Familial uveal melanoma, III. Is the occurrence of familial uveal melanoma coincidental?
A. D. Singh, M. X. Wang, L. A. Donoso, C. L. Shields, P. D. Potter, J. A. Shields, R. C. Elston and B. Fijal
Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pa, USA.
OBJECTIVE: To ascertain whether the familial occurrence of uveal melanoma
was coincidental in kindreds in which 1 first-degree relative of the
proband had also been affected with primary uveal melanoma. PATIENTS: In a
series of 4500 patients with primary uveal melanoma, 17 kindreds were
identified in which a first-degree relative of the proband had also been
affected with primary uveal melanoma. DESIGN: In the 17 families in which a
first-degree relative of the proband had been affected, primary uveal
melanoma was classified as familial. In the remaining 4483 families,
primary uveal melanoma was classified as sporadic. The expected number of
affected first-degree relatives of probands for a family was estimated,
assuming an incidence rate of 6 cases per million population per year in
each type of family. RESULTS: The expected number of affected first-degree
relatives was calculated to be 0.81, with an SE of 0.08, compared with 17
observed affected first-degree relatives (P < .001). CONCLUSION: Our
study provides strong statistical evidence that occurrence of familial
uveal melanoma is not coincidental.
