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Mod Pathol 2020 May 28. Epub 2020 May 28.

Department of Pathology, Faculty of Medicine in Pilsen, Charles University, Pilsen, Czech Republic.

ALK rearranged renal cell carcinoma (ALK-RCC) has recently been included in 2016 WHO classification as a provisional entity. In this study, we describe 12 ALK-RCCs from 8 institutions, with detailed clinical, pathological, immunohistochemical (IHC), fluorescence in situ hybridization (FISH), and next generation sequencing (NGS) analyses. Patients' age ranged from 25 to 68 years (mean, 46. Read More

Virchows Arch 2020 May 23. Epub 2020 May 23.

Department of Anatomic Pathology, Graduate School of Medical Sciences, Kyushu University, Maidashi 3-1-1, Higashi-ku, Fukuoka, 812-8582, Japan.

The presence of sarcomatoid or rhabdoid features (which are associated with advanced disease and poor prognosis) is rarely observed in the subtypes of renal cell carcinoma (RCC). The SWI/SNF chromatin-remodeling complex, which is composed of evolutionarily conserved core subunits including SMARCB1/INI1 (SMARCB1), SMARCA4/BRG1 (SMARCA4), SMARCC1/BAF155 (SMARCC1), and SMARCC2/BAF170 (SMARCC2), can be regarded as the prototype of an epigenetic regulator of gene expression that is involved in tumor suppression. We analyzed the histological, immunohistochemical, and clinicopathological status in 72 cases of RCC with sarcomatoid or rhabdoid features, focusing on the expression status of the subunits of SWI/SNF chromatin-remodeling complex proteins. Read More

Hum Pathol 2020 Jul 30;101:31-39. Epub 2020 Apr 30.

Department of Anatomic Pathology, Graduate School of Medical Sciences, Kyushu University, Higashi-ku, Fukuoka, Japan. Electronic address:

Renal cell carcinoma (RCC) with sarcomatoid changes and rhabdoid features has shown poor outcomes. Several immune checkpoint inhibitors including programmed cell death protein 1 (PD-1)/programmed death ligand-1 (PD-L1) inhibitors have been approved for the treatment of RCC. Combination therapy using PD-1/PD-L1 and indoleamine 2,3-dioxygenase 1 (IDO1) inhibitors has also been used to treat various malignancies. Read More

J Urol 2020 Apr 6:101097JU0000000000001063. Epub 2020 Apr 6.

Cancer Prognostics and Health Outcomes Unit, Division of Urology, University of Montréal Health Center, Montréal, Québec, Canada.

Background: To evaluate stage at presentation and cancer specific mortality (CSM) according to variant histology (VH) relative to clear cell renal cell carcinoma (ccRCC).

Methods: Within Surveillance, Epidemiology and End Results registry (SEER, 2001-2016), we identified VH and ccRCC patients. Cumulative incidence-plots, multivariate Cox regression models matched for stage, grade and other patient characteristics addressed CSM. Read More

BMC Urol 2020 Feb 18;20(1):14. Epub 2020 Feb 18.

Department of Urology, Peking University Third Hospital, 49 North Garden Road, Haidian District, Beijing, 100191, People's Republic of China.

Background: Sarcomatoid differentiation in renal cell carcinoma (RCC) with vena caval tumour thrombus has been shown to be associated with aggressive behaviours and poor prognosis; however, evidence of the impact of rhabdoid differentiation on prognosis is lacking. This study evaluated the impact of sarcomatoid differentiation and rhabdoid differentiation on oncological outcomes for RCC with vena caval tumour thrombus treated surgically.

Methods: We retrospectively analysed patients treated surgically for RCC with vena caval tumour thrombus at our institute from Jan 2015 to Nov 2018. Read More

Virchows Arch 2020 Jul 16;477(1):111-120. Epub 2020 Jan 16.

Department of Surgery and Pathology, Faculty of Medicine, Cordoba University, Cordoba, Spain.

