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Basal cell carcinomas (BCC), odontogenic keratocysts and medulloblastomas are the main tumor types encountered, but meningiomas, ovarian or cardiac fibromas and sarcomas have also been described. The clinical features and tumor risks are different depending on the causative gene. Due to the rarity of this condition, there is little data on phenotype-genotype correlations. This report summarizes genotype-based recommendations for screening patients with\n                    <jats:italic>PTCH1<\/jats:italic>\n                    and\n                    <jats:italic>SUFU<\/jats:italic>\n                    -related Gorlin syndrome, discussed during a workshop of the Host Genome Working Group of the European branch of the International Society of Pediatric Oncology (SIOPE HGWG) held in January 2020. In order to allow early detection of BCC, dermatologic examination should start at age 10 in\n                    <jats:italic>PTCH1<\/jats:italic>\n                    , and at age 20 in\n                    <jats:italic>SUFU<\/jats:italic>\n                    PV carriers. Odontogenic keratocyst screening, based on odontologic examination, should begin at age 2 with annual orthopantogram beginning around age 8 for\n                    <jats:italic>PTCH1<\/jats:italic>\n                    PV carriers only. For medulloblastomas, repeated brain MRI from birth to 5\u00a0years should be proposed for\n                    <jats:italic>SUFU<\/jats:italic>\n                    PV carriers only. Brain MRI for meningiomas and pelvic ultrasound for ovarian fibromas should be offered to both\n                    <jats:italic>PTCH1<\/jats:italic>\n                    and\n                    <jats:italic>SUFU<\/jats:italic>\n                    PV carriers. Follow-up of patients treated with radiotherapy should be prolonged and thorough because of the risk of secondary malignancies. Prospective evaluation of evidence of the effectiveness of these surveillance recommendations is required.\n                  <\/jats:p>","DOI":"10.1007\/s10689-021-00247-z","type":"journal-article","created":{"date-parts":[[2021,4,16]],"date-time":"2021-04-16T02:04:37Z","timestamp":1618538677000},"page":"317-325","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":42,"title":["Current recommendations for cancer surveillance in Gorlin syndrome: a report from the SIOPE host genome working group (SIOPE HGWG)"],"prefix":"10.1007","volume":"20","author":[{"given":"L.","family":"Guerrini-Rousseau","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M. 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