Gorlin-Goltz syndrome and neoplasms: a case study

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 var...

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Veröffentlicht in:The Journal of clinical pediatric dentistry 2010-12, Vol.35 (2), p.203-206
Hauptverfasser: Lopes, Nilza N F, Caran, Eliana M, Lee, Maria Lucia, Silva, Nasjla Saba, Rocha, André Caroli, Macedo, Carla R D
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container_end_page 206
container_issue 2
container_start_page 203
container_title The Journal of clinical pediatric dentistry
container_volume 35
creator Lopes, Nilza N F
Caran, Eliana M
Lee, Maria Lucia
Silva, Nasjla Saba
Rocha, André Caroli
Macedo, Carla R D
description 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. 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.
doi_str_mv 10.17796/jcpd.35.2.x01248284w166485
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subjects Basal Cell Nevus Syndrome - pathology
Brain Stem Neoplasms - radiotherapy
Brain Stem Neoplasms - surgery
Child
Cranial Irradiation
Dentistry
Disease Progression
Fatal Outcome
Follow-Up Studies
Humans
Leukemia, Promyelocytic, Acute - pathology
Leukemia, Radiation-Induced - pathology
Male
Medulloblastoma - radiotherapy
Medulloblastoma - surgery
Neoplasm Recurrence, Local - pathology
Neoplasms, Multiple Primary - pathology
Neoplasms, Second Primary - pathology
Spine - radiation effects
title Gorlin-Goltz syndrome and neoplasms: a case study
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