Journal
AMERICAN JOURNAL OF MEDICAL GENETICS PART A
Volume 155A, Issue 9, Pages 2091-2097Publisher
WILEY-BLACKWELL
DOI: 10.1002/ajmg.a.34128
Keywords
basal cell nevus syndrome; nevoid basal cell carcinoma syndrome; basal cell carcinoma nevus syndrome; Gorlin syndrome; Gorlin-Goltz syndrome; odontogenic cysts; basal cell carcinoma; medulloblastoma; primitive neuroectodermal tumor; hereditary neoplastic syndromes; developmental bone diseases; genodermatoses
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The first international colloquium on basal cell nevus syndrome (BCNS) was held at Saint Louis University School of Medicine and supported by the Basal Cell Carcinoma Nevus Syndrome (BCCNS) Life Support Network (www.gorlinsyndrome.org). The foremost goal of the conference was to review and revise the prior diagnostic criteria and define the surveillance recommendations for affected pediatric and adult patients to allow for early intervention. The invited consensus group participants included geneticists, dermatologists, orthopedists, neurologists, and dental/oral medicine specialists, who treat patients with BCNS or related disorders. This group also included individuals who have a research interest in BCNS and who additionally serve on the medical advisory board of the BCCNS Life Support Network. Expert opinion was based on the collective clinical and research experience of the consensus group participants after presentation and review of the previously published literature regarding diagnosis and treatment of BCNS. A consensus was achieved and agreed upon by open roundtable discussion of the group participants. The consensus statement outlines the proposed diagnostic and management protocols that will hopefully limit morbidity and mortality for affected individuals until more specific and targeted therapies are widely available. (C) 2011 Wiley-Liss, Inc.
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