{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,8]],"date-time":"2026-09-08T11:02:41Z","timestamp":1788865361681,"version":"build-2803163510"},"reference-count":37,"publisher":"SAGE Publications","issue":"5","license":[{"start":{"date-parts":[[2022,9,28]],"date-time":"2022-09-28T00:00:00Z","timestamp":1664323200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"funder":[{"DOI":"10.13039\/100012543","name":"Shanghai Science and Technology Development Foundation","doi-asserted-by":"publisher","award":["19MC1911000"],"award-info":[{"award-number":["19MC1911000"]}],"id":[{"id":"10.13039\/100012543","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100007219","name":"Shanghai Municipal Natural Science Foundation","doi-asserted-by":"crossref","award":["18ZR1407300"],"award-info":[{"award-number":["18ZR1407300"]}],"id":[{"id":"10.13039\/100007219","id-type":"DOI","asserted-by":"crossref"}]},{"name":"Shanghai Municipal Key Clinical Specialty","award":["shslczdzk01301"],"award-info":[{"award-number":["shslczdzk01301"]}]}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Int J Surg Pathol"],"published-print":{"date-parts":[[2023,8]]},"abstract":"<jats:p>\n                    Desmoplastic small round cell tumor (DSRCT) is a rare aggressive malignancy typically originating from the abdominal or pelvic cavity. DSRCT presenting as a primary head and neck tumor has rarely been described in the literature. We present three cases of DSRCT arising in the head and neck to further characterize its clinicopathological features. All three patients were male and aged 36, 30 and 17 years. The involved sites included the orbit (1 case) and submandibular gland (2 cases). The tumors ranged in size from 2.4 to 3.5\u2005cm (mean, 2.1\u2005cm). Histologically, all tumors showed irregular-shaped, variable-sized nests of small round cells deposited in an abundant desmoplastic stroma. Tumor cells contained scant amounts of eosinophilic cytoplasm and small hyperchromatic nuclei with inconspicuous nucleoli. Immunohistochemically, the tumors were positive for keratin (AE1\/AE3) (3\/3), desmin (3\/3), vimentin (2\/2), NSE (1\/1) and EMA (1\/1). Fluorescence in situ hybridization (FISH) analysis demonstrated the presence of\n                    <jats:italic toggle=\"yes\">EWSR1<\/jats:italic>\n                    and\n                    <jats:italic toggle=\"yes\">WT1<\/jats:italic>\n                    rearrangements in all three cases. All patients received surgery and adjuvant chemotherapy and\/or radiotherapy. There was no evidence of recurrence and metastasis in two patients, and the third suffered lung metastasis. DSRCT arising in the head and neck represents an extremely rare condition. It is easily mistaken as poorly differentiated carcinoma due to similar morphology and expression of epithelial markers. Immunohistochemistry assay in conjunction with molecular detection of\n                    <jats:italic toggle=\"yes\">EWSR1::WT1<\/jats:italic>\n                    fusion will be helpful for arriving at an accurate diagnosis to avoid misdiagnosis and inappropriate treatment.\n                  <\/jats:p>","DOI":"10.1177\/10668969221117989","type":"journal-article","created":{"date-parts":[[2022,9,29]],"date-time":"2022-09-29T02:06:10Z","timestamp":1664417170000},"page":"742-750","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":11,"title":["Desmoplastic Small Round Cell Tumor of the Head and Neck: A Clinicopathological, Immunohistochemical and Molecular Analysis of Three Cases with Literature Review"],"prefix":"10.1177","volume":"31","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1402-1000","authenticated-orcid":false,"given":"Meng","family":"Sun","sequence":"first","affiliation":[{"name":"Fudan University Shanghai Cancer Center"},{"name":"Shanghai Medical College, Fudan University"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mengyuan","family":"Shao","sequence":"additional","affiliation":[{"name":"Fudan University Shanghai Cancer Center"},{"name":"Shanghai Medical College, Fudan University"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xuebing","family":"Jiang","sequence":"additional","affiliation":[{"name":"Department of Pathology, Fudan University Minhang Hospital, Fudan University, Shanghai 200032, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tangchen","family":"Yin","sequence":"additional","affiliation":[{"name":"Fudan University Shanghai Cancer Center"},{"name":"Shanghai Medical College, Fudan University"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Qifeng","family":"Wang","sequence":"additional","affiliation":[{"name":"Fudan University Shanghai Cancer Center"},{"name":"Shanghai Medical College, Fudan University"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lu","family":"Zhao","sequence":"additional","affiliation":[{"name":"Fudan University Shanghai Cancer Center"},{"name":"Shanghai Medical College, Fudan University"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jiahan","family":"Liu","sequence":"additional","affiliation":[{"name":"Fudan University Shanghai Cancer Center"},{"name":"Shanghai Medical College, Fudan University"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"I Weng","family":"Lao","sequence":"additional","affiliation":[{"name":"Fudan University Shanghai Cancer Center"},{"name":"Shanghai Medical College, Fudan University"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1033-0914","authenticated-orcid":false,"given":"Lin","family":"Yu","sequence":"additional","affiliation":[{"name":"Fudan University Shanghai Cancer Center"},{"name":"Shanghai Medical College, Fudan University"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jian","family":"Wang","sequence":"additional","affiliation":[{"name":"Fudan University Shanghai Cancer Center"},{"name":"Shanghai Medical College, Fudan University"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"179","published-online":{"date-parts":[[2022,9,28]]},"reference":[{"key":"e_1_3_3_2_2","volume-title":"WHO Classification of Tumours Soft Tissue and Bone Tumours","author":"The WHO Classification of Tumours Editorial Board","year":"2020","unstructured":"The WHO Classification of Tumours Editorial Board. 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