Article Menu

Export Article

More by Authors Links

Article Data

  • Views 1215
  • Dowloads 126

Case Reports

Open Access

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

  • M. Giuliani1,*,
  • L. Di Stefano1
  • G. Zoccali1
  • E. Angelone1
  • P. Leocata2
  • G. Mascaretti1

1Department of Surgical Science, Italy

2Department of Anatomopathology, University of L'Aquila, Italy

DOI: 10.12892/ejgo200605519 Vol.27,Issue 5,September 2006 pp.519-522

Published: 10 September 2006

*Corresponding Author(s): M. Giuliani E-mail:

PDF (3.2 MB) View Full-text

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


Cite and Share

M. Giuliani,L. Di Stefano,G. Zoccali,E. Angelone,P. Leocata,G. Mascaretti. Gorlin syndrome associated with basal cell carcinoma of the vulva: A case report. European Journal of Gynaecological Oncology. 2006. 27(5);519-522.

URL:

https://www.ejgo.net/articles/10.12892/ejgo200605519

References

[1] Diaz-Fernandez J.M., Infante-Cossio P.. Belmonte-Caro R., RuizLaza L.. Garcia-Perla-Garcia A., Gutierrez-Perez J.L.: "Basal cell nevus syndrome. Presentation of six cases and literature review". Med. Oral. Patol. Oral. Cir. Bucal., 2005, 10 (suppl. 1), E57.

[2] Gorlin R.J.: "Nevoid basal-cell carcinoma syndrome". Dermatol Clin., 1995, 13, 113.

[3] Holikova Z., Massi D., Lotti T., Hercogova J.: "Insight into the pathogenesis of sporadic basal cell carcinoma". Int..T. Dermatol., 2004, 43, 865.

[4] Allen E.B., Kuan-Ping Y.:'The hedgehog pathway and basal cell carcinomas". Hum. Mot. Gen., 2001, 10, 757.

[5] Grosjean L.D.. Privat S.C.: "Sonic hedgehog signalling in basal cell carcinomas". Cancer Lett., 225 (2205), 181.

[6] Minami M. et al.: "Germline mutation of the PTCH gene in Japanese patients with nevoid basal cell carcinoma syndrome"..T. Dermatol. Sci., 2001, 27, 21.

[7] Manfredi M., Vescovi P., Bonannni M., Porter S.: "Nevoid basal cell carcinoma syndrome: a review of the literature". Int. J. Oral. Maxillofac. Surg., 2004, 33, 117.

[8] Lo Muzio L., Nocini P.F., Savoia A., Consolo U., Procaccini L., Zelante L., et al.: "Nevoid basal cell carcinoma syndrome. Clinical findings in 37 Italian affected individuals". Clin. Genet., 1999, 55, 34.

[9] Lo Muzio L., Nocini P.F., Bucci P., Pannone G.. Cousolo U., Procaccino M.: "Early diagnosis of nevoid basal cell carcinoma syndrome". J. Am. Dent. Assoc., 1999, 130, 669.

[10] Cohen M.M. Jr.: "Nevoid basal cell carcinoma syndrome: molecular biology and new hypotheses". Int. J. Oral. Ma.xilleofac. Surg., 1999, 28, 216.

[11] Evans D.G. et al.: "Complications of the nevoid basal cell carcinoma syndrome: results of a population based study". J. Med Genet., 1993, 30, 460.

[12] de Giorgi Y., Salvini C., Massi D., Raspollini M.R., Carli P "Yul var basal cell carcinoma: retrospective study and review of literature". Gynecol. Oncol., 2005, 97, 192.

Submission Turnaround Time

Top