{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,4]],"date-time":"2026-05-04T23:32:04Z","timestamp":1777937524815,"version":"3.51.4"},"reference-count":24,"publisher":"SAGE Publications","issue":"5","license":[{"start":{"date-parts":[[2022,6,22]],"date-time":"2022-06-22T00:00:00Z","timestamp":1655856000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"funder":[{"name":"Al Ain Medical District Human Research Ethics Committee","award":["IRR541\/17"],"award-info":[{"award-number":["IRR541\/17"]}]}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Pediatr Dev Pathol"],"published-print":{"date-parts":[[2022,9]]},"abstract":"<jats:p>Teratocarcinosarcoma is an extremely rare malignancy of the nasal cavity and paranasal sinuses. It exhibits both sarcomatous and carcinomatous components. Less than 100 cases are reported. It presents in adults with only two reported cases in infancy. Here we present a case of 3-week-old female with antenatally detected ocular mass. MRI revealed an exophytic right ocular mass (10 \u00d7 7.0 \u00d7 7.0\u00a0cm) with intracranial extension. The tumor consisted of malignant glands and mesenchymal elements of undifferentiated blastema-like cells and immature neuroepithelium. After an initial diagnosis and treatment for a Wilms tumor protocol, the mass showed no response. A second opinion rendered a diagnosis of sinonasal teratocarcinosarcoma. The patient underwent surgical resection and seven cycles of CNS ICE chemotherapy. A second debulking surgery revealed a very scant viable tumor with post-treatment changes. The patient is alive at 43\u00a0months on weekly vincristine maintenance. Molecular testing revealed a somatic CTNNB1 gene mutation. In conclusion, this is a rare and aggressive tumor which showed disease free survival beyond that reported in the literature with the appropriate use of multimodality therapy.<\/jats:p>","DOI":"10.1177\/10935266221111127","type":"journal-article","created":{"date-parts":[[2022,6,22]],"date-time":"2022-06-22T18:42:09Z","timestamp":1655923329000},"page":"562-567","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":4,"title":["Congenital Teratocarcinosarcoma With\n                    <i>CTNNB1<\/i>\n                    Gene Mutation Presenting as an Ocular Mass"],"prefix":"10.1177","volume":"25","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5147-0657","authenticated-orcid":false,"given":"Saeeda","family":"Almarzooqi","sequence":"first","affiliation":[{"name":"Pathology Department, College of Medicine and Health Sciences, UAE University, Al-Ain, United Arab Emirates"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5698-3702","authenticated-orcid":false,"given":"Miguel","family":"Reyes-M\u00fagica","sequence":"additional","affiliation":[{"name":"UPMC Children\u2019s Hospital of Pittsburgh, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bassam R","family":"Ali","sequence":"additional","affiliation":[{"name":"Department of Genetics and Genomics, College of Medicine and Health Sciences, United Arab Emirates University, Al-Ain, United Arab Emirates"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Aya","family":"Habbal","sequence":"additional","affiliation":[{"name":"Tawam Hospital, Al Ain, United Arab Emirates"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mohammad J","family":"Asha","sequence":"additional","affiliation":[{"name":"Department of Surgery, Tawam Hospital, Al Ain, United Arab Emirates"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Eman T","family":"AlShamsi","sequence":"additional","affiliation":[{"name":"Al Qassimi Women\u2019s and Children\u2019s Hospital, Sharjah, United Arab 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