{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,5]],"date-time":"2026-03-05T12:06:45Z","timestamp":1772712405431,"version":"3.50.1"},"reference-count":11,"publisher":"Journal of Neurosurgery Publishing Group (JNSPG)","issue":"4","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JNS"],"published-print":{"date-parts":[[2015,4]]},"abstract":"<jats:p>Desmoplastic small round cell tumors (DSRCTs) are rare, aggressive neoplasms that typically arise from abdominal and pelvic peritoneum in young adults. Other primary sites are uncommon, and an intracranial origin is exceptionally rare. Here the authors report the first case of a DSRCT presenting as a primary suprasellar tumor causing panhypopituitarism and severe bitemporal hemianopia in a young man. Macroscopic debulking of the tumor was undertaken, and histology revealed features of DSRCT. Reverse transcription polymerase chain reaction confirmed the presence of Ewing's sarcoma\u2013Wilms tumor 1 (EWS-WT1) gene rearrangement specific to DSRCT. Postoperative whole-body imaging showed no primary malignancy elsewhere. The tumor recurred 4 months after surgery, and this was followed by cervical and mediastinal lymph node metastases. The patient died 20 months after initial presentation of rapidly progressive disease. DSRCTs should be included in the differential diagnosis of an unusual suprasellar mass in young adults. Early diagnosis is essential, and once the tumor is identified histologically, gross-total resection and radical postoperative treatment involving radiotherapy, chemotherapy, and close surveillance are required because of the lesion's potential for rapidly progressive malignancy.<\/jats:p>","DOI":"10.3171\/2014.10.jns132490","type":"journal-article","created":{"date-parts":[[2014,12,5]],"date-time":"2014-12-05T14:59:29Z","timestamp":1417791569000},"page":"773-777","source":"Crossref","is-referenced-by-count":22,"title":["Intracranial desmoplastic small round cell tumor presenting as a suprasellar mass"],"prefix":"10.3171","volume":"122","author":[{"given":"Sravan K.","family":"Thondam","sequence":"first","affiliation":[{"name":"Department of Endocrinology, University Hospital Aintree, Liverpool;"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Daniel du","family":"Plessis","sequence":"additional","affiliation":[{"name":"Neuropathology Unit and Clinical Neurosciences Center, Salford Royal Hospital, Salford, Greater Manchester;"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Daniel J.","family":"Cuthbertson","sequence":"additional","affiliation":[{"name":"Department of Endocrinology, University Hospital Aintree, Liverpool;"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kumar S. V.","family":"Das","sequence":"additional","affiliation":[{"name":"Walton Center for Neurology & Neurosurgery, Liverpool; and"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mohsen","family":"Javadpour","sequence":"additional","affiliation":[{"name":"Walton Center for Neurology & Neurosurgery, Liverpool; and"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ian A.","family":"MacFarlane","sequence":"additional","affiliation":[{"name":"Department of Endocrinology, University Hospital Aintree, Liverpool;"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"James","family":"Leggate","sequence":"additional","affiliation":[{"name":"Neuropathology Unit and Clinical Neurosciences Center, Salford Royal Hospital, Salford, Greater Manchester;"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Brian","family":"Haylock","sequence":"additional","affiliation":[{"name":"Clatterbridge Cancer Center, Liverpool, United Kingdom"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Christina","family":"Daousi","sequence":"additional","affiliation":[{"name":"Department of Endocrinology, University Hospital Aintree, Liverpool;"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1754","reference":[{"key":"b1-jns132490","series-title":"Pediatr Blood Cancer","doi-asserted-by":"publisher","first-page":"545","DOI":"10.1002\/pbc.21648","article-title":"Intracerebral small round cell tumor: an unusual case with EWS-WT1 translocation","volume":"51","author":"Bouchireb","year":"2008"},{"key":"b2-jns132490","series-title":"J Clin Oncol","doi-asserted-by":"publisher","first-page":"3028","DOI":"10.1200\/JCO.1998.16.9.3028","article-title":"Clinical, pathologic, and molecular spectrum of tumors associated with t(11;22)(p13;q12): desmoplastic small round-cell tumor and its variants","volume":"16","author":"Gerald","year":"1998"},{"key":"b3-jns132490","series-title":"Pediatr Pathol","doi-asserted-by":"publisher","first-page":"177","DOI":"10.3109\/15513818909022347","article-title":"Case 2. 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