We report on the clinicopathologic features of 115 cases of high-grade urothelial carcinoma of the upper urinary tract with variant histology present in 39 (34%). Variant histology was typically seen in high pathological stage (pT2-pT4) (82%, 32 cases) patients with lower survival rate (70%, 27 cases, median survival 31 months) and consisted in urothelial with one (23%), two (3%), and three or more variants (3%); 4% of cases presented with pure variant histology. Squamous divergent differentiation was the most common variant (7%) followed by sarcomatoid (6%) and glandular (4%), followed by 3% each of micropapillary, diffuse-plasmacytoid, inverted growth, clear cell glycogenic, or lipid-rich. Read More

Am J Surg Pathol 2019 10;43(10):1413-1420

Departments of Pathology.

Tumor size has been used for decision making in the management of patients with renal masses. Active surveillance in selected patients is now increasingly common in tumors ≤4 cm in size. Clear cell renal cell carcinoma (CCRCC) is the most common type of renal malignancy. Read More

J Clin Pathol 2019 Sep 12;72(9):573-578. Epub 2019 Jul 12.

Department of Pathology, University Health Network, Toronto, Ontario, Canada.

The International Collaboration on Cancer Reporting (ICCR) has developed a suite of detailed datasets for international implementation. These datasets are based on the reporting protocols developed by the Royal College of Pathologists (UK), The Royal College of Pathologists of Australasia and the College of American Pathologists, with modifications undertaken by international expert groups appointed according to ICCR protocols. The dataset for the reporting of renal biopsy for tumour is designed to provide a structured reporting template containing minimum data recording key elements suitable for international use. Read More

Urology 2019 Aug 8;130:86-92. Epub 2019 May 8.

Glickman Urological and Kidney Institute, Cleveland Clinic, Cleveland, OH. Electronic address:

Objective: To analyze the full spectrum of patients presenting with radiologically-identified infiltrative renal masses (IRMs), including those managed surgically or otherwise, with focus on clinical presentation/prognosis.

Methods: All 280 patients presenting with radiologically-identified renal mass with infiltrative features (2008-2017) were retrospectively reviewed. Poorly-defined interface between tumor and parenchyma and irregular shape (nonelliptical) in one or more distinct/unequivocal areas were required for classification as IRM. Read More

Ci Ji Yi Xue Za Zhi 2019 Apr-Jun;31(2):129-132

Department of Pathology, Ditmanson Medical Foundation, Chiayi Christian Hospital, Chiayi, Taiwan.

We present a case of a 22-year-old female with gross hematuria for 1 month. A 9.5-cm tumor was found at her left kidney. Read More

Zhonghua Bing Li Xue Za Zhi 2019 Feb;48(2):120-126

Department of Pathology, the Affiliated Hospital of Qingdao University, Qingdao 266003, China.

To investigate the clinicopathologic characteristics, molecular and genetic features, differential diagnoses and prognosis of fumarate hydratase-deficient renal cell carcinoma (FH-RCC). The immunohistochemical (IHC) expression of FH in 391 renal neoplasms in tissue chips collected from the Affiliated Hospital of Qingdao University and 971 Hospital of PLA Navy from January 2011 to December 2017 was evaluated. The clinicopathologic data of eight FH negative cases were collected. Read More

Histopathology 2019 Jan;74(1):4-17

Aquesta Uropathology, Brisbane, Qld, Australia.

Grading of renal cell carcinoma (RCC) has been recognised as a prognostic factor for almost 100 years. Numerous grading systems have been proposed, initially focusing upon a constellation of cytological features and more recently on nuclear morphology. It has been recommended that grading of RCC should be based upon nucleolar prominence/eosinophilia for grades 1-3, while grade 4 requires nuclear anaplasia (including tumour giant cells, sarcomatoid differentiation and/or rhabdoid morphology). Read More

J Kidney Cancer VHL 2018 13;5(4):6-13. Epub 2018 Oct 13.

Department of Genitourinary Oncology, H. Lee Moffitt Cancer Center and Research Institute, Tampa, FL, USA.

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. Read More

Histopathology 2019 Feb;74(3):377-390

Department of Pathology, NYU Langone Medical Center, New York, NY, USA.

