{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,10]],"date-time":"2026-07-10T13:29:19Z","timestamp":1783690159158,"version":"3.55.0"},"reference-count":28,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2015,7,14]],"date-time":"2015-07-14T00:00:00Z","timestamp":1436832000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Clinical Genetics"],"published-print":{"date-parts":[[2016,3]]},"abstract":"<jats:p>Neurofibromatosis type 1 (<jats:styled-content style=\"fixed-case\">NF1<\/jats:styled-content>) is an autosomal dominant hereditary neurocutaneous syndrome characterized by multi\u2010system involvement and an increased incidence of both benign and malignant tumors. In this study, we evaluated the clinical presentation and prognosis of <jats:styled-content style=\"fixed-case\">NF1<\/jats:styled-content> and malignancy. Between 1975 and 2013, 26 (5%) of the 473 patients with <jats:styled-content style=\"fixed-case\">NF1<\/jats:styled-content> at our center developed non\u2010neurofibroma neoplasms. The patient files of 26 subjects with tumors, other than optic glioma, were analyzed retrospectively to evaluate clinical features and treatment results. The age at diagnosis of <jats:styled-content style=\"fixed-case\">NF1<\/jats:styled-content> ranged from 3 months to 16 years (median 5.5 years). The age range at tumor diagnosis was 1.5\u201333 years (median 8 years) in these 26 patients. The tumor histological subtypes included the following: 12 soft\u2010tissue tumors (6 malignant peripheral nerve sheath tumors (<jats:styled-content style=\"fixed-case\">MPNST<\/jats:styled-content>), 5 rhabdomyosarcomas (<jats:styled-content style=\"fixed-case\">RMS<\/jats:styled-content>) and 1 malignant fibrous histiocytoma), 11 brain tumors (6 low\u2010grade gliomas, 3 high\u2010grade gliomas, and 2 medulloblastoma), 2 neuroblastomas and 1 non\u2010Hodgkin's lymphoma. Twelve of 26 patients were alive at the time of the study. Although benign brain tumors with <jats:styled-content style=\"fixed-case\">NF1<\/jats:styled-content> are more common, high\u2010grade brain tumors also occur. Thus, careful and regular follow\u2010up is crucial for early detection of malignancy in <jats:styled-content style=\"fixed-case\">NF1<\/jats:styled-content> patients.<\/jats:p>","DOI":"10.1111\/cge.12625","type":"journal-article","created":{"date-parts":[[2015,6,15]],"date-time":"2015-06-15T08:53:27Z","timestamp":1434358407000},"page":"341-345","source":"Crossref","is-referenced-by-count":51,"title":["Neurofibromatosis type 1 and malignancy in childhood"],"prefix":"10.1111","volume":"89","author":[{"given":"A.","family":"Varan","sequence":"first","affiliation":[{"name":"Department of Pediatric Oncology Hacettepe University, Cancer Institute  Ankara Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"H.","family":"\u015een","sequence":"additional","affiliation":[{"name":"Department of Pediatric Oncology Hacettepe University, Cancer Institute  Ankara Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"B.","family":"Ayd\u0131n","sequence":"additional","affiliation":[{"name":"Department of Pediatric Oncology Hacettepe University, Cancer Institute  Ankara Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"B.","family":"Yal\u00e7\u0131n","sequence":"additional","affiliation":[{"name":"Department of Pediatric Oncology Hacettepe University, Cancer Institute  Ankara Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"T.","family":"Kutluk","sequence":"additional","affiliation":[{"name":"Department of Pediatric Oncology Hacettepe University, Cancer Institute  Ankara Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C.","family":"Aky\u00fcz","sequence":"additional","affiliation":[{"name":"Department of Pediatric Oncology Hacettepe University, Cancer Institute  Ankara Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2015,7,14]]},"reference":[{"key":"e_1_2_5_2_1","doi-asserted-by":"publisher","DOI":"10.1093\/oxfordjournals.aje.a010118"},{"key":"e_1_2_5_3_1","doi-asserted-by":"crossref","first-page":"136","DOI":"10.2340\/0001555575136140","article-title":"Life expectancy, mortality and prognostic factors in neurofibromatosis type 1. 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