.1186\/1477-7819-9-85","article-title":"Primary sarcoma of the pancreas, a rare histopathological entity. A case report with review of literature","volume":"9","author":"Ambe","year":"2011","journal-title":"World J Surg Oncol"},{"key":"2026042311540726200_ref6","article-title":"Survival in pancreatic Ewing's sarcoma family of tumors improved with ifosfamide and etoposide regimens: a systematic review and survival analysis","volume":"169","author":"Kaiser","journal-title":"Gastroenterology"},{"key":"2026042311540726200_ref7","doi-asserted-by":"publisher","first-page":"1368564","DOI":"10.3389\/fonc.2024.1368564","article-title":"Ewing sarcoma of the pancreas: a pediatric case report and narrative literature review","volume":"14","author":"Liu","year":"2024","journal-title":"Front Oncol"},{"key":"2026042311540726200_ref8","doi-asserted-by":"publisher","first-page":"1668671","DOI":"10.3389\/fonc.2025.1668671","article-title":"Editorial: management of rare oncological cases","volume":"15","author":"Castagneto-Gissey","year":"2025","journal-title":"Front Oncol"},{"key":"2026042311540726200_ref9","doi-asserted-by":"publisher","first-page":"674","DOI":"10.1038\/modpathol.2017.162","article-title":"ZC3H7B-BCOR high-grade endometrial stromal sarcomas: a report of 17 cases of a newly defined entity","volume":"31","author":"Lewis","year":"2018","journal-title":"Mod Pathol"},{"key":"2026042311540726200_ref10","doi-asserted-by":"publisher","first-page":"1766","DOI":"10.1148\/rg.2021210008","article-title":"Pancreatic masses in children and young adults: multimodality review with pathologic correlation","volume":"41","author":"Qiu","year":"2021","journal-title":"Radiographics"},{"key":"2026042311540726200_ref11","doi-asserted-by":"publisher","first-page":"12","DOI":"10.1111\/pin.13293","article-title":"Ewing and Ewing-like sarcomas: a morphological guide through genetically-defined entities","volume":"73","author":"Yoshida","year":"2023","journal-title":"Pathol Int"},{"key":"2026042311540726200_ref12","doi-asserted-by":"publisher","first-page":"993","DOI":"10.1097\/PAS.0b013e31824ee43c","article-title":"NKX2.2 is a useful immunohistochemical marker for Ewing sarcoma","volume":"36","author":"Yoshida","year":"2012","journal-title":"Am J Surg Pathol"},{"key":"2026042311540726200_ref13","doi-asserted-by":"publisher","first-page":"1587","DOI":"10.18632\/oncotarget.20098","article-title":"Robust diagnosis of Ewing sarcoma by immunohistochemical detection of super-enhancer-driven EWSR1-ETS targets","volume":"9","author":"Baldauf","year":"2017","journal-title":"Oncotarget"},{"key":"2026042311540726200_ref14","doi-asserted-by":"publisher","first-page":"194","DOI":"10.1310\/hpj5003-194","article-title":"Doxorubicin and dacarbazine (AD) regimen for soft tissue sarcomas","volume":"50","author":"Adkins","year":"2015","journal-title":"Hosp Pharm"},{"key":"2026042311540726200_ref15","doi-asserted-by":"publisher","first-page":"150","DOI":"10.1186\/s43055-023-01094-x","article-title":"Primary pancreatic Ewing sarcoma with metastases on FDG PET\/CT","volume":"54","author":"Singh","year":"2023","journal-title":"Egypt J Radiol Nucl Med"}],"container-title":["Journal of Surgical Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/jscr\/article-pdf\/2026\/4\/rjag295\/68143396\/rjag295.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/jscr\/article-pdf\/2026\/4\/rjag295\/68143396\/rjag295.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,4,23]],"date-time":"2026-04-23T15:54:14Z","timestamp":1776959654000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/jscr\/article\/doi\/10.1093\/jscr\/rjag295\/8661419"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,4,1]]},"references-count":15,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2026,4,1]]}},"URL":"https:\/\/doi.org\/10.1093\/jscr\/rjag295","relation":{},"ISSN":["2042-8812"],"issn-type":[{"value":"2042-8812","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2026,4]]},"published":{"date-parts":[[2026,4,1]]},"article-number":"rjag295"}}