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Background: The Gorlin–Goltz syndrome (the Nevoid basal cell carcinoma syndrome (NBCCS)) is reviewed as a rare autosomal dominant syndrome caused by gene mutations located on chromosome arm 9 q. It has a variety of presentations and is characterized by basal cell carcinomas (BCC), odontogenic keratocysts (OKCs), and ectopic calcifications of the falx cerebri or plantar pits. The diagnosis depends mainly on a number of minor and major criteria. This syndrome can be misdiagnosed, which makes its early detection important in preventing consensual complications. Case presentation: In our case a 50-year-old male was reported and presented with one minor and four major criteria of the Gorlin–Goltz syndrome, the first presentation was pathologically proven facial basosquamous carcinoma post-surgical excision, subsequent contrast-enhanced maxillofacial MRI as well as CT Head, Neck revealed OKCs of the maxillary and mandible as well as calcifications of the falx and vertebral fusion with palmar pits on physical examination with definite diagnosis of Gorlin–Goltz syndrome. Conclusion: Gorlin–Goltz syndrome is an unusual inherited multi-systemic syndrome, which may be misdiagnosed. It is crucial that health care specialists have the necessary knowledge of the fundamental features of NBCC syndrome and the determination of its associated disorders. All patients should undergo genetic screening as well as their first-degree relatives. Morbidity can be avoided by early treatment of BCC and OKCs to avoid deformities of surrounding dermal and osseous structures. [ABSTRACT FROM AUTHOR]

Abstract Background The Gorlin–Goltz syndrome (the Nevoid basal cell carcinoma syndrome (NBCCS)) is reviewed as a rare autosomal dominant syndrome caused by gene mutations located on chromosome arm 9 q. It has a variety of presentations and is characterized by basal cell carcinomas (BCC), odontogenic keratocysts (OKCs), and ectopic calcifications of the falx cerebri or plantar pits. The diagnosis depends mainly on a number of minor and major criteria. This syndrome can be misdiagnosed, which makes its early detection important in preventing consensual complications. Case presentation In our case a 50-year-old male was reported and presented with one minor and four major criteria of the Gorlin–Goltz syndrome, the first presentation was pathologically proven facial basosquamous carcinoma post-surgical excision, subsequent contrast-enhanced maxillofacial MRI as well as CT Head, Neck revealed OKCs of the maxillary and mandible as well as calcifications of the falx and vertebral fusion with palmar pits on physical examination with definite diagnosis of Gorlin–Goltz syndrome. Conclusion Gorlin–Goltz syndrome is an unusual inherited multi-systemic syndrome, which may be misdiagnosed. It is crucial that health care specialists have the necessary knowledge of the fundamental features of NBCC syndrome and the determination of its associated disorders. All patients should undergo genetic screening as well as their first-degree relatives. Morbidity can be avoided by early treatment of BCC and OKCs to avoid deformities of surrounding dermal and osseous structures.

Gorlin-Goltz syndrome encompasses a variety of clinical signs and symptoms, including significant oral manifestations of which dentists should be aware. Given the risk of malignancy, it is important to be aware of this syndrome and recognize the need for early referral to multidisciplinary management. This article aims to discuss the wide range of clinical manifestations, diagnosis and management of Gorlin-Goltz syndrome through a clinical case.

Gorlin-Goltz syndrome (GGS) is a hereditary condition; it is transmitted as an autosomal dominant trait and characterized by a wide range of developmental abnormalities and a tendency to neoplasm. This article includes a case report and an extensive review of the GGS with regard to its history, incidence, etiology, features, investigations, diagnostic criteria, and treatment modalities.

Nevoid basal cell carcinoma syndrome (NBCCS) or Gorlin syndrome is a multi system disease exhibiting varied developmental abnormalities and a predisposition to neoplasms.A dentist may play a key role in early diagnosis of this disorder considering multiple & recurrent Odntogenic Keratocysts (OKC) are one of the first manifestations and are evident on routine X-rays.Present case report presents a patient diagnosed as NBCCS by clinical, radiographic and histological findings in a 13-year-old patient. We highlights the importance of early diagnosis of NBCCS which can help in preventive multidisciplinary approach to provide a better prognosis for the patient.

A bifid rib is a congenital anomaly of the thoracic wall, being presented most commonly unilateral with a higher incidence among male individuals. We present a case of a 4th right rib, unearthed during an educational study of the donated cadavers in the Anatomy Department of the Democritus University of Thrace, Greece. We have studied 251 skeletons, resulting at an incidence of only 0.4%.

Gorlin–Goltz syndrome, also known as nevoid basal cell carcinoma syndrome, is a genetic disorder with several neurological, cutaneous and skeletal manifestations. Epilepsy has been previously reported as a finding in Gorlin–Goltz syndrome but remains ill-described in the context of this disease. We report two new patients with Gorlin–Goltz syndrome featuring epilepsy and review the existing literature on the topic.