Aims: The International Collaboration on Cancer Reporting (ICCR) has provided detailed data sets based upon the published reporting protocols of the Royal College of Pathologists, the Royal College of Pathologists of Australasia and the College of American Pathologists.

Methods And Results: The data set for carcinomas of renal tubular origin treated by nephrectomy was developed to provide a minimum structured reporting template suitable for international use, and incorporated recommendations from the 2012 Vancouver Consensus Conference of the International Society of Urological Pathology (ISUP) and the fourth edition of the World Health Organisation Bluebook on tumours of the urinary and male genital systems published in 2016. Reporting elements were divided into those, which are required and recommended components of the report. Read More

An Sist Sanit Navar 2018 Aug 29;41(2):191-199. Epub 2018 Aug 29.

Hospital San Jorge, Huesca.

Objetives. Our aim is to analyze and compare the clinico-pathological features in renal cell carcinomas (RCC) with sarcomatoid and rhaboid phenotype.

Material And Methods: We reviewed consecutive patients with nephrectomy RCC from January 1988 to January 2015. Read More

Cancer Immunol Res 2018 07 10;6(7):758-765. Epub 2018 May 10.

Dana-Farber Cancer Institute, Boston, Massachusetts.

Programmed death 1 (PD-1) and PD ligand 1 (PD-L1) inhibitors have shown activity in metastatic clear cell renal cell carcinoma (ccRCC). Data on the activity of these agents in patients with non-clear cell RCC (nccRCC) or patients with sarcomatoid/rhabdoid differentiation are limited. In this multicenter analysis, we explored the efficacy of PD-1/PD-L1 inhibitors in patients with nccRCC or sarcomatoid/rhabdoid differentiation. Read More

Int J Oncol 2018 Apr 28;52(4):1139-1148. Epub 2018 Feb 28.

Department of Urology, Tokai University School of Medicine, Isehara, Kanagawa 259-1193, Japan.

The aim of the present study was to evaluate the validity of potential prognostic parameters of clear cell renal cell carcinoma (ccRCC) recommended by the 2012 International Society of Urological Pathology (ISUP) Consensus Conference in the Japanese population. We reviewed 406 Japanese patients with localized or locally advanced ccRCC who underwent curative surgery during 2004-2014 at Tokai University Hospital (Isehara, Japan) and were followed up for >2 years after surgery. A single pathologist reviewed all the histological slides. Read More

Mod Pathol 2018 06 6;31(6):974-983. Epub 2018 Feb 6.

Réseau National pour Cancers Rares de l'Adulte PREDIR AP-HP labellisé par l'Institut National du Cancer (INCa), Hôpital de Bicêtre, 94275, Le Kremlin Bicêtre, France.

Hereditary leiomyomatosis and renal cell carcinoma syndrome is characterized by an increased risk of agressive renal cell carcinoma, often of type 2 papillary histology, and is caused by FH germline mutations. A prominent eosinophilic macronucleolus with a perinucleolar clear halo is distinctive of hereditary leiomyomatosis and renal cell carcinoma syndrome-associated renal cell carcinoma according to the 2012 ISUP and 2016 WHO kidney tumor classification. From an immunohistochemistry perspective, tumors are often FH-negative and S-(2-succino)-cysteine (2SC) positive. Read More

Am J Surg Pathol 2018 04;42(4):423-441

University Paris Diderot, INSERM, IAME, UMR_1137, Paris.

We developed and validated an architecture-based grading for clear cell renal cell carcinoma (ccRCC) in an observational retrospective cohort study including 506 tumors (principal cohort, n=254; validation cohort, n=252). Study endpoints were disease-free survival (DFS) and cancer-specific survival (CSS). Relationships with outcome were analyzed using Harrell concordance index, time-dependent receiver operating characteristic curve, area under curve, and Cox regression model. Read More

Hum Pathol 2017 09 15;67:134-145. Epub 2017 Jul 15.

