This document summarizes rare and unusual types of urological cancers. It discusses rare subtypes of renal cell carcinoma defined by their histology and genetic abnormalities. It also describes rare histological variants of bladder cancer and non-urothelial cancers of the bladder such as squamous cell carcinoma, adenocarcinoma, small cell carcinoma, and lymphomas. Finally, it briefly outlines other rare non-urothelial cancers including sarcomas, carcinoid tumors, and other even less common malignancies of the bladder.

1. Rare Urological Cancers Professor Vesa V Kataja Head of Department Cancer Center, UEF / KUH Kuopio, FINLAND Kuopio University Hospital

5. Rare Renal Cancers *Not in WHO 2004 classification Histological subtypes of sporadic renal cell cancer Histology/Entity Prognosis Note End-stage renal disease associated carcinoma* 40 – 100 x the risk of general population Depends on histopathological subtype Spectrum of recognised types Two novel neoplasia 1) Cystic disease associated RCC 2) Papillary clear cell RCC Follicular RC* Good Note! metastatic follicular carcinoma of the thyroid Clear cell papillary and cystic RCC* Excellent TFE3 negative Oncocytic papillary RCC* Not established; mainly on better side Genetically similar to papillary RCC type 1 and 2 (trisomy 7, 17, loss of Y in FISH); male predominance (87 %)

6. Umberto Capitanio et al., BJU Int 103:1496-1500;2009

13. Rare Urothelial Bladder Cancers Variants of invasive urothelial carcinoma Squamous differentiation Glandular differentiation Nested pattern Microcystic Micropapillary Lymphoepithelioma-like Plasmacytoid and lymphoma-like Sarcomatoid/carcinosarcoma Giant cell Trophoblastic differentiation Clear cell Lipid cell Undifferentiated Adapted from the World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Urinary System and Male Genital Organs Compared to pure urothelial cancers: - Higher clinical stage at presentation - More likely to be invasive

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