What is Gorlin's syndrome? The diagnosis and management of the basal cell naevus syndrome, based on a study of thirty-seven patients

Rayner, Colin R.; Towers, J.F.; Wilson, Jeff

doi:10.1016/s0007-1226(77)90037-6

Cited by 79 publications

(21 citation statements)

References 15 publications

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“…Gorlin et al (1965) referred to three cases he had heard of by personal communication. There were no details of these cases and two other cases (Rayner et al, 1977;Woolgar et al, 1987) who were noted to have died of the disease and had relatives with GS, also had no further details. The details of those surviving (Table I) and those cases noted to have died from the disease (Table II) are presented.…”

Section: Literature Reviewmentioning

confidence: 99%

The incidence of Gorlin syndrome in 173 consecutive cases of medulloblastoma

Evans

Farndon²,

Burnell³

et al. 1991

Br J Cancer

272

150

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We have investigated the incidence of Gorlin syndrome (GS) in patients with the childhood brain tumour, medulloblastoma. One hundred and seventy-three consecutive cases of medulloblastoma in the North-West Regional Health Authority between 1954 and 1989 (Manchester Regional Health Board before 1974) were studied. After review of case notes, X-rays and health surveys only 2/173 cases had evidence supporting a diagnosis of GS. A further case at 50% risk of GS died of a brain tumour aged 4 years. The incidence of GS in medulloblastoma is, therefore, probably between 1-2%. A population based study of GS in the region started in 1983 was used to assess the incidence of medulloblastoma in GS, which was found to be between 3-5%. This figure is lower than previous estimates, but this is the first population based study undertaken. In view of the early age of onset in GS (mean 2 years) children presenting with medulloblastoma, especially under 5 years, should be examined for signs of the syndrome. Those at high risk of developing multiple invasive basal cell carcinomata will then be identified.

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Section: Literature Reviewmentioning

confidence: 99%

The incidence of Gorlin syndrome in 173 consecutive cases of medulloblastoma

Evans

Farndon²,

Burnell³

et al. 1991

Br J Cancer

272

150

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“…Other diagnostic findings in adults with nevoid basal cell carcinoma reported by Gorlin et al 13 (1977) and their incidence of occurrences are:…”

Section: Discussionmentioning

confidence: 88%

Gorlin and Goltz Syndrome: A Case Report with Surgical Review

Bahadure

Jain

Badole

2013

International Journal of Clinical Pediatric Dentistry

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Gorlin and Goltz syndrome are a very complex syndrome and a multisystemic process that is characterized by the presence of multiple pigmented basocellular carcinomas, keratocysts in the jaws, palmar and/or plantar pits and calcification of the falx cerebri. Along with these major features a great number minor features have also been described which involves numerous skeletical, dermatology related, neurological, ophthalmological and reproductive anomalies. It exhibits high penetrance and variable expressivity. Presented here is the case of Gorlin-Goltz in a 12 years old male patient which was diagnosed through its oral and maxillofacial manifestations. Treatment of odontogenic keratocyst was done by enucleation without primary suturing. Iodoform dressing was kept to enhance the healing and to reduce the recurrence of the lesion.It is important to provide the early diagnosis for detection of clinical and radiological manifestations in young patients and for provision of advice concerning preventive treatment like protection of the skin from the sunlight and genetic sensitivity testing so that possible complications associated with this syndrome can be prevented.How to cite this article: Bahadure RN, Jain ES, Badole GP. Gorlin and Goltz Syndrome: A Case Report with Surgical Review. Int J Clin Pediatr Dent 2013;6(2):104-108.

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“…KCOT's are usually the first sign and most constant finding of this syndrome. The presence of even one KCOT in a patient younger than 20 years should alert the dentist to the possibility of NBCCS, particularly in case of multiple KCOTs occurring simultaneously or one after the other [3,21,22].…”

Section: Discussionmentioning

confidence: 99%

Nevoid-basal cell carcinoma syndrome: a case report and overview on diagnosis and management

Baliga¹,

Rao²

2010

J. Maxillofac. Oral Surg.

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Nevoid Basal Cell Carcinoma Syndrome (NBCCS) is a rare condition characterized by varied clinical manifestations like multiple Basal Cell Carcinomas (BCC), multiple Keratocystic Odontogenic Tumours (KCOT), palmar and/or plantar pits and ectopic calcification of the falx cerebri, which are considered as the major criteria for diagnosis. The occurrence of jaw manifestations makes it an important diagnostic problem for oral and maxillofacial surgeons and often clinicians encounter this aspect which finally leads to the diagnosis of this syndrome. This paper reports a case of NBCCS and provides an overview on the diagnosis and management of this enigmatic entity.

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What is Gorlin's syndrome? The diagnosis and management of the basal cell naevus syndrome, based on a study of thirty-seven patients

Cited by 79 publications

References 15 publications

The incidence of Gorlin syndrome in 173 consecutive cases of medulloblastoma

The incidence of Gorlin syndrome in 173 consecutive cases of medulloblastoma

Gorlin and Goltz Syndrome: A Case Report with Surgical Review

Nevoid-basal cell carcinoma syndrome: a case report and overview on diagnosis and management

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