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“Renal medullary carcinoma”

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https://www.readbyqxmd.com/read/29956055/cabozantinib-induced-serum-creatine-kinase-elevation-and-musculoskeletal-complaints
#1
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...
June 28, 2018: Investigational New Drugs
https://www.readbyqxmd.com/read/29905933/the-classification-of-pediatric-and-young-adult-renal-cell-carcinomas-registered-on-the-children-s-oncology-group-cog-protocol-aren03b2-after-focused-genetic-testing
#2
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...
June 15, 2018: Cancer
https://www.readbyqxmd.com/read/29848739/renal-cell-carcinoma-unclassified-with-medullary-phenotype-and-synchronous-renal-clear-cell-carcinoma-present-in-a-patient-with-no-sickle-cell-trait-disease-diagnostic-and-therapeutic-challenges
#3
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
https://www.readbyqxmd.com/read/29689242/swi-snf-protein-expression-status-in-fumarate-hydratase-deficient-renal-cell-carcinoma-immunohistochemical-analysis-of-32-tumors-from-28-patients
#4
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
https://www.readbyqxmd.com/read/29687244/a-population-pharmacokinetic-model-of-cabozantinib-in-healthy-volunteers-and-patients-with-various-cancer-types
#5
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
https://www.readbyqxmd.com/read/29665139/high-grade-infiltrative-adenocarcinomas-of-renal-cell-origin-new-insights-into-classification-morphology-and-molecular-pathogenesis
#6
REVIEW
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
https://www.readbyqxmd.com/read/29582957/-renal-manifestation-of-autosomal-dominant-polycystic-kidney-disease
#7
Marco Galliani, Silvana Chicca, Elio Vitaliano, Luca Di Lullo, Konstantinos Giannakakis, Antonio Paone
Autosomal dominant polycystic kidney disease affects over 12 million people in the world and is the fourth cause of ESRD. It is the main monogenic kidney disease and causes the progressive formation of cysts leading to renal failure after a few decades. The main manifestations of the disease are observed even at a young age. The early sign of ADPKD is impaired urinary concentrating capacity, due to medullary alteration by cysts, and resistance to vasopressin. These anatomical alterations determine hyperfiltration, altered ammonium transport, nephrolithiasis, and, above all, hypertension even in pediatric age...
March 2018: Giornale Italiano di Nefrologia: Organo Ufficiale Della Società Italiana di Nefrologia
https://www.readbyqxmd.com/read/29440190/a-model-linking-sickle-cell-hemoglobinopathies-and-smarcb1-loss-in-renal-medullary-carcinoma
#8
Pavlos Msaouel, Nizar M Tannir, Cheryl Lyn Walker
Renal medullary carcinoma (RMC) is a highly aggressive malignancy that predominantly afflicts young adults and adolescents with sickle hemoglobinopathies. It is characterized by complete loss of expression of the chromatin remodeler and tumor suppressor SMARCB1 Despite therapy, the outcomes of patients with RMC remain very poor, highlighting the need to understand the etiology of this cancer, and develop new diagnostic, preventive, and therapeutic strategies. A key knowledge gap in RMC biology is why sickle hemoglobinopathies predispose to the development of this cancer...
May 1, 2018: Clinical Cancer Research: An Official Journal of the American Association for Cancer Research
https://www.readbyqxmd.com/read/29429165/-clinicopathologic-features-with-collecting-duct-carcinoma-of-kidney-report-of-10-cases
#9
J X Zhou, X R He, G X Song, Z G Zou, L H Wang, R Hu, H X Li
Objective: To study the pathological features, immunophenotypes, differential diagnoses and prognostic parameters of collecting duct carcinoma of the kidney (CDC). Methods: Clinical imaging, histopathology, immunohistochemistry, and survival data of 10 patients at First Affiliated Hospital of Nanjing Medical University from January 2009 to August 2017 were retrospectively analyzed along with a review of literatures. Results: The clinical symptoms of CDC were not specific, and image examinations showed space-occupying mass lesions...
