medigraphic.com
ISSN 0001-0944 (Print)
Órgano Oficial de la Asociación Dental Mexicana

2013, Number 1

<< Back Next >>

Rev ADM 2013; 70 (1)

Gorlin-Goltz Syndrome: A Clinical case report

Escamilla OCE, Sánchez RLR, Sánchez GLR, Treviño AMG, Martínez MHR, Rivera SG

Language: Spanish
References: 8
Page: 43-45
PDF size: 72.02 Kb.


ABSTRACT

The Gorlin-Goltz syndrome or nevoid basal cell carcinoma syndrome is a hereditary autosomal dominant disorder characterized by development defects and a high predisposition to cancer. It was first reported in 1894 by Jarisch and White and later described by Gorlin and Goltz in 1960.The condition is characterized by development of basal cell carcinomas, odontogenic keratocysts, medulloblastomas and ovarian fibromas. Due to its high predisposition to agresive basal cell carcinomas an early diagnosis is vital to the prognosis. This paper aims to present a case of a patient presenting this rare condition.


REFERENCES

  1. Farndon PA, Del Mastro RG, Evans DG, Kilpatrick MW. Location of gene for Gorlin syndrome. Lancet 1992;339:581-2.

  2. Friedrich R. P. 207 Diagnosis and treatment of patients with nevoid basal cell carcinoma syndrome (Gorling-Goltz-Syndrome). J Craniomaxill Surg 2006;34:185-86.

  3. Bale AE. The nevoid basal cell carcinoma syndrome: genetics and mechanism of carcinogenesis. Cancer Invest1997;15:180-6.

  4. Gandage S, Rahalkar M, Domkundwar S. Gorlin's syndrome-radiographic and CT manifestations. Indian J Radiol Imaging 2003;13:19-22.

  5. Kimonis VE, Goldstein AM, Pastakia B, Yang ML, Kase R, Di Giovanna JJ, Bale AE, Bale SJ. Clinical manifestations in 105 persons with nevoid basal cell carcinoma syndrome. AM J Med Genet 1997;69:299-308.

  6. Yi-Fang Z, Jin-Xiong W, Shi-Ping W. Treatment of odontogenic keratocyst: A foolow up of 255 Chinese patients. Oral Maxillofac J 2002;94:151-6.

  7. Reddy J, Laufer MR. Congenital anomalies of the female reproductive tract in a patient with Goltz syndrome. J Pediatr Adolesc Gynecol 2009;22:71-72.

  8. Borgonovo A, Di Lascia S, Grossi G, Maiorana C. Two-stage treatment protocol of keratocyst odontogenic tumor in young patients with Gorlin-Goltz syndrome: marsupialization and later enucleation with peripheral ostectomy. A 5-year-follow-up experience. Int J Pediatr Otorhinol. 2011;75:1565-71.




2020     |     www.medigraphic.com