IMR Press / EJGO / Volume 27 / Issue 5 / pii/2006226

European Journal of Gynaecological Oncology (EJGO) is published by IMR Press from Volume 40 Issue 1 (2019). Previous articles were published by another publisher on a subscription basis, and they are hosted by IMR Press on imrpress.com as a courtesy and upon agreement with S.O.G.

Open Access Case Report

Gorlin syndrome associated with basal cell carcinoma of the vulva: A case report

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1 Department of Surgical Science, University of L’Aquila, Italy
2 Department of Anatomopathology, University of L’Aquila, Italy
Eur. J. Gynaecol. Oncol. 2006, 27(5), 519–522;
Published: 10 October 2006
Abstract

Gorlin syndrome, also known as nevoid basal cell carcinoma syndrome (NBCCS), is a hereditary condition transmitted as an autosomal dominant trait with high penetrance and variable expressivity. The syndrome is characterized by numerous manifesta­tions: basal cell carcinomas (BCCs) and odontogenic keratocysts (OKCs) are the leading ones. In this article a typical Gorlin syndrome case associated with basal cell carcinoma of the vulva is described.

Keywords
Gorlin syndrome
NBCCS
PTCH gene
Basal cell carcinoma
Odontogenic keratocyst
Carcinoma of the vulva
Lym­phadenectomy
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