scholarly journals Gorlin-Goltz syndrome: systemic and maxillofacial characteristics

2020 ◽  
Vol 10 (1) ◽  
pp. 49-54
Author(s):  
Jefferson David Melo de Matos ◽  
Leonardo Jiro Nomura Nakano ◽  
Pedro Jacy Santos Diamantino ◽  
Guilherme da Rocha Scalzer Lopes ◽  
Marco Antonio Bottino ◽  
...  
Keyword(s):  
Case Report ◽  
Basal Cell Carcinoma ◽  
Cell Carcinoma ◽  
Basal Cell ◽  
Genetic Disorder ◽  
Suppressor Gene ◽  
Skeletal Malformations ◽  
Goltz Syndrome ◽  
Basal Cell Carcinomas ◽  
Endocrine Alterations

Gorlin-Goltz Syndrome, also known as Nevoid Basal Cell Carcinoma Syndrome, is a rare genetic disorder characterized by the presence of multiple keratocysts in the jaw and basal cell carcinomas, at young age, of palmar and/or plantar depressions, of calcification of the sickle cerebral and skeletal malformations. This syndrome is caused by a mutation of the PTCH1 (patched homolog 1 from Drosophila) gene, a tumor suppressor gene. In this work, the systemic and maxillofacial characteristics of the Gorlin-Goltz syndrome, as well as some neurological, dermatological, musculoskeletal and endocrine alterations, are reviewed. In addition, a case report was added for the purpose of support this study.

Confocal and dermoscopic features of basal cell carcinoma in Gorlin-Goltz syndrome: A case report

2016 ◽  
Vol 58 (2) ◽  
pp. e48-e50 ◽  
Author(s):  
Alice Casari ◽  
Giuseppe Argenziano ◽  
Elvira Moscarella ◽  
Aimilios Lallas ◽  
Caterina Longo
Keyword(s):  
Case Report ◽  
Basal Cell Carcinoma ◽  
Cell Carcinoma ◽  
Basal Cell ◽  
Goltz Syndrome

scholarly journals Basal Cell Carcinoma of the Penis: A Case Report and Review of the Literature

2014 ◽  
Vol 2014 ◽  
pp. 1-3 ◽  
Author(s):  
R. J. Roewe ◽  
Matthew A. Uhlman ◽  
Nathan A. Bockholt ◽  
Amit Gupta
Keyword(s):  
Case Report ◽  
Basal Cell Carcinoma ◽  
Cell Carcinoma ◽  
Local Excision ◽  
Basal Cell ◽  
Wide Local Excision ◽  
Rare Entity ◽  
Review Of The Literature ◽  
Basal Cell Carcinomas

Basal cell carcinoma of the penis is an extremely rare entity, accounting for less than 0.03% of all basal cell carcinomas. Fortunately, wide local excision of such lesions is generally curative. Fewer than 25 cases have been reported in the literature describing penile basal cell carcinoma. Here we report a case of penile basal cell carcinoma cured with wide local excision.

scholarly journals Locally advanced basal cell carcinoma of the auricle and parotid region: A case report

2021 ◽  
Vol 68 (2) ◽  
pp. 94-98
Author(s):  
Srdjan Milanovic ◽  
Suzana Stojanovic-Rundic ◽  
Nikola Milosevic ◽  
Branko Dozic ◽  
Marko Dozic
Keyword(s):  
Case Report ◽  
Basal Cell Carcinoma ◽  
Cell Carcinoma ◽  
Basal Cell ◽  
Multidisciplinary Approach ◽  
Locally Advanced ◽  
Parotid Region ◽  
Basal Cell Carcinomas ◽  
The Right ◽  
Advanced Basal Cell Carcinoma

Skin cancers are the most common malignant tumors in general. The most significant risk factor is exposure to UV radiation. They mainly occur in the head and neck region, and the majority of about 80% are basal cell carcinomas. Surgery is standard treatment of uncomplicated basal cell carcinomas, but a multidisciplinary approach is necessary in advanced cases. The case report refers to a patient with locally advanced recurrent basal cell carcinoma with primary tumor localization in the right auricle and parotid region in 2012 when primary surgery was performed. Due to the local recurrence, amputation of the right auricle and trepanation of the mastoid process was done in November 2018, and after that, radiation therapy of a recurrent tumor in the area of the trepanation cavity was applied. In the course of follow-up so far, there is good local control, without signs of toxicity. The case report points to the importance of a multidisciplinary approach and the role of radiotherapy in the treatment and control of advanced basal cell carcinoma of this region.

