{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,8,6]],"date-time":"2025-08-06T13:52:11Z","timestamp":1754488331949},"reference-count":0,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2000,1,1]],"date-time":"2000-01-01T00:00:00Z","timestamp":946684800000},"content-version":"vor","delay-in-days":1095,"URL":"http:\/\/creativecommons.org\/licenses\/by\/3.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Sarcoma"],"published-print":{"date-parts":[[1997,1]]},"abstract":"<jats:p><jats:italic>Purpose<\/jats:italic>. Four further cases of desmoplastic small round cell tumour with multi\nphenotypic differentiation are described.<\/jats:p><jats:p><jats:italic>Subjects<\/jats:italic>. Two patients were typical (male, adolescent with peritoneal tumour and, in one case, liver metastases) and\ndid not respond well to treatment. Two other patients showed less usual features (young, female with retroperitoneal\ndisease, both with intraspinal extension and renal tract obstruction). Both responded favourably to multi\u2010modal treatment\nregimens including extensive and invasive supportive care.<\/jats:p><jats:p><jats:italic>Results<\/jats:italic>. Histologically, all tumours showed clear features of this diagnosis, namely angulated nests of small cells in a\nbackground of fibrovascular stroma. Immunohistochemistry typically showed divergent differentiation with neural, muscle\nand epithelial marker positivity. All four tumours stained positive for the Wilms\u2032 tumour 1 suppressor gene product.\nElectron microscopy showed intercellular tight junctions, cytoplasmatic intermediate filaments and absence of microvilli.\nRare neurosecretory\u2010type granules were observed.<\/jats:p><jats:p><jats:italic>Discussion<\/jats:italic>. These cases illustrate a broader spectrum of clinical presentation than previously\nassociated with this diagnosis.<\/jats:p>","DOI":"10.1080\/13577149778380","type":"journal-article","created":{"date-parts":[[2002,7,26]],"date-time":"2002-07-26T16:11:58Z","timestamp":1027699918000},"page":"103-108","update-policy":"http:\/\/dx.doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Desmoplastic Small Round Cell Tumour of Childhood: A Report of Four Cases Demonstrating                   Wider Clinical Features and Variable Outcome"],"prefix":"10.1155","volume":"1","author":[{"given":"Ruma","family":"Ray","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Heidi C. L.","family":"Traunecker","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Faro","family":"Raafat","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michael C. G.","family":"Stevens","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[1997,1]]},"container-title":["Sarcoma"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/downloads.hindawi.com\/journals\/sarcoma\/1997\/423257.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1080\/13577149778380","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,8,8]],"date-time":"2024-08-08T18:46:36Z","timestamp":1723142796000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1080\/13577149778380"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1997,1]]},"references-count":0,"journal-issue":{"issue":"2","published-print":{"date-parts":[[1997,1]]}},"alternative-id":["10.1080\/13577149778380"],"URL":"https:\/\/doi.org\/10.1080\/13577149778380","archive":["Portico"],"relation":{},"ISSN":["1357-714X","1369-1643"],"issn-type":[{"type":"print","value":"1357-714X"},{"type":"electronic","value":"1369-1643"}],"subject":[],"published":{"date-parts":[[1997,1]]},"assertion":[{"value":"1997-01-01","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}