Nevoid basal cell carcinoma: Case report

Zana Agani,Aida Namani,Jehona Ahmedi,Arben Murtezani,Mergime Prekazi Loxha,Vjosa Hamiti Krasniqi

doi:10.4236/ojst.2014.41006

Abstract

Nevoid basal cell carcinoma, known as Gorlin Goltz Syndrome, is a rare hereditary condition, characterized by a wide range of developmental abnormalities and a predisposition to neoplasms. In this report, we discuss a case of a patient with Gorlin Goltz Syndrome, who was 16 years old when first admitted for an initial appointment. The patient was diagnosed, treated and followed up for 7 years to present day. This syndrome is associated with a broad spectrum of anomalies and neoplasms as basal cell carcinomas, odontogenic keratocysts, palmar and/or plantar pits, and ectopic calcifications of the falx cerebri. It affects multiple organ systems, which include skeletal, teeth, jaw, skin, eyes, reproductive organs, and neural system. All the features however, are rarely observed in a single patient. The following paper presents the significance of early diagnosis of Gorlin Goltz Syndrome and the importance of a multidisciplinary approach in providing proper treatment for the patient.

Highlights

Gorlin-Goltz syndrome is a hereditary condition which is transmitted as an autosomal dominant trait and characterized with a defect or mutation in 9th chromosome [1]
We discuss a case of a patient with Gorlin Goltz Syndrome, who was 16 years old when first admitted for an initial appointment
There are different synonyms for this condition known as nevoid basal cell carcinoma syndrome, Gorlin Syndrome, Gorlin Goltz syndrome, and basal cell nevus syndrome fifth pacomatosis [3]

Summary

Introduction

Gorlin-Goltz syndrome is a hereditary condition (disorder) which is transmitted as an autosomal dominant trait and characterized with a defect or mutation in 9th chromosome [1]. Jarich and White made the first description of patients with this syndrome in 1894, by highlighting the presence of multiple basocellular carcinomas. Decades later in 1960, Gorlin and Goltz established a classic-. Al triad with multiple basocellular carcinomas, keratocyst of the jaw and bifid ribs [2]. There are different synonyms for this condition known as nevoid basal cell carcinoma syndrome, Gorlin Syndrome, Gorlin Goltz syndrome, and basal cell nevus syndrome fifth pacomatosis [3]. The estimated prevalence varies from 1/57,000 to 1/256,000 among a large number of studies, with a male-to-female ratio of 1:1 [4]. The differential diagnosis should be done with Bazex syndrome, Trichoepitelioma papulosum multiplex and Torre’s syndrome [3]

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