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“Renal medullary carcinoma”

Leandro Blas, Javier Roberti, Jorgelina Petroni, Liliana Reniero, Federico Cicora
PURPOSE OF THE REVIEW: We present an updated report of renal medullary carcinoma (RMC), a rare and aggressive condition. RECENT FINDINGS: There is a majority of male patients, of African descent, in the second or third decade of life. In differential diagnosis, other tumors, such as malignant rhabdoid tumor (MRT), vinculin-anaplastic lymphoma kinase (VCL-ALK) translocation renal cell carcinoma, and collecting duct carcinoma, may present difficulties. Abnormalities of tumor suppressor gene SMARCB1 have been found in RMC...
January 17, 2019: Current Urology Reports
Xiaopeng He, Hanmei Zhang, Tong Zhang, Fugang Han, Bin Song
To evaluate the values of conventional image features (CIFs) and radiomic features (RFs) extracted from multi-detector computed tomography (MDCT) images for predicting low- and high-grade clear cell renal cell carcinoma (ccRCC).Two hundred twenty-seven patients with ccRCC were retrospectively recruited. Five hundred seventy features including 14 CIFs and 556 RFs were extracted from MDCT images of each ccRCC. The CIFs were extracted manually and RFs by the free software-MaZda. Least absolute shrinkage and selection operator (Lasso) was applied to shrink the high-dimensional data set and select the features...
January 2019: Medicine (Baltimore)
Luisa Paschke, Thomas Lincke, Katja Sibylle Mühlberg, Wolfram J Jabs, Tom H Lindner, Ralf Paschke
Cabozantinib and lenvatinib have been approved for the treatment of progressive medullary thyroid cancer and radioiodine-resistant thyroid cancer, respectively. Both phase III trials of cabozantinib and lenvatinib reported that renal adverse events (AEs) rarely occurred. The cabozantinib phase III study reported no AEs related to renal toxicity. In the lenvatinib phase III trial grade 3 (CTCAE), proteinuria (urinary protein ≥3.5 g/24 h) was found in 10.0% of the lenvatinib and 0.0% of the placebo patients...
November 2018: European Thyroid Journal
Robert H Young, John N Eble
This article begins with the testis and a legendary figure, Sir Astley Cooper, who wrote an early text on the organ. The early 20th century saw the first major development, the description of the seminoma by the French investigator Maurice Chevassu, but the pace of knowledge did not accelerate until after World War II with a major article from the Armed Forces Institute of Pathology (AFIP) by Nathan B. Friedman and Robert A. Moore, soon followed by the first series testis fascicle by Frank J. Dixon and Moore...
January 2019: Histopathology
Juan Chipollini, Mounsif Azizi, Charles C Peyton, Dominic H Tang, Jasreman Dhillon, Philippe E Spiess
The purpose of this study was to assess the prognostic value of programmed death ligand-1 (PD-L1) positivity in a non-clear cell renal cell carcinoma (non-ccRCC) cohort. PD-L1 expression was evaluated by immunohistochemistry (IHC) using formalin-fixed paraffin-embedded (FFPE) specimens from 45 non-ccRCC patients with available tissue. PD-L1 positivity was defined as ≥1% of staining. Histopathological characteristics and oncological outcomes were correlated to PD-L1 expression. Cancer-specific survival (CSS) and recurrence-free survival (RFS) stratified by PD-L1 status were estimated using the Kaplan-Meier method...
2018: Journal of Kidney Cancer and VHL
Laurence Albiges, Ronan Flippot, Nathalie Rioux-Leclercq, Toni K Choueiri
Non-clear cell renal cell carcinomas (RCCs) account for up to 25% of kidney cancers and encompass distinct diseases with distinct pathologic features, different molecular alterations, and various patterns of response to systemic therapies. Recent advances in molecular biology and large collaborative efforts helped to better define the oncogenic mechanisms at play in papillary, chromophobe, collecting duct, medullary, translocation, and sarcomatoid RCCs. Papillary RCCs are divided into several subsets of tumors characterized by distinct gene expression profiles, chromatin remodeling genes, cell cycle changes, and alterations of the MET pathway...
October 29, 2018: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
C Christofer Juhlin, Robert Bränström, Ivan Shabo, Anders Höög
Follicular neoplasms of the thyroid gland are most often characterized by follicular-patterned thyrocytes with a neutrally stained cytoplasm, while a minority of cases present with oncocytic differentiation (Hürthle cell tumors). Exceedingly rare variants with a clear cell phenotype have also been reported, both as clear cell follicular thyroid adenomas (ccFTAs) and clear cell follicular carcinomas (ccFTCs). We present a patient with a 30-mm lesion in the thyroid isthmus in which the preoperative cytology proposed a follicular tumor...
