Gorlin Goltz sendromu: Bir olgu sunumu

Öz

Gorlin Goltz sendromu esas olarak kutanöz bazal hücreli karsinom, çoklu keratokistik odontojenik tümörler ve iskeletsel anomaliler ile karakterize otozomal dominant bir rahatsızlıktır. Gorlin ve Goltz tarafından tanımlanan bu klasik triada ek olarak diğer nörolojik, oftalmolojik, endokrin ve genital bulgular da sendromun özellikleri arasında yer almaktadır. Bu raporda 12 yaşındaki Gorlin Goltz sendromlu erkek hastanın tedavisi ve 1,5 yıllık takibi sunulmuştur. Bu olgu deride herhangi bir lezyon bulunmayan genç yaştaki hastalarla ilgili olarak diş hekimlerinin farkındalığını arttırmak ve erken teşhisin önemini vurgulamak amacıyla sunulmuştur. Anahtar Kelimeler: Gorlin Goltz, bazal hücreli karsinoma, keratokist.

Anahtar Kelimeler

• Gorlin Goltz, bazal hücreli karsinoma, keratokist

Gorlin Goltz syndrome: A case report

Öz

Gorlin-Goltz syndrome is an autosomal dominant disorder principally characterized by cutaneous basal cell carcinomas, multiple keratocystic odontogenic tumors(KCOTs), and skeletal anomalies. In addition to the classical triad described by Gorlin and Goltz, other neurological, ophthalmic, endocrine, and genital manifestations have also been established as features of the syndrome. In this report a 12-year-old male patient with Gorlin-Goltz syndrome and its teratment is presented with 1.5 year follow up period. The aim of this case report is to increase the awareness of the syndrome among dentists about younger age patients with no lesions of the skin and emphesize the importance of early diagnosis. Keywords: Gorlin Goltz, basal cell carcinoma, keratocyct.

Anahtar Kelimeler

• Gorlin Goltz, basal cell carcinoma, keratocyct

Kaynakça

1. Ortakoğlu K, Köymen R, Karasu HA, Doğan N. Gorlin Goltz sendromu (Bazal hücreli nevus sendromu) (İki olgu nedeniyle). T Klin Diş Hek Bil 2000;6:168-173.
2. Kopp WK, Klatell J, Blade M: Basal cell nevus syndrome with other abnormalities. Oral Surg Oral Med Oral Pathol 1969;27:29.
3. Ellis DJ, Akin RK, Berhard R: Nevoid basal cell carcinoma syndrome. J Oral Surg 1972; 30: 851.
4. Gorlin RJ, Goltz RW. Multiple nevoid basal-cell epithe- lioma, jaw cysts and bifid rib. A syndrome. N Engl J Med 1960;262:908-912.
5. Rayner CR, Towers JF, Wilson JS. What is Gorlin’s syn- drome? The diagnosis and management of the basal cell nae- vus syndrome, based on a study of thirty-seven patients. Br J Plast Surg 1977;30:62-67.
6. Gorlin RJ: Nevoid basal-cell carcinoma syndrome. Medi- cine 1987;66:98.
7. Akdeniz BG, Seçkin T. Nevoid bazal hücreli karsinum sen- dromu: olgu bildirimi. T Klin Diş Hek Bil 2000;6:1-6.
8. Ahlfors E, Larsson A, Sjogren S. The odontogenic ker- atocyst. A benign cystic tumour? J Oral Maxillofac Surg 1984;42:10-19.

9. Kamiya Y, Narita H, Yamamoto T, Kameyama Y, Maeda H, Nakane S. Fanilial odontogenic keratocyst. Int J Oral Surg 1985;14:73-80.
10. Mc Loughlin PM, Dickenson AJ, Avery BS. Gorlin’s Syn- drome and von Recklinghausen’s Disease occuring in one family. Br J Oral Maxillofac Surg 1991;29:189-193.
11. Goaz PW, Stuart CW. Oral Radiology. Principles and In- terpretation. 2nd ed. St Louis: Mosby; 1987;497-498.
12. Timoçin N, Doğan Ö. Gorlin Goltz sendromu. Olgu bildi- risi.İ.Ü. Diş Hek Fak Der 1994;28:53-56.
13. Stoelinga PJW, Peters JH, Staak WJB, Cohen M: Some new findings in the basal cell nevus syndrome. Oral Surg Oral Med Oral Pathol 1973;36:686-692.
14. Lovin JD, Talario CL, Wegret SL, Gaynor LF, Sutley SS: Gorlin’s syndrome with associated odontogenic cysts. Pediatr Radiol 1991;21:584-587.
15. Hodgkinson DJ, Woods JE, Dahlin DC, Tolman DE: Ker- atocysts of the jaw. Clinicopathologic study of 79 cases. Can- cer 1978;41:803-813.
16. Flyn TR, Lieblich SE, Topazian RG: Odontogenic cyst and tumors. In: Keith DA: Atlas of Oral and Maxillofacial Sur- gery Philadelphia:WB Saunders Co;1992;87.