Hum. Genet. 51, 73--78 (1979)
© by Springer-Vedag 1979
Absence of Chromosome Breakage
in Patients with Retinoblastoma
Louise A. Knight 1, H. Allen Gardner l* , and Brenda L. Gallie 2
1 Division of Cytogenetics, Department of Pathology, Toronto General Hospital and
The University of Toronto, Toronto, Canada
2Department of Ophthalmology, Wellesley Hospital, and the University of Toronto, Toronto,
Canada
Summary. Mixed lymphocyte cultures were employed to assess the degree of
spontaneous chromosome fragility in patients with retinoblastoma. There was
no difference between the patients and their controls. If chromosome in-
stability plays a role in the inherited tumour, more sensitive methods need be
employed to elucidate it.
Introduction
A significantly elevated frequency of aneuploidy, chromatid-type aberrations,
and stable abnormalities of the chromosomes has been reported by Czeizel et al.
(1974) in 12 children suffering from retinoblastoma. The authors interpreted
these results as reflecting increased fragility of the patient's somatic chromosomes
and pointed out that chromosome instability is also present in patients with
Bloom's Syndrome, Fanconi's anaemia, Louis-Bar Syndrome (ataxia telangiec-
tasia) and xeroderma pigmentosum (German, 1972), all of which cary an
increased risk of developing malignant disease. Although the cytogenetic instabil-
ity of these four syndromes has been confirmed in many laboratories, that
associated with retinoblastoma has not.
More recently, Wiechselbaum et al. (1978) showed that skin fibroblasts from 6
patients with hereditary retinoblastoma appear to be more sensitive to the effects
of X-rays than do fibroblasts from patients with sporadic retinoblastoma or
normal controls. The sensitivity was intermediate between that observed in ataxia
telangiectasia and the normal control.
In order to elucidate more fully the role of chromosome fragility, 12 patients
with retinoblastoma were studied.
* To whom offprint requests should be sent
0340-6717/79/0051/0073/$ 01.20