PMID- 26975037 OWN - NLM STAT- MEDLINE DCOM- 20170719 LR - 20180814 IS - 1532-0979 (Electronic) IS - 0147-5185 (Linking) VI - 40 IP - 7 DP - 2016 Jul TI - Renal Cell Carcinoma Occurring in Patients With Prior Neuroblastoma: A Heterogenous Group of Neoplasms. PG - 989-97 LID - 10.1097/PAS.0000000000000632 [doi] AB - Renal cell carcinoma (RCC) associated with neuroblastoma (NB) was included as a distinct entity in the 2004 World Health Organization classification of kidney tumors. A spectrum of RCC subtypes has been reported in NB survivors. We herein describe a series of 8 RCCs diagnosed in 7 patients with a history of NB. Microscopic evaluation, immunohistochemical staining for PAX8, cathepsin K, and succinate dehydrogenase subunit B (SDHB), and fluorescence in situ hybridization (FISH) for TFE3 and TFEB were performed. Four distinct morphologic subtypes were identified: 3 tumors were characterized by cells with abundant oncocytoid cytoplasm and irregular nuclei; 3 showed features of microphthalmia transcription factor family translocation RCC (MiTF-RCC); 1 had features of hybrid oncocytic-chromophobe tumor; 1 had papillary RCC histology. All RCCs expressed PAX8 and retained SDHB expression. Cathepsin K was positive in 2 MiTF-RCCs, 1 was TFEB FISH positive, and the other was indeterminate. Cathepsin K was negative in a third MiTF-RCC with TFE3 rearrangement. TFE3 FISH was negative in 4 and insufficient in 1 of the other 5 RCCs. While a subset of RCCs associated with NB is characterized by cells with prominent oncocytoid cytoplasm, other RCC subtypes also occur in post-NB patients. Renal neoplasms occurring in patients with a history of NB do not represent a single entity but a heterogenous group of RCCs. SDHB mutations do not explain the subset of nontranslocation RCCs with oncocytoid features; therefore, further studies are needed to clarify whether they may represent a distinct entity with unique molecular abnormalities or may belong to other emerging RCC subtypes. FAU - Falzarano, Sara M AU - Falzarano SM AD - *Robert J. Tomsich Pathology and Laboratory Medicine Institute, Cleveland Clinic, Cleveland, OH double dagger Department of Pathology and Laboratory Medicine, Indiana University, Indianapolis, IN section signDepartment of Pathology, Emory University School of Medicine, Atlanta, GA parallelDepartment of Pathology, Kaiser Permanente Panorama City Medical Center, Panorama City paragraph signDivision of Pathology, Children's Hospital Los Angeles, Los Angeles, CA #Department of Pathology and Michigan Center for Translational Pathology, University of Michigan, Ann Arbor, MI daggerSection of Pathological Anatomy, University of Ancona, Ancona, Italy. FAU - McKenney, Jesse K AU - McKenney JK FAU - Montironi, Rodolfo AU - Montironi R FAU - Eble, John N AU - Eble JN FAU - Osunkoya, Adeboye O AU - Osunkoya AO FAU - Guo, Juan AU - Guo J FAU - Zhou, Shengmei AU - Zhou S FAU - Xiao, Hong AU - Xiao H FAU - Dhanasekaran, Saravana M AU - Dhanasekaran SM FAU - Shukla, Sudhanshu AU - Shukla S FAU - Mehra, Rohit AU - Mehra R FAU - Magi-Galluzzi, Cristina AU - Magi-Galluzzi C LA - eng PT - Journal Article PL - United States TA - Am J Surg Pathol JT - The American journal of surgical pathology JID - 7707904 RN - 0 (Biomarkers, Tumor) SB - IM MH - Biomarkers, Tumor/analysis MH - Carcinoma, Renal Cell/*pathology MH - Child MH - Child, Preschool MH - Cohort Studies MH - Female MH - Humans MH - Immunohistochemistry MH - In Situ Hybridization, Fluorescence MH - Infant MH - Kidney Neoplasms/*pathology MH - Male MH - Neoplasms, Second Primary/*pathology MH - Neuroblastoma/*pathology MH - Polymerase Chain Reaction MH - Survivors EDAT- 2016/03/15 06:00 MHDA- 2017/07/20 06:00 CRDT- 2016/03/15 06:00 PHST- 2016/03/15 06:00 [entrez] PHST- 2016/03/15 06:00 [pubmed] PHST- 2017/07/20 06:00 [medline] AID - 10.1097/PAS.0000000000000632 [doi] PST - ppublish SO - Am J Surg Pathol. 2016 Jul;40(7):989-97. doi: 10.1097/PAS.0000000000000632.