Nevoid Basal-Cell Syndrome: literature review and case report in a family

Abstract

The Nevoid Basal-Cell Carcinoma Syndrome (NBCC), or as it is also referred to, basal-cell nevus syndrome or Gorlin-Goltz syndrome, is characterized by multiple early-appearing basal cell carcinomas, keratocytosis of the mandible, and anomalies of the ocular, skeletal reproductive system. We describe four patients in the same family, all of them possessing a large number of skin tumors associated with other typical clinical and X-Ray anomalies of NBCC. The definitive treatment of NBCC has yet to be established, however, early diagnosis is very important as well as the periodical follow-up examination of ten patients, mainly due to the transformations in the skin lesions that may occur.

Keratocystic odontogenic tumor related to nevoid basal cell carcinoma syndrome: clinicopathological study

“…In addition, we have found only 13 welldocumented patients have been found in English language literature with such association in the Brazilian population in the last 20 years [5][6][7][8][9][10] . The purpose of this paper is to describe clinical and histopathological aspects of KCOTs associated with NBCCS and report other manifestations associated with the syndrome.…”

Keratocystic odontogenic tumors related to Gorlin–Goltz syndrome: A clinicopathological study

“…The abovementioned research involving patients with KCOT related to Gorlin-Goltz syndrome. [5][6][7][8][9][10] Regarding the treatment of KCOT, Zecha et al demonstrated that 58 patients who did not have an NBCCS diagnosis and were treated with enucleation alone had recurrence in 20.7% of the case. 22 Recurrence was observed in 2 tumors which corresponded to 1 patient.…”

Photodynamische Therapie von Lidbasaliomen bei 13-jährigem Patienten mit Gorlin-Goltz-Syndrom

“…Bei 78 % der Patienten werden odontogene keratozystische Tumore vor dem 11. Lebensjahr diagnostiziert [14]. Medulloblastome wurden bei 5 % der Patienten beschrieben [9,19].…”