October 29, 2018: International Journal of Surgical Pathology
Naoto Kuroda, Emiko Sugawara, Hironori Kusano, Yoshiaki Yuba, Kenji Yorita, Kengo Takeuchi
ALK-rearranged renal cell carcinoma (ALK-RCC) has been recently proposed and incorporated into the recent World Health Organisation Classification of renal tumours as a provisional entity. In this article, we review ALK-RCC with a focus on clinical and pathobiological aspects. Seventeen cases have been described to date. ALK-RCC accounts for less than 1% of all renal tumours. The age of patients ranges from 6 to 61 years with a mean age of 29.6 years. Grossly, the tumour forms were ill-demarcated or well demarcated solid mass in the renal medulla...
2018: Polish Journal of Pathology: Official Journal of the Polish Society of Pathologists
Xin-Wen Ke, Xing Zeng, Xian Wei, Yuan-Qing Shen, Jia-Hua Gan, Ji-Hua Tian, Zhi-Quan Hu
This study was designed to explore the safety and feasibility of robotic-assisted laparoscopic nephrectomy with vein thrombectomy (RAL-NVT) for the treatment of renal cell carcinoma (RCC) with venous tumor thrombus (VTT). Clinical data of 6 patients treated with RAL-NVT between July 2016 and November 2017 in our hospital were retrospectively collected and analyzed. There were 5 males and 1 female with their age ranging from 48 years to 68 years. Five renal tumors were right-sided and one left-sided. Three cases fell in level 0 VTT, one in level I and two in level II...
October 2018: Current medical science
Giovanni M Frascà, Fabiana Brigante, Alessandro Volpe, Laura Cosmai, Maurizio Gallieni, Camillo Porta
Due to the increasing occurrence of renal cell carcinoma (RCC) in the general population and the high prevalence of chronic kidney disease among cancer patients, many people with a previous RCC may eventually require renal replacement therapy including kidney transplantation. They should accordingly be evaluated to assess their life expectancy and the risk that the chronic immunosuppressive therapy needed after grafting might impair their long-term outcome. Current guidelines on listing patients for renal transplantation suggest that no delay is required for subjects with small or incidentally discovered RCC, while the recommendations for patients who have been treated for a symptomatic RCC or for those with large or invasive tumours are conflicting...
October 16, 2018: Journal of Nephrology
Ali Al-Daghmin, Malek Gaashan, Hussam Haddad
No abstract text is available yet for this article.
January 2019: Urology Case Reports
Pavlos Msaouel, Andrew L Hong, Elizabeth A Mullen, Michael B Atkins, Cheryl Lyn Walker, Chung-Han Lee, Marcus A Carden, Giannicola Genovese, W Marston Linehan, Priya Rao, Maria J Merino, Howard Grodman, Jeffrey S Dome, Conrad V Fernandez, James I Geller, Andrea B Apolo, Najat C Daw, H Courtney Hodges, Marva Moxey-Mims, Darmood Wei, Donald P Bottaro, Michael Staehler, Jose A Karam, W Kimryn Rathmell, Nizar M Tannir
Renal medullary carcinoma (RMC) is one of the most aggressive renal cell carcinomas. It predominantly afflicts young adults and adolescents with sickle cell trait and other sickle hemoglobinopathies, and is refractory to targeted and antiangiogenic therapies used in patients with clear-cell renal cell carcinoma. Platinum-based cytotoxic chemotherapy is the mainstay for RMC treatment. On the basis of recent advances in the diagnosis, management, and clinical trial development for RMC, a panel of experts met in October 2017 and developed updated consensus recommendations to inform clinicians, researchers, and patients...
September 12, 2018: Clinical Genitourinary Cancer
Guoshuang Shen, Fangchao Zheng, Dengfeng Ren, Feng Du, Qiuxia Dong, Ziyi Wang, Fuxing Zhao, Raees Ahmad, Jiuda Zhao
Anlotinib is a new, orally administered tyrosine kinase inhibitor that targets vascular endothelial growth factor receptor (VEGFR), fibroblast growth factor receptor (FGFR), platelet-derived growth factor receptors (PDGFR), and c-kit. Compared to the effect of placebo, it improved both progression-free survival (PFS) and overall survival (OS) in a phase III trial in patients with advanced non-small-cell lung cancer (NSCLC), despite progression of the cancer after two lines of prior treatments. Recently, the China Food and Drug Administration (CFDA) approved single agent anlotinib as a third-line treatment for patients with advanced NSCLC...