Basal cell carcinoma of the external auditory canal and Gorlin-Goltz syndrome: a case report

1997 ◽  
Vol 111 (9) ◽  
pp. 850-851 ◽  
Author(s):  
C. J. Lobo ◽  
M. S. Timms ◽  
V. C. Puranik
Keyword(s):  
Case Report ◽  
Basal Cell Carcinoma ◽  
Cell Carcinoma ◽  
Basal Cell ◽  
External Auditory Canal ◽  
Goltz Syndrome

AbstractA case of nevoid basal cell carcinoma syndrome (Gorlin-Goltz syndrome) with basal cell carcinoma of the external auditory canal is reported. This is only the second such case.

scholarly journals Gorlin-Goltz Syndrome (Nevoid Basal Cell Carcinoma Syndrome)-A Case Report

2016 ◽  
Vol 9 (2) ◽  
pp. 853-857 ◽  
Author(s):  
Balachander Balachander ◽  
L. Malathi ◽  
J. Jacobina ◽  
N. Babu
Keyword(s):  
Case Report ◽  
Basal Cell Carcinoma ◽  
Cell Carcinoma ◽  
Basal Cell ◽  
Goltz Syndrome

A Case Report of Complicated Multiple Facial Basal Cell Carcinoma Treatment in a Young Woman

2011 ◽  
Vol 11 (1) ◽  
pp. 156-158
Author(s):  
Aleksandrs Derjabo ◽  
Ingrida Cema ◽  
Sergejs Isajevs ◽  
Simona Donina
Keyword(s):  
Case Report ◽  
Basal Cell Carcinoma ◽  
Cell Carcinoma ◽  
Young Woman ◽  
Basal Cell ◽  
Disease Free Survival ◽  
University Hospital ◽  
Free Survival ◽  
Basal Cell Carcinomas ◽  
Oncology Centre

A Case Report of Complicated Multiple Facial Basal Cell Carcinoma Treatment in a Young Woman A 36-year-old woman was consulted in Riga Eastern Clinical University Hospital Latvian Oncology centre Outpatient Department with multiple histology-proven basal cell carcinomas. The patient was treated with diode laser 980 nm and immunotherapy and had 5 years disease free survival.

Basal Cell Carcinoma of the Nail Unit: A Case Report

2001 ◽  
Vol 22 (8) ◽  
pp. 675-678 ◽  
Author(s):  
Roman C. Orsini ◽  
Alan Catanzariti ◽  
Karl Saltrick ◽  
Robert W Mendicino ◽  
Lawrence Stokar
Keyword(s):  
Case Report ◽  
Basal Cell Carcinoma ◽  
Cell Carcinoma ◽  
Basal Cell ◽  
English Literature ◽  
Mohs Micrographic Surgery ◽  
Micrographic Surgery ◽  
Nail Bed ◽  
Basal Cell Carcinomas

Basal cell carcinomas (BCC) of the foot are rare. A review of the English literature found only 23 cases of BCC reported in the foot, none of which involved the nail unit. The nail unit, which is composed of the nail bed and nail-folds, is an exceedingly atypical site for basal cell carcinomas. A case of BCC of the proximal nail fold of the hallux which was treated with Mohs Micrographic Surgery (MMS) is presented. 16 – 21

scholarly journals Nevoid Basal Cell Carcinoma Syndrome (Gorlin Syndrome) in Children

2021 ◽  
Vol 3 (5) ◽  
pp. 15-17
Author(s):  
S. Binsheikhan ◽  
S. Mittal ◽  
M. Al Abadie
Keyword(s):  
Basal Cell Carcinoma ◽  
Cell Carcinoma ◽  
Basal Cell ◽  
Genetic Disorder ◽  
Female Child ◽  
Treatment Modalities ◽  
Gorlin Syndrome ◽  
Cutaneous Manifestations ◽  
Basal Cell Carcinomas ◽  
Multiple Basal Cell Carcinomas

Introduction: Gorlin syndrome or nevoid basal cell carcinoma syndrome (NBCCS) is a rare genetic disorder characterised by development of multiple basal cell carcinomas (BCC) at a young age. Case report: A 7 year female child presented with MULTIPLE skin growths on the neck, face and upper chest for 3 years, with prominent forehead and mild non-scarring alopecia. She also had a history of medulloblastoma treated 3 years ago. There was no significant family history. Biopsy from one of the lesions showed basal cell carcinoma (BCC). Discussion: Nevoid basal cell carcinoma syndrome (NBCCS) is an autosomal dominantly inherited disorder caused by mutations in the tumour suppressor patched 1 (PTCH-1) gene. Patients present with both cutaneous and extra-cutaneous manifestations. Multiple basal cell carcinomas (BCCs) are one of the most frequent cutaneous manifestations, occurring on both photo-exposed and non-exposed areas. The commonest extra-cutaneous tumours are medulloblastomas, which are often the first presentation of the disease. There are multiple but no established treatment modalities for the disease.

