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Item Clues to Recognition of Fumarate Hydratase-Deficient Renal Cell Carcinoma: Findings From Cytologic and Limited Biopsy Samples(Wiley, 2018) Shyu, Irene; Mirsadraei, Leili; Wang, Xiaoyan; Robila, Valentina; Mehra, Rohit; McHugh, Jonathan B.; Chen, Ying-Bei; Udager, Aaron M.; Gill, Anthony J.; Cheng, Liang; Amin, Mahul B.; Lin, Oscar; Smith, Steven Christopher; Pathology and Laboratory Medicine, School of MedicineBackground: Fumarate hydratase (FH)-deficient renal cell carcinoma (RCC) is rare and highly aggressive and is believed to arise mostly in the setting of hereditary leiomyomatosis-RCC syndrome with a germline mutation of FH. Because of the aggressiveness of these tumors and a frequent lack of ascertainable family history, these tumors may first present as metastases and be sampled by cytology. The cytologic findings of FH-deficient RCC have not previously been reported. Methods: Cytologic and limited biopsy samples from patients with FH-deficient RCC were reviewed retrospectively. Results: In total, 24 cytologic and limited biopsy samples from 19 patients (6 women and 13 men; age range, 22-69 years) who had FH-deficient RCC and metastasis at presentation were evaluated. These included 21 cytology samples ranging from malignant effusions (n = 7) to metastases (n = 11), to samples of primary kidney tumors (n = 3). The samples exhibited cells, often in clusters and abortive papillae, with voluminous, finely vacuolated cytoplasm and large, pleomorphic nuclei with prominent, viral inclusion-like nucleoli. A distinctive finding of peripheral cytoplasmic clearing frequently was apparent, and intranuclear cytoplasmic pseudoinclusions were less frequent. Of 7 cell block and biopsy samples, several of which represented sampling from the same patient, all demonstrated tissue fragments that had discernable morphologic patterns associated with FH-deficient RCC, including tubulocystic and intracystic papillary growth. Conclusions: Features characteristic and suggestive of FH-deficient RCC may be identified in cytologic and small biopsy samples. Although the current samples were identified retrospectively in well characterized cases of FH-deficient RCC, the authors argue that, with appropriate clinical correlation, these features are sufficiently distinctive to trigger recognition and confirmatory workup.Item Update for the practicing pathologist: The International Consultation On Urologic Disease-European association of urology consultation on bladder cancer(SpringerNature, 2015-05) Amin, Mahul B.; Smith, Steven C.; Reuter, Victor E.; Epstein, Jonathan I.; Grignon, David J.; Hansel, Donna E.; Lin, Oscar; McKenney, Jesse K.; Montironi, Rodolfo; Paner, Gladell P.; Al-Ahmadie, Hikmat A.; Algaba, Ferran; Ali, Syed; Alvarado-Cabrero, Isabel; Bubendorf, Lukas; Cheng, Liang; Cheville, John C.; Kristiansen, Glen; Cote, Richard J.; Delahunt, Brett; Eble, John N.; Genega, Elizabeth M.; Gulmann, Christian; Hartmann, Arndt; Langner, Cord; Lopez-Beltran, Antonio; Magi-Galluzzi, Cristina; Merce, Jorda; Netto, George J.; Oliva, Esther; Rao, Priya; Ro, Jae Y.; Srigley, John R.; Tickoo, Satish K.; Tsuzuki, Toyonori; Umar, Saleem A.; Van der Kwast, Theo; Young, Robert H.; Soloway, Mark S.; Department of Pathology and Laboratory Medicine, IU School of MedicineThe International Consultations on Urological Diseases are international consensus meetings, supported by the World Health Organization and the Union Internationale Contre le Cancer, which have occurred since 1981. Each consultation has the goal of convening experts to review data and provide evidence-based recommendations to improve practice. In 2012, the selected subject was bladder cancer, a disease which remains a major public health problem with little improvement in many years. The proceedings of the 2nd International Consultation on Bladder Cancer, which included a 'Pathology of Bladder Cancer Work Group,' have recently been published; herein, we provide a summary of developments and consensus relevant to the practicing pathologist. Although the published proceedings have tackled a comprehensive set of issues regarding the pathology of bladder cancer, this update summarizes the recommendations regarding selected issues for the practicing pathologist. These include guidelines for classification and grading of urothelial neoplasia, with particular emphasis on the approach to inverted lesions, the handling of incipient papillary lesions frequently seen during surveillance of bladder cancer patients, descriptions of newer variants, and terminology for urine cytology reporting.