title: Gorlin-Goltz syndrome and neoplasms: a case study.
abstract: Gorlin syndrome is a rare autosomal dominant disorder exhibiting high penetrance and variable expressivity. It is characterized by facial dysmorphism, skeletal anomalies, multiple basal cell carcinomas, odontogenic keratocysts (OKC), palmar and plantar pits, bifid ribs, vertebral anomalies and a variety of other malformations. Various neoplasms, such as medulloblastomas, meningiomas, ovarian and cardiac fibromas are also found in this syndrome.
  
  
  OBJECTIVE
  To describe a twelve-year-old patient with Gorlin-Goltz syndrome, with basal cell carcinomas and promyelocytic leukemia developed after receiving craniospinal radiation for a medulloblastoma. Bifid ribs as well as mandibular and maxillar OKC were also diagnosed Conclusion: The patient with Gorlin-Goltz syndrome should receive close follow-up for early detection of malformations nd malignant neoplasias.
authors.affiliation: Pediatric Oncology Institute--GRAACC, Medical School of São Paulo, Federal University of São Paulo, SP, Brazil. nnflopes@terra.com.br
authors.forename: Nilza N F
authors.initials: NN
authors.lastname: Lopes
authors.forename: Eliana M
authors.initials: EM
authors.lastname: Caran
authors.forename: Maria Lucia
authors.initials: ML
authors.lastname: Lee
authors.forename: Nasjla Saba
authors.initials: NS
authors.lastname: Silva
authors.forename: André Caroli
authors.initials: AC
authors.lastname: Rocha
authors.forename: Carla R D
authors.initials: CR
authors.lastname: Macedo
mesh_terms: D001478:Basal Cell Nevus Syndrome / Q000473:pathology; D020295:Brain Stem Neoplasms / Q000532:radiotherapy* / Q000601:surgery; D002648:Child; D016371:Cranial Irradiation; D018450:Disease Progression; D017809:Fatal Outcome; D005500:Follow-Up Studies; D006801:Humans; D015473:Leukemia, Promyelocytic, Acute / Q000473:pathology*; D007953:Leukemia, Radiation-Induced / Q000473:pathology; D008297:Male; D008527:Medulloblastoma / Q000532:radiotherapy* / Q000601:surgery; D009364:Neoplasm Recurrence, Local / Q000473:pathology; D009378:Neoplasms, Multiple Primary / Q000473:pathology*; D016609:Neoplasms, Second Primary / Q000473:pathology*; D013131:Spine / Q000528:radiation effects