{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,18]],"date-time":"2026-06-18T06:43:16Z","timestamp":1781764996496,"version":"3.54.5"},"reference-count":10,"publisher":"Oxford University Press (OUP)","issue":"11","license":[{"start":{"date-parts":[[2025,11,26]],"date-time":"2025-11-26T00:00:00Z","timestamp":1764115200000},"content-version":"vor","delay-in-days":25,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2025,11,26]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Background: Ataxia-telangiectasia (A-T) is a rare autosomal recessive disorder characterized by progressive cerebellar ataxia, oculocutaneous telangiectasia, and profound immunodeficiency. Although individuals with A-T exhibit a heightened predisposition to malignancies, the incidence of hepatocellular carcinoma is exceptionally rare where diagnosis may be further complicated by the underlying immunodeficiency. Case Presentation: We present a 14-year-old male with a confirmed diagnosis of A-T since the age of two, experiencing progressive neurological decline. Hepatocellular carcinoma was identified during routine systemic surveillance through imaging studies and rising alpha-fetoprotein levels. The patient underwent chemotherapy with PLADO regimen and sorafenib, alongside monthly intravenous immunoglobulin. He had an exceptionally satisfactory course and successful outcome following chemotherapy. Conclusion: The coexistence of A-T and hepatocellular carcinoma is an exceptionally rare phenomenon, with only a limited number of cases reported globally. Comprehensive, multidisciplinary management is crucial in optimizing survival outcomes and enhancing the quality of life in these medically complex patients.<\/jats:p>","DOI":"10.1093\/omcr\/omaf237","type":"journal-article","created":{"date-parts":[[2025,10,16]],"date-time":"2025-10-16T12:12:53Z","timestamp":1760616773000},"source":"Crossref","is-referenced-by-count":2,"title":["When the unexpected strikes: hepatocellular carcinoma in a teen with ataxia-telangiectasia"],"prefix":"10.1093","volume":"2025","author":[{"given":"Sogol","family":"Alesaeidi","sequence":"first","affiliation":[{"name":"Pediatric Congenital Hematologic Disorders Research Center, Research Institute for Children\u2019s Health, Mofid Children\u2019s Hospital, Shahid Beheshti University of Medical Sciences , Tehran ,","place":["Iran"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8118-7329","authenticated-orcid":false,"given":"Samin","family":"Alavi","sequence":"additional","affiliation":[{"name":"Pediatric Congenital Hematologic Disorders Research Center, Research Institute for Children\u2019s Health, Mofid Children\u2019s Hospital, Shahid Beheshti University of Medical Sciences , Tehran ,","place":["Iran"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zahra","family":"Chavoshzadeh","sequence":"additional","affiliation":[{"name":"Department of Allergy and Clinical Immunology, Mofid Children\u2019s Hospital, Shahid Beheshti University of Medical Sciences , Tehran ,","place":["Iran"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maryam","family":"Kazemi Aghdam","sequence":"additional","affiliation":[{"name":"Department of Pediatric Pathology, Mofid Children\u2019s Hospital, Shahid Beheshti University of Medical Sciences , Tehran ,","place":["Iran"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mitra","family":"Khalili","sequence":"additional","affiliation":[{"name":"Department of Pediatric Radiology, Mofid Children\u2019s Hospital, Shahid Beheshti University of Medical Sciences , Tehran ,","place":["Iran"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fatemeh","family":"Dastmalchi","sequence":"additional","affiliation":[{"name":"Pediatric Congenital Hematologic Disorders Research Center, Research Institute for Children\u2019s Health, Mofid Children\u2019s Hospital, Shahid Beheshti University of Medical Sciences , Tehran ,","place":["Iran"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2025,11,26]]},"reference":[{"key":"2025112607530302000_ref1","doi-asserted-by":"publisher","volume-title":"Seminars in Pediatric 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