September 19, 2018: Journal of Hematology & Oncology
Rosario F Di Stefano, Consuelo Buttigliero, Emmanuele De Luca, Maria Lucia Reale, Chiara Pisano, Gianmarco Leone, Clizia Zichi, Federica Massa, Matteo Manfredi, Francesca Vignani, Enrico Bollito, Francesco Porpiglia, Massimo Di Maio, Marcello Tucci
No abstract text is available yet for this article.
July 21, 2018: Clinical Genitourinary Cancer
Sarah E Stump, Young E Whang, Daniel J Crona
Cabozantinib is a multikinase inhibitor approved for the treatment of metastatic medullary thyroid cancer and advanced renal cell carcinoma (RCC) in patients who have received prior anti-angiogenic therapy. While associations between serum creatine kinase (CK) elevations and other tyrosine kinase inhibitors used for the treatment of solid malignancies have been previously reported, we report a case of cabozantinib-associated CK elevation that was associated with musculoskeletal complaints by an RCC patient...
December 2018: Investigational New Drugs
Mariana M Cajaiba, Lisa M Dyer, James I Geller, Lawrence J Jennings, David George, Dawn Kirschmann, Stephen M Rohan, Nicholas G Cost, Geetika Khanna, Elizabeth A Mullen, Jeffrey S Dome, Conrad V Fernandez, Elizabeth J Perlman
BACKGROUND: Renal cell carcinomas (RCCs) are rare in young patients. Knowledge of their pathologic and molecular spectrum remains limited, and no prospective studies have been performed to date in this population. This study analyzes patients diagnosed with RCC who were prospectively enrolled in the AREN03B2 Children's Oncology Group (COG). The objective was to classify these tumors with the aid of focused genetic testing and to characterize their features. METHODS: All tumors registered as RCC by central review were retrospectively re-reviewed and underwent additional ancillary studies...
August 2018: Cancer
Jenny Z Lai, H Henry Lai, Dengfeng Cao
Renal medullary carcinoma (RMC) is an aggressive high-grade renal cell carcinoma (RCC) associated almost exclusively with sickle cell trait or sickle cell disease. However, RCC with RMC features has rarely been reported in patients with no sickle cell trait or disease. Renal cell carcinoma unclassified with medullary phenotype (RCCU-MP) is a newly-coined term used by an international panel of experts to describe renal cell carcinoma showing morphologic and immunohistochemical features of renal medullary carcinoma in patients without sickle cell trait/disease...
June 2018: Anticancer Research
Abbas Agaimy, Mahul B Amin, Anthony J Gill, Bernt Popp, André Reis, Daniel M Berney, Cristina Magi-Galluzzi, Mathilde Sibony, Steven C Smith, Saul Suster, Kiril Trpkov, Ondřej Hes, Arndt Hartmann
Fumarate hydratase-deficient renal cell carcinoma (FH-RCC) is a rare, aggressive RCC type, originally described in the setting of hereditary leiomyomatosis and RCC syndrome, which is defined by germline FH gene inactivation. Inactivation of components of the switch/sucrose nonfermentable (SWI/SNF) chromatin remodeling complex is involved in renal medullary carcinoma (SMARCB1/INI1 loss), clear cell RCC (PBRM1 loss), and subsets of dedifferentiated RCC of clear cell, chromophobe, and papillary types (loss of different SWI/SNF components)...
July 2018: Human Pathology
Steven Lacy, Bei Yang, Jace Nielsen, Dale Miles, Linh Nguyen, Matt Hutmacher
PURPOSE: An integrated population pharmacokinetic (popPK) model was developed to describe the pharmacokinetics (PK) of tyrosine kinase inhibitor cabozantinib in healthy volunteers (HVs) and patients with various cancer types and to identify any differences in cabozantinib PK across these populations. METHODS: Plasma concentration data used to develop the popPK model were obtained from nine clinical trials (8072 concentrations from 1534 HVs or patients) of cabozantinib in HVs and patients with renal cell carcinoma (RCC), medullary thyroid carcinoma (MTC), glioblastoma multiforme, castration-resistant prostate cancer, or other advanced malignancies...
June 2018: Cancer Chemotherapy and Pharmacology
Jaime A Singh, Chisato Ohe, Steven Christopher Smith
Collecting duct carcinoma was described over 30 years ago as a renal tumor, based in the medullary collecting system, with tubulopapillary morphology, prominent infiltrative growth, and stromal desmoplasia. While diagnostic workup has always emphasized exclusion of upper tract urothelial carcinoma and metastatic adenocarcinoma to the kidney, the molecular era of renal cell carcinoma classification has enabled recognition of and provided tools for diagnosis of new entities in this morphologic differential. In this review, we consider these developments, with emphasis on renal medullary carcinoma, closely related renal cell carcinoma, unclassified with medullary phenotype, and fumarate hydratase-deficient renal cell carcinoma...
May 2018: Pathology International
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