ISSN 1306-0015 | E-ISSN 1308-6278
Case Report
Gorlin-Goltz syndrome
1 Department of Dermatological and Veneral Diseases, Abant İzzet Baysal University Faculty of Medicine, Bolu, Turkey  
2 Department of Otolaryngology, Abant İzzet Baysal University Faculty of Medicine, Bolu, Turkey  
3 Department of Radiology, Abant İzzet Baysal University Faculty of Medicine, Bolu, Turkey  
4 Department of Pathology, Abant İzzet Baysal University Faculty of Medicine, Bolu, Turkey  
5 Department of Oral and Maxillofacial Radiology, Abant İzzet Baysal University Faculty of Dentristry, Bolu, Turkey  
Turk Pediatri Ars 2017; 52: 173-177
DOI: 10.5152/TurkPediatriArs.2017.2992
Key Words: Basal cell carcinoma, Gorlin-Goltz syndrome, medulloblastoma, retinoblastoma
Abstract

Gorlin-Goltz syndrome is a rare multisystemic disease inherited in an autosomal dominant pattern. It is characterized by numerous basal cell carcinoma of the skin, jaw cysts, and skeletal anomalies such as frontal bossing, vertebral anomalies, palmoplantar pits, and falx cerebri calcification. There is a tendency to tumors including medullablastoma, fibroma, rabdomyoma, leiomyosarcoma etc.. The diagnosis is based on major and minor clinical and radiologic criteria. Early diagnosis and treatment are of utmost importance in reducing the severity of long-term sequelae of this syndrome. In this article, we present a 15-year-old boy who was admitted to our clinic with brown-black papules and plaques on his scalp and was thought to have Gorlin-Goltz syndrome. He had a history of medulloblastoma that was treated with surgical resection followed by cranial radiotherapy and unilateral retinoblastoma.  We present this case, because association of Gorlin-Goltz syndrome and retinoblastoma has not been described previously in the literature and we aimed to draw attention to radiation-induced basal cell carcinomas.

 

 

Cite this article as: Şereflican B, Tuman B, Şereflican M, Halıcıoğlu S, Özyalvaçlı G, Bayrak S. Gorlin- Goltz syndrome. Turk Pediatri Ars 2017; 52: 173-7.

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