2008
DOI: 10.1186/1750-1172-3-32
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Nevoid basal cell carcinoma syndrome (Gorlin syndrome)

Abstract: Nevoid basal cell carcinoma syndrome (NBCCS), also known as Gorlin syndrome, is a hereditary condition characterized by a wide range of developmental abnormalities and a predisposition to neoplasms.

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Cited by 421 publications
(580 citation statements)
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References 143 publications
(186 reference statements)
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“…Three major criteria: Histologically proven odontogenic keratocyst of jaw, palmar pits, and calcification of falx cerebri; minor criteria, i.e., skeletal anomalies such as frontal bossing, bifid ribs, mild Multiple odontogenic keratocysts are the first as well as the most consistent and representative sign in this syndrome with an incidence of 75% and mandible being the most common site. [3][4][5][6][7] There may be remarkably few symptoms until cyst reach a large size, especially when in the ascending ramus which supports our case who was asymptomatic at presentation and multiple cystic lesions were noted on routine radiographic examination. Microscopically, they are multilocular with a parakeratinized stratified squamous epithelium most common type (96%) which is identical to our case.…”
Section: Discussionsupporting
confidence: 73%
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“…Three major criteria: Histologically proven odontogenic keratocyst of jaw, palmar pits, and calcification of falx cerebri; minor criteria, i.e., skeletal anomalies such as frontal bossing, bifid ribs, mild Multiple odontogenic keratocysts are the first as well as the most consistent and representative sign in this syndrome with an incidence of 75% and mandible being the most common site. [3][4][5][6][7] There may be remarkably few symptoms until cyst reach a large size, especially when in the ascending ramus which supports our case who was asymptomatic at presentation and multiple cystic lesions were noted on routine radiographic examination. Microscopically, they are multilocular with a parakeratinized stratified squamous epithelium most common type (96%) which is identical to our case.…”
Section: Discussionsupporting
confidence: 73%
“…[2][3][4][5] The age predilection for this condition is in the 1-3 rd decade of life supporting our case who presented to us in the second decade of life. [5] Although in our case, the patient did not present with basal cell carcinoma, there were other major and minor criteria fulfilling the diagnosis of the syndrome. Three major criteria: Histologically proven odontogenic keratocyst of jaw, palmar pits, and calcification of falx cerebri; minor criteria, i.e., skeletal anomalies such as frontal bossing, bifid ribs, mild Multiple odontogenic keratocysts are the first as well as the most consistent and representative sign in this syndrome with an incidence of 75% and mandible being the most common site.…”
Section: Discussionsupporting
confidence: 57%
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“…Mesenteric cysts in NBCCS are thin walled and can range from 2 to 14 cm in diameter. The content of mesenteric cysts is often chylous but can also be haemorrhagic 6. Histological examination of mesenteric cysts demonstrates a lining consisting of fibrous tissue and smooth muscle, often surrounded by a layer of lymphatic cells.…”
mentioning
confidence: 99%