Department of Pathology and Laboratory Medicine, Cedars-Sinai Medical Center, Los Angeles, CA, 90048, USA; Department of Pathology and Laboratory Medicine, University of Tennessee Health Sciences, Memphis, TN, 38163, USA. Electronic address:

Renal medullary carcinoma (RMC) is a highly aggressive renal cell carcinoma arising in the collecting system and requiring careful correlation with status of sickle cell trait. A panel of international experts has recently proposed provisional diagnostic terminology, renal cell carcinoma, unclassified, with medullary phenotype, based on encountering an extraordinarily rare tumor with RMC morphology and immunophenotype in an individual proven not to have a hemoglobinopathy. Herein, we extend this observation to a cohort of 5 such tumors, morphologically similar to RMC, lacking SMARCB1 expression by immunohistochemistry, but each without evidence of a hemoglobinopathy. Read More

Can J Urol 2017 Apr;24(2):8759-8764

Department of Urology, Yale School of Medicine, New Haven, Connecticut, USA.

Introduction: Evidence has demonstrated that tumor size is related to adverse oncologic outcomes in small renal tumors (≤ 4 cm). We evaluated the association of adverse pathologic features (APF) with tumor size and survival in patients with a small renal mass (SRM).

Materials And Methods: We retrospectively reviewed the pathologic characteristics of 380 surgically resected SRMs from a single institution. Read More

J Cardiovasc Ultrasound 2016 Dec 28;24(4):329-333. Epub 2016 Dec 28.

Department of Internal Medicine, Cardiovascular Center, Pusan National University Yangsan Hospital, Pusan National University School of Medicine, Yangsan, Korea.

Cardiac metastasis from renal cell carcinoma (RCC) without inferior vena cava (IVC) involvements is extremely rare with few reported cases. Sarcomatoid RCC with rhabdoid feature is a rare pathologic type of RCC having aggressive behavior due to great metastatic potential. Here, we report a case of rapidly growing cardiac metastasis of RCC which brought on right ventricular outflow tract (RVOT) obstruction without IVC and right atrial involvement in a 61-year-old woman. Read More

Pol J Pathol 2017;68(4):306-311

The disease entity of acquired cystic disease (ACD)-associated renal cell carcinoma (RCC) has been recently incorporated into the international renal tumor classification. However, there are a few descriptions on clinicopathologic features. We performed a clinicopathologic study of seven cases with ACD-RCC. Read More

Eur Urol 2017 01 11;71(1):e27-e28. Epub 2016 Jul 11.

Section of Pathological Anatomy, Polytechnic University of the Marche Region, School of Medicine, United Hospitals, Ancona, Italy. Electronic address:

Eur Urol 2017 01 11;71(1):e26. Epub 2016 Jul 11.

Servizo de Oncoloxía Médica, Complexo Hospitalario Universitario de Santiago de Compostela, Santiago de Compostela, Spain; Instituto de Investigación Sanitaria de Santiago de Compostela, Complexo Hospitalario Universitario de Santiago de Compostela, Santiago de Compostela, Spain.

Pathologe 2016 Jul;37(4):355-60

Institut für Pathologie und Molekularpathologie, UniversitätsSpital Zürich, Schmelzbergstrasse 12, 8091, Zürich, Schweiz.

Histological tumor grading is an accepted prognostic parameter of renal cell carcinoma (RCC). In 2012, the International Society of Urologic Pathologists (ISUP) proposed a novel grading system for RCC, mainly based on the evaluation of nucleoli: grade 1 tumors have nucleoli that are inconspicuous and basophilic at ×400 magnification; grade 2 tumors have nucleoli that are clearly visible at ×400 magnification and eosinophilic; grade 3 tumors have clearly visible nucleoli at ×100 magnification; and grade 4 tumors have extreme pleomorphism or rhabdoid and/or sarcomatoid morphology. This grading system was validated for clear cell renal cell carcinoma and papillary renal cell carcinoma. Read More

Int Urol Nephrol 2016 Aug 23;48(8):1253-1260. Epub 2016 May 23.

Glickman Urological and Kidney Institute, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH, 44016, USA.

