Evidence of chromosomal instability in the lymphocytes of Gorlin basal-cell carcinoma patients

Mutat Res. 1995 Nov;332(1-2):27-32. doi: 10.1016/0027-5107(95)00082-0.

Abstract

The Gorlin syndrome, or naevoid basal-cell carcinoma syndrome (NBCS) is an autosomal dominant disease. It has been suspected for long that this cancer prone disease (multiple basal-cell carcinomas; other malignant or benign proliferations) is a chromosome instability syndrome. We previously reported a lengthening in the cell cycle of lymphocytes from two patients with NBCS. With a larger sample (n = 7), we confirm this disease to be a chromosome instability syndrome, although clearly, expression of this characteristic can vary between patients: (1) spontaneous chromatid breaks occurred more often in a subset of the patients; (2) spontaneous micronuclei were found more frequently in NBCS than in the controls; (3) we confirm the cell cycle to be affected in this disease. As these results were obtained on lymphocytes--a cell lineage not affected in NBCS manifestations--the chromosome instability we found would appear to be part of the general condition of this syndrome.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Basal Cell Nevus Syndrome / genetics*
  • Basal Cell Nevus Syndrome / pathology
  • Cell Cycle / genetics
  • Chromosome Aberrations*
  • Female
  • Humans
  • Lymphocytes / pathology*
  • Lymphocytes / ultrastructure
  • Male
  • Micronucleus Tests
  • Middle Aged
  • Sister Chromatid Exchange*