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Sounak Gupta Rafael E. Jimenez Loren Herrera-Hernandez Christine M. Lohse R. Houston Thompson Stephen A. Boorjian Bradley C. Leibovich John C. Cheville 《Mayo Clinic proceedings. Mayo Clinic》2021,96(6):1470-1489
ObjectiveTo study the clinical features and identify unique renal neoplasia subtypes and their prognostic implications in individuals with tuberous sclerosis complex (TSC).Patients and MethodsThe Mayo Clinic nephrectomy registry included 37 patients with TSC diagnosed between 1970 and 2018. Four additional patients were identified from the pathology consultation and autopsy files. All available renal tumors were further characterized using immunohistochemistry and fluorescence in situ hybridization. Clinicopathologic features and follow-up were obtained from the medical record. The American Association for Cancer Research Project GENIE registry was accessed using cBioPortal for molecular profiling of angiomyolipoma (AML).ResultsA total of 276 renal tumors from 41 patients were analyzed. Renal tumors were classified into 9 distinct morphological subtypes, with AML predominating (238 [86%]). Interestingly, all these tumors acted in a benign fashion except one renal cell carcinoma with clear cells and fibromyomatous stroma and one epithelioid AML that metastasized. Molecular profiling studies revealed that epithelioid AMLs were enriched for alterations of TP53, RB1, and ATRX. Eight patients died of direct complications of TSC, including 3 of end-stage renal disease. To date, none have died of a renal epithelial neoplasm.ConclusionThe identification of unique renal neoplasia subtypes may provide important clues to establish a diagnosis of TSC, and in the somatic setting, this finding has important implications for accurate prognostication. These tumors tend to be indolent, and only 2 of 276 tumors in our study exhibited metastatic behavior. Our results support multidisciplinary management with a focus on preservation of renal function. 相似文献
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Michael G Fehlings Jetan H Badhiwala Henry Ahn H Francis Farhadi Christopher I Shaffrey Ahmad Nassr Praveen Mummaneni Paul M Arnold W Bradley Jacobs K Daniel Riew Michael Kelly Darrel S Brodke Alexander R Vaccaro Alan S Hilibrand Jason Wilson James S Harrop S Tim Yoon Kee D Kim Branko Kopjar 《Lancet neurology》2021,20(2):98-106
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Smetana Keaton S. Zakeri Amanda Dolia Jaydevsinh Huttinger Allyson May Casey C. Youssef Patrick Gross Bradley A. Nimjee Shahid M. 《Journal of thrombosis and thrombolysis》2021,52(4):1182-1186
Journal of Thrombosis and Thrombolysis - Tandem occlusions exist in 17–32% of large vessel occlusion (LVO) strokes. A significant concern is bleeding when carotid stenting is performed in... 相似文献
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Swanson Amy A. Erickson Dana Donegan Diane Mary Jenkins Sarah M. Van Gompel Jamie J. Atkinson John L. D. Erickson Bradley J. Giannini Caterina 《Pituitary》2021,24(2):192-206
Pituitary - Growth hormone-producing pituitary adenomas are divided into two clinically relevant histologic subtypes, densely (DG-A) and sparsely (SG-A) granulated. Histologic subtype was evaluated... 相似文献
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Patrick Hayes H. Sherman Jonathan H. Elder J. Bradley Olson Jeffrey J. 《Journal of neuro-oncology》2022,158(2):167-177
Journal of Neuro-Oncology - In patients with previously diagnosed glioblastoma who are suspected of experiencing progression, does repeat cytoreductive surgery improve progression free survival or... 相似文献
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