Purpose: Bladder cancer is a common malignancy, and a frequent cause o
f urological consultation and surgical intervention. Except for smokin
g and certain occupational exposures, the etiology of bladder cancer i
s largely unknown, Although the majority of patients with bladder canc
er do not have a family history of transitional cell carcinoma of the
urinary tract, the study of familial transitional cell carcinoma may l
ead to knowledge of the pathogenesis of this disease. Materials and Me
thods: To evaluate the current understanding of familial transitional
cell carcinoma, we reviewed the contemporary literature for case repor
ts and epidemiological studies about this disease. Results: Numerous c
ase reports document the clustering of transitional cell carcinoma in
families, several of which demonstrate an extremely early age at onset
of disease, which argues in favor of a genetic component to familial
transitional cell carcinoma. The results of large epidemiological stud
ies also suggest the existence of familial transitional cell carcinoma
, and first degree relatives appear to have an increased risk for dise
ase by a factor of 2, Familial clustering of smoking does not appear t
o be the cause of this increased risk. Conclusions: Familial transitio
nal cell carcinoma may be the result of a genetically transmitted pred
isposition to disease, at least in some affected families. Further stu
dies are required to identify candidate genes that may be responsible
for this form of bladder cancer.