Search tips
Search criteria 


Logo of jmedgeneJournal of Medical GeneticsVisit this articleSubmit a manuscriptReceive email alertsContact usBMJ
J Med Genet. 1993 June; 30(6): 460–464.
PMCID: PMC1016416

Complications of the naevoid basal cell carcinoma syndrome: results of a population based study.


There are many potential complications which have been reported in association with the naevoid basal cell carcinoma syndrome. We have been able to show the relative frequencies of these problems in a population based study of 84 cases in the north west of England. The major complications of basal cell carcinomas and jaw cysts occur in over 90% of patients by 40 years of age, but may both occur before 10 years of age. Less well described complications are ovarian calcification or fibroma (24%), medulloblastoma (5%), cardiac fibroma (3%), cleft palate (5%), and ophthalmic abnormalities such as squint or cataract (26%). This study more clearly defines the possible complications of the syndrome and gives clearer guidelines for counselling and screening affected and at risk persons.

Full text

Full text is available as a scanned copy of the original print version. Get a printable copy (PDF file) of the complete article (924K), or click on a page image below to browse page by page. Links to PubMed are also available for Selected References.

Images in this article

Click on the image to see a larger version.

Selected References

These references are in PubMed. This may not be the complete list of references from this article.
  • BINKLEY GW, JOHNSON HH., Jr Epithelioma adenoides cysticum; basal cell nevi, agenesis of the corpus callosum and dental cysts; a clinical and autopsy study. AMA Arch Derm Syphilol. 1951 Jan;63(1):73–84. [PubMed]
  • GORLIN RJ, GOLTZ RW. Multiple nevoid basal-cell epithelioma, jaw cysts and bifid rib. A syndrome. N Engl J Med. 1960 May 5;262:908–912. [PubMed]
  • Farndon PA, Del Mastro RG, Evans DG, Kilpatrick MW. Location of gene for Gorlin syndrome. Lancet. 1992 Mar 7;339(8793):581–582. [PubMed]
  • Gilhuus-Moe O, Haugen LK, Dee PM. The syndrome of multiple cysts of the jaws, basal cell carcinomata and skeletal anomalies. Br J Oral Surg. 1968 Mar;5(3):211–222. [PubMed]
  • Evans DG, Farndon PA, Burnell LD, Gattamaneni HR, Birch JM. The incidence of Gorlin syndrome in 173 consecutive cases of medulloblastoma. Br J Cancer. 1991 Nov;64(5):959–961. [PubMed]
  • Evans DG, Birch JM, Orton CI. Brain tumours and the occurrence of severe invasive basal cell carcinoma in first degree relatives with Gorlin syndrome. Br J Neurosurg. 1991;5(6):643–646. [PubMed]
  • CLENDENNING WE, BLOCK JB, RADDE IG. BASAL CELL NEVUS SYNDROME. Arch Dermatol. 1964 Jul;90:38–53. [PubMed]
  • Nagasawa H, Little FF, Burke MJ, McCone EF, Targovnik HS, Chan GL, Little JB. Study of basal cell nevus syndrome fibroblasts after treatment with DNA-damaging agents. Basic Life Sci. 1984;29(Pt B):775–785. [PubMed]
  • McClatchey K, Batsakis JG, Hybels R, Van Wieren CR. Odontogenic keratocysts and nevoid basal cell carcinoma syndrome. Arch Otolaryngol. 1975 Oct;101(10):613–616. [PubMed]
  • Leppard BJ. Skin cysts in the basal cell naevus syndrome. Clin Exp Dermatol. 1983 Nov;8(6):603–612. [PubMed]
  • CLENDENNING WE, BLOCK JB, RADDE IG. BASAL CELL NEVUS SYNDROME. Arch Dermatol. 1964 Jul;90:38–53. [PubMed]
  • Brannon RB. The odontogenic keratocyst. A clinicopathologic study of 312 cases. Part II. Histologic features. Oral Surg Oral Med Oral Pathol. 1977 Feb;43(2):233–255. [PubMed]
  • Johnson AD, Hebert AA, Esterly NB. Nevoid basal cell carcinoma syndrome: bilateral ovarian fibromas in a 3 1/2-year-old girl. J Am Acad Dermatol. 1986 Feb;14(2 Pt 2):371–374. [PubMed]
  • Strong LC. Genetic and environmental interactions. Cancer. 1977 Oct;40(4 Suppl):1861–1866. [PubMed]
  • Harris SA, Large DM. Gorlin's syndrome with a cardiac lesion and jaw cysts with some unusual histological features. A case report and review of the literature. Int J Oral Surg. 1984 Feb;13(1):59–64. [PubMed]
  • Herman TE, Siegel MJ, McAlister WH. Cardiac tumor in Gorlin syndrome. Nevoid basal cell carcinoma syndrome. Pediatr Radiol. 1991;21(3):234–235. [PubMed]
  • Williams DB, Danielson GK, McGoon DC, Feldt RH, Edwards WD. Cardiac fibroma: long-term survival after excision. J Thorac Cardiovasc Surg. 1982 Aug;84(2):230–236. [PubMed]
  • Bunting PD, Remensnyder JP. Basal cell nevus syndrome. Plast Reconstr Surg. 1977 Dec;60(6):895–901. [PubMed]
  • Littler BO. Gorlin's syndrome and the heart. Br J Oral Surg. 1979 Nov;17(2):135–146. [PubMed]
  • Jones KL, Wolf PL, Jensen P, Dittrich H, Benirschke K, Bloor C. The Gorlin syndrome: a genetically determined disorder associated with cardiac tumor. Am Heart J. 1986 May;111(5):1013–1015. [PubMed]
  • Totten JR. The multiple nevoid basal cell carcinoma syndrome. Report of its occurrence in four generations of a family. Cancer. 1980 Sep 15;46(6):1456–1462. [PubMed]
  • Potaznik D, Steinherz P. Multiple nevoid basal cell carcinoma syndrome and Hodgkin's disease. Cancer. 1984 Jun 15;53(12):2713–2715. [PubMed]

Articles from Journal of Medical Genetics are provided here courtesy of BMJ Publishing Group