Objective: Sarcomatoid and rhabdoid differentiation are associated with poor outcomes in renal cell carcinoma (RCC). We examined the impact of differentiation on cancer-specific survival (CSS) in surgically treated patients with grade 4 RCC.

Materials And Methods: Using our institutional database of 1176 nephrectomies from 2005 to 2013, we identified patients with grade 4 RCC or any grade and the presence of sarcomatoid or rhabdoid differentiation. Read More

JNMA J Nepal Med Assoc 2015 Apr-Jun;53(198):83-8

Rajiv Gandhi Cancer Institute and Research Centre, Delhi, India.

Introduction: Our study aims to evaluate the use of targeted therapy in metastatic renal cell carcinoma Methods: This is a prospective study done over three years from December 2010 to December, 2013.Out of Forty seven patients of metastatic renal cell carcinoma 8(neo-adjuvant cases) were excluded and 39 were included in this study. All patients received Tyrosine kinase inhibitor, sunitinib therapy (50 mg OD, 4/2 scheme). Read More

Eur Urol 2016 09 3;70(3):e75-6. Epub 2016 Mar 3.

Department of Hematology/Oncology, Fox Chase Cancer Center, Philadelphia, PA, USA. Electronic address:

Eur Urol 2016 09 3;70(3):e72-4. Epub 2016 Mar 3.

Section of Pathological Anatomy, Polytechnic University of the Marche Region, School of Medicine, United Hospitals, Ancona, Italy. Electronic address:

Am J Surg Pathol 2016 Feb;40(2):141-54

Robert J Tomsich Pathology and Laboratory Medicine Institute, Cleveland Clinic, Cleveland, OH.

Clear cell papillary renal cell carcinoma (CCPRCC) was recently included in the International Society of Urological Pathology Vancouver Classification of Renal Neoplasia as a subtype of RCC that is morphologically, immunohistochemically, and genetically distinct from both clear cell renal cell carcinoma (CCRCC) and papillary renal cell carcinoma. In our clinical practice we have observed tumors with overlapping histologic features of CCPRCC and CCRCC; therefore, our aim was to describe the morphologic, immunohistochemical, and clinical characteristics of these tumors. We examined a large series of consecutive nephrectomies diagnosed as CCRCC and found 37 tumors with morphologic overlap between CCRCC and CCPRCC, identifying 2 patterns. Read More

Histopathology 2016 Mar 16;68(4):475-81. Epub 2015 Nov 16.

Department of Pathology and Molecular Medicine, McMaster University, Hamilton, Ontario, Canada.

Grading is an important prognostic parameter for prostate adenocarcinoma and renal cell carcinoma (RCC); however, the most frequently used classifications fail to account for advances in our understanding of the diagnostic features, classification and/or behaviour of these tumours. In 2005 and 2014, the International Society of Urological Pathology (ISUP) proposed changes to Gleason scoring with the adoption of the ISUP grading for prostate cancer in 2014 (grade 1, score 3 + 3; grade 2, score 3 + 4; grade 3, score 4 + 3; grade 4, score 8; grade 5, score 9-10). Internationally the Fuhrman grading system is widely employed despite criticisms related to its application, validity, and reproducibility. Read More

Eur Urol 2015 Nov 18;68(5):912-4. Epub 2015 Jul 18.

Department of Hematology/Oncology, Fox Chase Cancer Center, Temple Health, Philadelphia, PA, USA. Electronic address:

Rev Med Inst Mex Seguro Soc 2015 Jul-Aug;53(4):454-65

Servicio de Anatomía Patológica, Centro Médico Nacional La Raza, Instituto Mexicano del Seguro Social, Distrito Federal, México.