February 8, 2018: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
https://www.readbyqxmd.com/read/29405994/alk-tpm3-rearrangement-in-adult-renal-cell-carcinoma-report-of-a-new-case-showing-loss-of-chromosome-3-and-literature-review
#10
Yohan Bodokh, Damien Ambrosetti, Valérie Kubiniek, Branwel Tibi, Matthieu Durand, Jean Amiel, Morgane Pertuit, Anne Barlier, Florence Pedeutour
Seven cases of translocation-associated renal cell carcinoma involving ALK (ALK-tRCC) were referenced in the last World Health Organization's classification (2016), in a group of emerging/provisional RCC. The first three cases were pediatric, medullary-based, associated with sickle-cell trait and showed a fusion of ALK with VCL. Thirteen cases have been further described. They displayed clinical, morphological and genomic heterogeneity. Most of them occurred in adults. None of the patients was affected by sickle-cell disease...
February 2018: Cancer Genetics
https://www.readbyqxmd.com/read/29396817/renal-medullary-carcinoma-a-nearly-fatal-malignancy-specifically-affecting-patients-with-a-so-called-benign-condition
#11
Yamila Goenaga-Vázquez, Gloria Colón, Nilka Barrios, María Correa
Renal medullary carcinoma (RMC) is a highly aggressive and rare malignancy found almost exclusively in young patients with sickle cell trait (SCT). Metastatic disease is commonly present at diagnosis. There is very limited experience treating disseminated disease and the prognosis is dismal. We report the case of a young 9-year-old boy with SCT, who presented with 4 months' progression of abdominal pain, nausea and vomiting associated with cough spells, dysphagia, and weight loss. Upon evaluation, he was underweight, pale, and in mild respiratory distress...
May 2018: CEN Case Reports
https://www.readbyqxmd.com/read/29311252/pazopanib-induced-hypertension-in-patients-with-renal-cell-carcinoma-is-associated-with-low-urine-excretion-of-no-metabolites
#12
Anne Robdrup Tinning, Camilla Bengtsen, Niels Viggo Jensen, Lars Bastholt, Boye Lagerbon Jensen, Kirsten Madsen
Drugs targeting VEGF (vascular endothelial growth factor) are often associated with rapid development of hypertension by a yet not fully understood mechanism. VEGF is expressed in renal epithelial cells and stimulates NO production. In the renal medulla, inhibition of NO formation by local L-NAME or by impaired endothelin-1 leads to hypertension. The present study was designed to test the hypothesis that VEGF receptor inhibitor treatment leads to hypertension through decreased renal medullary formation of NO and endothelin-1...
March 2018: Hypertension
https://www.readbyqxmd.com/read/29283440/cabozantinib-a-multitargeted-oral-tyrosine-kinase-inhibitor
#13
REVIEW
Jordan N Markowitz, Karen M Fancher
Cabozantinib is an oral small-molecule multitargeted tyrosine kinase inhibitor (TKI) that may confer an advantage over other TKIs that target a single receptor. It was approved by the U.S. Food and Drug Administration for the treatment of both advanced renal cell carcinoma and progressive metastatic medullary thyroid cancer, and it is being investigated for a wide array of other malignancies. Rationale for use, clinical trial data, and current recommendations for cabozantinib in renal cell cancer, thyroid cancer, prostate cancer, hepatocellular cancer, and lung cancer are detailed in this article...
March 2018: Pharmacotherapy
https://www.readbyqxmd.com/read/29280680/smarcb1-deficient-tumors-of-childhood-a-practical-guide
#14
Bruce R Pawel
The SMARCB1 gene ( INI1, BAF47) is a member of the SWItch/Sucrose Non-Fermentable (SWI/SNF) chromatin remodeling complex, involved in the epigenetic regulation of gene transcription. SMARCB1 acts as a tumor suppressor gene, and loss of function of both alleles gives rise to SMARCB1-deficient tumors. The prototypical SMARCB1-deficient tumor is the malignant rhabdoid tumor (MRT) which was first described in the kidney but also occurs in soft tissue, viscera, and the brain (where it is referred to as atypical teratoid rhabdoid tumor or AT/RT)...