scholarly journals O diagnostico da síndrome do carcinoma nevoide basocelular (Gorlim-Goltz)

2020 ◽  
Vol 9 (3) ◽  
Author(s):  
Maylson Alves Nogueira Barros ◽  
Vitor Bruno Teslenco ◽  
Guilherme Nucci dos Reis ◽  
Arnobio Luiz Nunes ◽  
Everton Floriano Pancini ◽  
...  
Keyword(s):  
Case Report ◽  
Basal Cell Carcinoma ◽  
Cell Carcinoma ◽  
Basal Cell ◽  
Clinical Manifestations ◽  
Odontogenic Tumours ◽  
Goltz Syndrome ◽  
Ciclo Celular ◽  
A Prospective Study ◽  
Head Neck

Introdução: A síndrome de Gorlin-Goltz é compreendida como uma condição hereditária autossômica dominante, causada por uma alteração no gene patched (PTCH), localizado no cromossomo 9q22.3-q31, este gene regula as funções de supressão tumoral, organização embrionária e ciclo celular, as mutações no gene PTCH levam a o efeito da perda de controle de vários outros genes responsaveis pela organogênese, carcinogênese e odontogênese. Objetivo: O presente trabalho tem como objetivo descrever um caso onde o paciente foi diagnosticado com a Síndrome do carcinoma nevoide basocelular. Relato de caso: Paciente, feoderma, 17 anos, sexo masculino, foi encaminhando ao serviço de Cirurgia e Traumatologia Bucomaxilofacial, com queixa de aumento de volume na região de maxila e mandíbula à direta, associado a quadros de dores. Durante a anamnese o paciente negou doenças ou comorbidades prévias. Ao exame físico, apresentou assimetria facial, com tumefação em hemiface direita, discreto hipertelorismo, bossa frontal acentuada, múltiplas máculas na região torax e membro superior direito. O exame tomográfico de face evidenciavam-se múltiplas imagens hipodensas  em todos os quadrantes da maxila e mandibula. Com base nos critérios diagnósticos o paciente foi diagnostico com a síndrome de Gorlin-Goltz. Após o fechamento do diagnóstico optou-se pela descompressão das lesões maiores e enucleação das menores. Conclusão: O diagnostico clinico para a síndrome de Gorlin-Goltz baseado nos critérios maiores e menores se mostrou eficaz para definição do caso apresentado.Descritores: Diagnostico; Síndrome do Nevo Basocelular; Patologia.ReferênciasNeville BW, Allen CM, Damm DD, Bouquot JE. Patologia: Oral e Maxilofacial. 4.ed. Rio de Janeiro: Guanabara Koogan; 2016. p. 654-63Ribeiro PL, Souza Filho JB, Abreu KD, Brezinscki MS, Pignaton CC. Syndrome in question: Gorlin-Goltz syndrome. An Bras Dermatol. 2016;91(4):541-43.Bresler SC, Padwa BL, Granter SR. Nevoid Basal Cell Carcinoma Syndrome (Gorlin Syndrome). Head Neck Pathol. 2016;10(2):119-24.Ramesh M, Krishnan R, Chalakkal P, Paul G. Gorlin-Goltz Syndrome: Case report and literature review. J Oral Maxillofac Pathol. 2015;19(2):267.Evans DG, Sims DG, Donnai D. Family implications of neonatal Gorlin's syndrome. Arch Dis Child. 1991;66(10 Spec No):1162-63.Kimonis VE, Goldstein AM, Pastakia B, Yang ML, Kase R, DiGiovanna JJ et al. Clinical manifestations in 105 persons with nevoid basal cell carcinoma syndrome. Am J Med Genet 1997;69:299-308.Seo DU, Kim SG, Oh JS, You JS. Treatment of nevoid basal cell carcinoma syndrome: a case report. J Korean Assoc Oral Maxillofac Surg. 2016;42(5):284-87.Gupta A, Bansal P, Sharma R, Sharma SD. Treatment of Keratocystic Odontogenic Tumours: A Prospective Study of 30 Cases. J Maxillofac Oral Surg. 2016;15(4):521-27.Park JH, Kwak EJ, You KS, Jung YS, Jung HD. Volume change pattern of decompression of mandibular odontogenic keratocyst. Maxillofac Plast Reconstr Surg. 2019;41(1):2.Vieira EC, Abbade LPF, Marques SA, Marques MEA, Stolf HO. Síndrome de Gorlin. Diagn Tratamento. 2012;17(3):110-14.

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