Background: New morphologic entities for Renal Cell Carcinoma (RCC) that influence the prognosis have been described. Clinical staging has also undergone several modifications, the last one published in 2010 7th edition of the American Joint Committee on Cancer. The aim of this article is to determine the prevalence of histological subtypes, Fuhrman grading and clinical staging of renal cell carcinomas. Read More

Pol J Pathol 2015 Mar;66(1):3-8

Naoto Kuroda MD, Department of Pathology, Kochi Red Cross Hospital, Shin-honmachi 2-13-51, Kochi City,, Kochi 780-8562, Japan, tel. +81-88-822-1201, fax +81-88-822-1056, e-mail:

Rhabdoid morphology in renal cell carcinoma (RCC) may, like sarcomatoid change, be perceived as a type of dedifferentiation, and is a poor prognostic factor. Histologically, rhabdoid neoplastic cells are round to polygonal cells with globular eosinophilic cytoplasmic inclusions and eccentric vesicular nuclei and enlarged nucleoli. All types of RCC, including clear cell, papillary, chromophobe, collecting duct carcinoma, renal medullary carcinoma, acquired cystic disease-associated RCC, ALK-positive renal cancer and unclassified RCC, may display a variably prominent rhabdoid phenotype. Read More

Semin Diagn Pathol 2015 Mar 7;32(2):172-83. Epub 2015 Feb 7.

Department of Laboratory Medicine and Pathology, Mayo Clinic, 200 First St, Southwest Rochester, Minnesota 55905. Electronic address:

Outcome assessment for renal cell carcinoma is somewhat controversial. Despite numerous studies, a very limited variety of features have been recognized as having prognostic significance in clinical practice. In this review, tumor features considered to be of importance in outcome prediction for surgically treated patients with the 3 most commonly encountered morphotypes of renal cell carcinoma (clear cell, papillary, and chromophobe renal cell carcinoma) are evaluated. Read More

Ann Diagn Pathol 2015 Apr 2;19(2):57-63. Epub 2015 Feb 2.

Department of Urology, Peking University First Hospital, Institute of Urology, Peking University, Beijing, China.

Renal cell carcinoma (RCC) with rhabdoid differentiation is a recently described variant of RCC, which has seldom been reported in China. This form of differentiation has been generally associated with a poor prognosis and is often present in tumors with a poorly differentiated morphology. The development of a rhabdoid morphology appears to represent a common dedifferentiation pathway for renal parenchymal malignancies. Read More

Eur Urol 2015 Jul 28;68(1):5-7. Epub 2015 Jan 28.

Department of Oncology, Mayo Clinic, Rochester, MN, USA. Electronic address:

Renal cell carcinoma (RCC) with rhabdoid differentiation is thought to portend a poor prognosis, similar to RCC with sarcomatoid differentiation. Both features are currently classified as grade 4 RCC based on the most recent International Society of Urological Pathology (ISUP) grading system. We reviewed a large series of patients with grade 4 RCC to determine the differential effects of rhabdoid and sarcomatoid differentiation on patient outcome. Read More

Pathologe 2015 May;36(3):310-6

Institut für Pathologie, Universitätsklinikum Bonn, Sigmund-Freud-Str. 25, 53127, Bonn, Deutschland,

The 2012 consensus conference of the International Society of Urological Pathology (ISUP) has formulated recommendations on classification, prognostic factors and staging as well as immunohistochemistry and molecular pathology of renal tumors. Agreement was reached on the recognition of five new tumor entities: tubulocystic renal cell carcinoma (RCC), acquired cystic kidney disease-associated RCC, clear cell (tubulo) papillary RCC, microphthalmia transcription factor family RCC, in particular t(6;11) RCC and hereditary leiomyomatosis-associated RCC. In addition three rare forms of carcinoma were considered as emerging or provisional entities: thyroid-like follicular RCC, succinate dehydrogenase B deficiency-associated RCC and anaplastic lymphoma kinase (ALK) translocation RCC. Read More

Rare Tumors 2014 Jan 18;6(1):5037. Epub 2014 Mar 18.

Department of Pathology, Faculty of Medicine, Menofiya University , Shebein Elkom, Egypt.

