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Purpose: Nonurothelial malignancies represent a small fraction of bladder malignancies and are less extensively studied, resulting in sparse empirical data on these tumors. We sought insight into tumor characteristics and survival. Materials and Methods: Data were obtained from the nationwide Netherlands Cancer Registry on patient and tumor characteristics, and followup in all patients with primary invasive (T1 or greater) bladder tumors in The Netherlands between 1995 and 2006. Data were analyzed using frequency tables. Relative survival analysis was done. Results: We identified 28,807 patients with invasive bladder cancer, of whom 7.7% presented with nonurothelial carcinoma. Mean patient age range at diagnosis of adenocarcinoma and soft tissue tumors was 66.4 years, and 78.3 years at diagnosis of nonspecified tumors. Most histological subtypes were more common in males except squamous cell carcinoma and lymphoma. Muscle invasion was seen in 52.2% of urothelial carcinoma cases vs 87.5%, 71.9% and 89.0% of squamous cell carcinoma, adenocarcinoma and neuroendocrine tumor cases, respectively. For urothelial carcinoma, squamous cell carcinoma and adenocarcinoma women presented at more advanced stage. In the neuroendocrine group this stage difference was the opposite. Survival analysis showed a 5-year relative survival rate of 32.2%, 22.9%, 31.8% and 21.1% for T2 or greater urothelial carcinoma, squamous cell carcinoma, adenocarcinoma and neuroendocrine tumors, respectively. Conclusions: Patients with nonurothelial carcinoma present at more advanced stage and overall have worse survival. Relative survival of muscle invasive adenocarcinoma equals survival of muscle invasive urothelial carcinoma. For stage II and III disease these cases do even better. Muscle invasive squamous cell carcinoma and neuroendocrine tumors show worse survival regardless of stage. [Copyright &y& Elsevier]

A family history of urothelial cell carcinoma (UCC) confers an almost two-fold increased risk of developing UCC. It is unknown whether (part of) this aggregation of UCC has a Mendelian background. We performed complex segregation analyses on 1193 families ascertained through a proband with UCC of the bladder, ureter, renal pelvis or urethra, who were newly diagnosed between January 1, 1995 and December 31, 1997 and registered by two population-based cancer registries in the southeastern part of the Netherlands. Data were reported on 10 738 first-degree relatives by postal questionnaire; 101 of these relatives had UCC. All reported occurrences of UCC were verified (if possible) using medical records. Analyses were performed with the S.A.G.E. segregation package. Five restricted models (Mendelian dominant, Mendelian recessive, Mendelian co-dominant, 'no major gene' model and environmental model) were tested against the general unrestricted model. Sex and smoking status were incorporated as covariates. Strong evidence of Mendelian inheritance of UCC through a single major gene was not found in these 1 193 families. However, since none of the Mendelian models could be rejected, an inherited subtype of UCC cannot be excluded. A major gene may segregate in some families but this effect may have been masked in a background of high sporadic incidence. The 'no major gene' (or sporadic) model appeared to be the most parsimonious one to describe the occurrence of UCC in these families.