January 2018: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/29239041/hereditary-and-familial-thyroid-tumours
#15
REVIEW
Julie Guilmette, Vania Nosé
The worldwide incidence of thyroid malignancies has been increasing rapidly. Sensitive imaging modalities and early detection of thyroid lesions have made thyroid cancers the most rapidly increasing cancers in the USA in 2017 (SEER Cancer Facts, 2017). Clinical awareness of potential risk factors, such as inherited thyroid cancers, has allowed earlier recognition of more vulnerable population clusters. Hereditary thyroid neoplasms arising from calcitonin-producing C cells are known as familial medullary thyroid carcinomas (FMTCs), and include well-documented syndromes such as multiple endocrine neoplasia IIA or IIB, and pure familial medullary thyroid carcinoma syndrome...
January 2018: Histopathology
https://www.readbyqxmd.com/read/29225982/sarcomatoid-renal-cell-carcinoma-in-an-adolescent-with-sickle-cell-anaemia
#16
H R Ahmad, J A Faruk, M A Bugaje, A Solomon, M O A Samaila, R M Akuse
Malignancies have been reported to occur in people with sickle cell disease. Renal medullary carcinoma (RMC), also tagged seventh sickle cell nephropathy, is an aggressive cancer seen almost exclusively in people with sickle cell disease with more than 160 cases reported worldwide, but only few cases were reported in patients with sickle cell anaemia (HBSS) and from Nigeria. Sarcomatoid renal cell carcinoma is a renal tumour of any histologic variant containing foci of high-grade malignant spindle cells. We report an adolescent girl with sickle cell anaemia (HBSS) who presented with left renal tumour, histology of which confirmed a diagnosis of sarcomatoid renal cell carcinoma (sRCC)...
2017: Case Reports in Oncological Medicine
https://www.readbyqxmd.com/read/29168338/potential-new-strategies-for-the-treatment-of-renal-medullary-carcinoma
#17
EDITORIAL
Paul Russo
No abstract text is available yet for this article.
December 2017: BJU International
https://www.readbyqxmd.com/read/28921623/one-step-synthesis-of-18-f-cabozantinib-for-use-in-positron-emission-tomography-imaging-of-c-met
#18
Vegard Torp Lien, Jo Klaveness, Dag Erlend Olberg
Cabozantinib is an FDA-approved kinase inhibitor for the treatment of medullary thyroid cancer and advanced renal cell carcinoma, which exerts its therapeutic effect by inhibiting, among others, the tyrosine kinase c-Met. Noninvasive imaging techniques are becoming increasingly important clinically to ensure drug efficacy, staging, monitoring, and patient stratification. PET isotope labelled tyrosine kinase inhibitors have, for the same reason, potential as PET tracers for imaging of various cancers. On the basis of cabozantinib, we synthesized the novel boronic acid pinacol ester 4 as a labelling precursor, where the boronic ester moiety replaces the fluorine native to this kinase inhibitor...
January 2018: Journal of Labelled Compounds & Radiopharmaceuticals
https://www.readbyqxmd.com/read/28898190/renal-tumors-of-childhood-radiologic-pathologic-correlation-part-2-the-2nd-decade-from-the-radiologic-pathology-archives
#19
REVIEW
Ellen M Chung, Grant E Lattin, Kimberly E Fagen, Andrew M Kim, Michael A Pavio, Adam J Fehringer, Richard M Conran
Malignant renal tumors account for 7% of childhood cancers, and Wilms tumors are by far the most common-but not in older children and adolescents. Among individuals in the latter half of their 2nd decade of life, renal cell carcinoma (RCC) is more common than Wilms tumor. The histopathologic spectrum of RCCs in children differs from that in adults. The most common subtype of RCC in children and adolescents is Xp11.2 translocation RCC, which is distinguished by hyperattenuation at nonenhanced computed tomography, a defined capsule, and associated retroperitoneal lymphadenopathy...
September 2017: Radiographics: a Review Publication of the Radiological Society of North America, Inc
https://www.readbyqxmd.com/read/28898179/renal-medullary-carcinoma-associated-with-sickle-cell-trait
#20
Aletta Ann Frazier
No abstract text is available yet for this article.
September 2017: Radiographics: a Review Publication of the Radiological Society of North America, Inc
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