Renal cell carcinoma (RCC) may metastasize anywhere in the body and sometimes the primary tumor is missing and necessitates extensive investigations to detect. In this report, we describe a case of RCC metastasizing to the thigh in a 70 year old male with a highly pleomorphic morphology suggesting a high grade sarcoma that showed unequivocal positivity for desmin directing the diagnosis for pleomorphic rhabdomyosarcoma. After completion of 33 cycles of radiotherapy, the patient developed large intraabdominal mass that showed conventional areas of RCC with immunoreactivity for CD10, CK, EMA, carbonic anhydrase IX and vimentin. Read More

Urology 2014 May 22;83(5):969-74. Epub 2014 Mar 22.

Department of Oncology-Pathology, Karolinska University Hospital, Solna, Stockholm, Sweden.

The International Society of Urological Pathology (ISUP) 2012 Consensus Conference made recommendations regarding the classification, prognostic factors, staging, and immunohistochemical and molecular assessment of adult renal tumors. There was consensus that 5 entities should be recognized as novel tumors: tubulocystic renal cell carcinoma (RCC), acquired cystic disease-associated RCC, clear cell papillary RCC, microphthalmia transcription factor-family translocation RCC [in particular t(6; 11) RCC], and hereditary leiomyomatosis RCC syndrome-associated RCC. In addition, 3 rare epithelial carcinomas were considered emerging or provisional entities: thyroid-like follicular RCC, succinate dehydrogenase B deficiency-associated RCC, and anaplastic lymphoma kinase translocation RCC. Read More

Am J Surg Pathol 2014 Apr;38(4):567-77

Departments of *Pathology #Urology †Internal Medicine, Division of Hematology and Oncology ‡Comprehensive Cancer Center, University of Michigan Health System ∥Michigan Center for Translational Pathology ¶Howard Hughes Medical Institute, Ann Arbor, MI §Department of Pathology, Memorial Sloan-Kettering Cancer Center, New York, NY.

Rapid ("warm") autopsies of patients with advanced metastatic cancer provide invaluable insight into the natural history, pathobiology, and morphology of advanced and treatment-resistant tumors. Here, we report a rapid autopsy case of a hereditary leiomyomatosis and renal cell carcinoma (HLRCC) patient with advanced metastatic renal cell carcinoma (RCC)-the first such case described for either a primary renal tumor or HLRCC-related cancer. Mutations in the fumarate hydratase (FH) gene underlie HLRCC, a rare syndrome involving cutaneous and uterine leiomyomata and aggressive kidney tumors. Read More

Ann Saudi Med 2013 Sep-Oct;33(5):495-9

Dr. Mohammed Akhtar, King Faisal Specialist Hospital and Research Center, Pathology & Laboratory Medicine, DPLM, MBC 10 PO Box 3354 Riyadh 11211 Saudi Arabia, T: +966-11-4424280, F: +966-11-4424280,

Sarcomatoid renal cell carcinoma (SRCC) with rhabdoid features is a rare tumor with aggressive behavior and poor prognosis. We report a case of a 71-year-old man with a large left-sided renal mass. Nephrectomy specimen revealed clear cell carcinoma with sarcomatoid and rhabdoid tumor cells. Read More

Am J Surg Pathol 2013 Oct;37(10):1490-504

*Department of Pathology and Molecular Medicine, Wellington School of Medicine and Health Sciences, University of Otago, Wellington, New Zealand †Department of Pathology, Mayo Clinic, Rochester, MN §Department of Pathology and Immunology, Washington University School of Medicine, St Louis, MO ∥Department of Anatomic Pathology, Cancer Biology and Glickman Urological Institute, Cleveland, OH ††Department of Pathology, Indiana University School of Medicine, Indianapolis, IN ‡Dipartimento di Patologia e Diagnostica, Universitá di Verona, Verona ‡‡The Institute of Pathological Anatomy and Histopathology, University of Ancona School of Medicine, Ancona, Italy ¶Department of Oncology and Pathology, Karolinska University Hospital, Solna, Stockholm, Sweden #Department of Pathology, Fundacion Puigvert-University Antonomous, Barcelona, Spain **University of Zurich, Switzerland §§Department of Laboratory Medicine, Credit Valley Hospital, Mississauga ∥∥Department of Pathology and Molecular Medicine, McMaster University, Toronto, ON, Canada.