The inherent proliferative capacity of the cystic lining of odontogenic keratocysts and tendency to recur with enucleation led to more aggressive management being widely recommended. Larger lesions preclude the possibility of resection. A case is reported in which extensive odontogenic keratocysts involve almost the entire mandibular medulla of a patient with basal cell nevus syndrome, illustrating the importance of customising treatment to accommodate the clinical and radiographic presentation of individual cases. The clinical outcome of using Carnoy's solution versus marsupialization is radiographically demonstrated in this case. Diagnostic criteria for basal cell nevus syndrome and treatment modalities of odontogenic keratocysts are discussed. [ABSTRACT FROM AUTHOR]

The Gorlin-Goltz syndrome is an infrequent multisystemic disease, which is inherited in an autosomal dominant manner. This shows a high level of penetrance and variable expressiveness, characterized by multiple basal cell nevi or carcinomas, odontogenic keratocysts, palmar and / or plantar pits, calcification of the falx cerebri, and is occasionally associated with internal malignancies. It is fundamental to know the major and minor criteria for the diagnosis and early preventive treatment of this syndrome. Here we report a case of a 30-year-old male with major and minor features of the Gorlin-Goltz syndrome, such as, strabismus, barrel-shaped chest, with drooping shoulders and mild kyphosis, polydactyly, hypertelorism, multiple basal cell carcinomas, calcification of the falx cerebri, C5-C7 bifida spine, and fusion of T1 and T2.

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The nevoid basal cell carcinoma syndrome (NBCCS) or Gorlin - Goltz syndrome is an autosomal disorder principally characterized by cutaneous basal cell carcinomas, multiple keratocysts, and skeletal anomalies. The major organ systems involved are skin, bones, central nervous system, eyes, gonads and endocrine. This particular syndrome is extensively described in the literature under different names. However, there are only few cases reported in the Indian literature. An unusual case of a 33-year old male with large odontogenic keratocyst involving impacted canine in the mandible, along with multiple cysts and impacted teeth in the maxilla; bifid rib and vertebral anomalies has been described.

Gorlin-Goltz syndrome is a rare autosomal-dominant syndrome related to mutation in " Patched" tumour suppressor gene on chromosome 9. Basocellular carcinomas, odontogenic keratocysts, palmar and/or plantar pits and ectopic calcifications of the falx cerebri are its major features, along with more than 100 minor features. Odontogenic cysts, notorious for recurrence, can make endotracheal intubation difficult, requiring modification of the standard intubation technique. We report such a case managed successfully by awake fibreoptic intubation. Direct laryngoscopy under anaesthesia later confirmed that it was a good decision.

Keratocystic odontogenic tumor (KCOT) is of particular interest because its recurrence rate is high and its behavior is aggressive. Nevoid basal cell carcinoma syndrome (NBCCS), which is also known as Gorlin syndrome, is a hereditary condition characterized by a wide range of developmental abnormalities and with a predisposition to neoplasms. These multiple KCOTs have warranted an aggressive treatment at the earliest because of the damage and possible complications. Recurrence of these lesions is a characteristic feature that has to be considered while explaining the prognosis to the patient. Here, we report a case of a 14-year-old boy with clinical features of basal cell nevus syndrome and multiple KCOTs. In addition to the other common features, congenitally missing third molars in all the four quadrants is a feature which has not been previously reported in association with NBCCS in Indian patients. [ABSTRACT FROM AUTHOR]

Gorlin and Goltz described a syndrome in which multiple basal cell carcinomas, odontogenic keratocysts and bifid ribs occurred in combination. The jaw keratocysts are a consistent feature of 'Gorlin-Goltz' or naevoid basal cell carcinoma syndrome. Central nervous system and ocular involvement occurred together with the fairly typical facial features of frontal bossing and hypertelorism. This case report documents the pathology associated with an impacted maxillary canine tooth in a boy with Gorlin-Goltz syndrome. The patient presented for investigation of the failure of eruption of the right permanent maxillary canine tooth. Radiographic investigation showed the presence of a well circumscribed radiolucency located around the crown of an impacted right maxillary canine tooth. The patient's medical history revealed a medulloblastoma that was treated 13 years ago. The right maxillary canine tooth and associated peri-coronal tissue were removed under general anaesthetic. A diagnosis of a keratocystic odontogenic tumour with an associated adenomatoid odontogenic tumour was made. The common differential diagnoses for a peri-coronal radiolucency in the maxilla that need to be considered by dentists include a dentigerous cyst, follicular keratocystic odontogenic tumour and adenomatoid odontogenic tumour. A rare case of both keratocystic odontogenic tumour and associated follicular adenomatoid odontogenic tumour is described in a patient with naevoid basal cell carcinoma syndrome. [ABSTRACT FROM AUTHOR]

Copyright of Revista Española de Cirugía Oral y Maxilofacial is the property of Sociedad Espanola de Cirugia Oral y Maxilofacial (SECOM) and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Gorlin-Goltz syndrome is a rare autosomal-dominant syndrome related to mutation in "Patched" tumour suppressor gene on chromosome 9. Basocellular carcinomas, odontogenic keratocysts, palmar and/or plantar pits and ectopic calcifications of the falx cerebri are its major features, along with more than 100 minor features. Odontogenic cysts, notorious for recurrence, can make endotracheal intubation difficult, requiring modification of the standard intubation technique. We report such a case managed successfully by awake fibreoptic intubation. Direct laryngoscopy under anaesthesia later confirmed that it was a good decision. [ABSTRACT FROM AUTHOR]