The International Society of Urological Pathology 2012 Consensus Conference made recommendations regarding classification, prognostic factors, staging, and immunohistochemical and molecular assessment of adult renal tumors. Issues relating to prognostic factors were coordinated by a workgroup who identified tumor morphotype, sarcomatoid/rhabdoid differentiation, tumor necrosis, grading, and microvascular invasion as potential prognostic parameters. There was consensus that the main morphotypes of renal cell carcinoma (RCC) were of prognostic significance, that subtyping of papillary RCC (types 1 and 2) provided additional prognostic information, and that clear cell tubulopapillary RCC was associated with a more favorable outcome. Read More

BMJ Case Rep 2013 Apr 22;2013. Epub 2013 Apr 22.

Department of Pathology, Howard University Hospital, Washington, District of Columbia, USA.

Renal cell carcinoma (RCC), the most common malignancy of kidney, originates from renal tubular epithelium. It is subclassified based on histological and molecular features. Rarely, RCC can show focal to extensive sarcomatoid or rhabdoid differentiation. Read More

Int J Clin Exp Pathol 2013 15;6(4):724-8. Epub 2013 Mar 15.

Department of Pathology, Shizuoka City Shimizu Hospital, Shizuoka, Japan.

The author presents a unique case of multiple cytokeratin-negative malignant tumors consisting only of rhabdoid cells in the renal pelvis. A 54-year-old man complained of hematuria. A transurethral endoscopic examination revealed multiple papillary tumors, and transurethral resection of the bladder tumors was performed. Read More

Int J Urol 2013 Sep 4;20(9):923-30. Epub 2013 Feb 4.

Department of Urology, Kochi University, Nankoku City, Japan.

Objectives: To investigate the potential mechanism of development of resistance to tyrosine kinase inhibitor in renal cell carcinoma.

Methods: A primary culture of renal cell carcinoma cells (KMRM-S2) was established from an advanced renal cell carcinoma patient with cutaneous metastasis, who had not responded to sorafenib. A total of 84 human angiogenesis-related genes were compared between cutaneous metastasis and the primary tumor by real time polymerase chain reaction. Read More

Am J Surg Pathol 2013 Mar;37(3):311-22

Department of Pathology and Molecular Medicine, Wellington School of Medicine and Health Sciences, University of Otago, Wellington, New Zealand.

Grading of renal cell carcinoma (RCC) has prognostic significance, and there is recent consensus by the International Society of Urological Pathology (ISUP) that for clear cell and papillary RCC, grading should primarily be based on nucleolar prominence. Microscopic tumor necrosis also predicts outcome independent of tumor grading. This study was undertaken to assess whether the incorporation of microscopic tumor necrosis into the ISUP grading system provides survival information superior to ISUP grading alone. Read More

Cancer 2012 Sep 17;118(18):4427-36. Epub 2012 Jan 17.

Japanese Foundation for Cancer Research, Tokyo, Japan.

Background: Several promising molecular-targeted drugs are used for advanced renal cancers. However, complete remission is rarely achieved, because none of the drugs targets a key molecule that is specific to the cancer, or is associated with "oncogene addiction" (dependence on one or a few oncogenes for cell survival) of renal cancer. Recently, an anaplastic lymphoma kinase (ALK) fusion, vinculin-ALK, has been reported in pediatric renal cell carcinoma (RCC) cases who have a history of sickle cell trait. Read More

Ann Diagn Pathol 2011 Oct 12;15(5):333-7. Epub 2011 Jun 12.

Department of Pathology, University of Miami Miller School of Medicine, Sylvester Comprehensive Cancer Center, FL 33138, USA.

Renal cell carcinoma (RCC) with rhabdoid morphology (RCC-RM) is a recently described variant of RCC, which has an aggressive biologic behavior and poor prognosis, akin to sarcomatoid RCC. The current World Health Organization classification of RCC does not include the rhabdoid phenotype as a distinct histologic entity. The aim of this study is to investigate whether RCC-RM represents a dedifferentiation of a classifiable-type World Health Organization RCC or a carcinosarcoma with muscle differentiation. Read More