scholarly journals ESC, ALK, HOT and LOT: Three Letter Acronyms of Emerging Renal Entities Knocking on the Door of the WHO Classification

Cancers ◽  
2020 ◽  
Vol 12 (1) ◽  
pp. 168 ◽  
Author(s):  
Farshid Siadat ◽  
Kiril Trpkov
Keyword(s):  
Renal Cell ◽  
Who Classification ◽  
Renal Tumor ◽  
Current Knowledge ◽  
World Health ◽  
Renal Tumors ◽  
Low Grade ◽  
Kidney Tumors ◽  
Anaplastic Lymphoma

Kidney neoplasms are among the most heterogeneous and diverse tumors. Continuous advancement of this field is reflected in the emergence of new tumour entities and an increased recognition of the expanding morphologic, immunohistochemical, molecular, epidemiologic and clinical spectrum of renal tumors. Most recent advances after the 2016 World Health Organization (WHO) classification of renal cell tumors have provided new evidence on some emerging entities, such as anaplastic lymphoma kinase rearrangement-associated RCC (ALK-RCC), which has already been included in the WHO 2016 classification as a provisional entity. Additionally, several previously unrecognized entities, not currently included in the WHO classification, have also been introduced, such as eosinophilic solid and cystic renal cell carcinoma (ESC RCC), low-grade oncocytic renal tumor (LOT) and high-grade oncocytic renal tumor (HOT) of kidney. Although pathologists play a crucial role in the recognition and classification of these new tumor entities and are at the forefront of the efforts to characterize them, the awareness and the acceptance of these entities among clinicians will ultimately translate into more nuanced management and improved prognostication for individual patients. In this review, we summarise the current knowledge and the novel data on these emerging renal entities, with an aim to promote their increased diagnostic recognition and better characterization, and to facilitate further studies that will hopefully lead to their formal recognition and consideration in the future classifications of kidney tumors.

scholarly journals The ISUP system of staging, grading and classification of renal cell neoplasia

2014 ◽  
Vol 1 (3) ◽  
pp. 26-39 ◽  
Author(s):  
Hemamali Samaratunga ◽  
Troy Gianduzzo ◽  
Brett Delahunt
Keyword(s):  
Renal Cell ◽  
Who Classification ◽  
Majority Vote ◽  
Cell Cancer ◽  
Consensus Conference ◽  
World Health ◽  
Renal Tumors ◽  
International Consensus ◽  
Staging Classification

There have been significant changes in the staging, classification and grading of renal cell neoplasia in recent times. Major changes have occurred in our understanding of extra-renal extension by renal cell cancer and how gross specimens must be handled to optimally display extra-renal spread. Since the 1981 World Health Organization (WHO) classification of renal tumors, in which only a handful of different entities were reported, many new morphological types have been described in the literature, resulting in 50 different entities reported in the 2004 WHO classification. Since 2004, further new entities have been recognized and reported necessitating an update of the renal tumor classification. There have also been numerous grading systems for renal cell carcinoma with Fuhrman grading, the most widely used system. In recent times, the prognostic value and the applicability of the Fuhrman grading system in practice has been shown to be, at best, suboptimal. To address these issues and to recommend reporting guidelines, the International Society of Urological Pathology (ISUP) undertook a review of adult renal neoplasia through an international consensus conference in Vancouver in 2012. The conduct of the conference was based upon evidence from the literature and the current practice amongst recognized experts in the field. Working groups selected to deal with key topics evaluated current data and identified points of controversy. A pre-meeting survey of the ISUP membership was followed by the consensus conference at which a formal ballot was taken on each key issue. A 65% majority vote was taken as consensus. This review summarizes the outcome and recommendations of this conference with regards to staging, classification and grading of renal cell neoplasia.

Validation of the 2016 Revision to the World Health Organization (WHO) Classification of Myelodysplastic Syndromes with Diploid Karyotype

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 4319-4319
Author(s):  
Rashmi Kanagal-Shamanna ◽  
Juliana E. Hidalgo Lopez ◽  
Hye Ryoun Kim ◽  
Zhuang Zuo ◽  
Michelle Janania Martinez ◽  
...  
Keyword(s):  
Platelet Count ◽  
Mutation Analysis ◽  
Research Funding ◽  
Who Classification ◽  
Older Age ◽  
World Health ◽  
Morphological Evidence ◽  
Low Grade ◽  
Transformation Rate

Abstract Introduction: The revised 2016 WHO classification of MDS has highlighted the value of morphologic evaluation and mutation analysis of bone marrow (BM)/ peripheral blood (PB) to further refine prognostication. These highlights include: (1) increased emphasis on lineage dysplasia compared with cytopenias; (2) objective enumeration of blast % for reproducibility; (3) accurate quantification of ring sideroblasts (RS); and (4) mutation analysis for SF3B1 in cases showing RS >5% and TP53 in MDS with isolated del(5q). Most of the proposed changes are within the categories of low-grade MDS. In this study, we evaluated 264 cases of MDS with diploid karyotype using the 2016 WHO system. Methods: We selected consecutive cases of MDS with diploid karyotype with BM morphological evidence of dysplasia and reclassified using the 2016 WHO system. Mutation analysis for SF3B1 (exons 14 and 15), SRSF2 (exon 1) and U2AF1 (exons 2 and 6) was performed using Sanger sequencing. Patient data were collected from the medical record. The Kaplan-Meier method was used to estimate OS and time-to-AML transformation. The associations between outcome and clinical and pathological parameters were determined using univariate and multivariate Cox proportional hazards regression models. Results: The study group included 264 MDS patients: 168 (64%) men and 96 (36%) women with a median age of 66.9 years (range, 28.3 - 89.1). The median hemoglobin, absolute neutrophil count (ANC), platelet count, and white blood cell (WBC) count were 10.0 g/dL, 1.9 x 109/L, 114.5 x 109/L, and 3.5 x 109/L, respectively. The median BM blast percentage was 2.5; 74% of the patients had < 5% BM blasts. MDS sub-classification according to the 2008 WHO classification was: RCUD, n=5 (2%); RA, n=9 (3%); RARS, n=16 (6%); RCMD, n=152 (58%); RAEB-1, n=56 (21%); RAEB-2, n=20 (8%), and MDS-U, n=6 (2%). Reclassification using the 2016 WHO classification: MDS with single lineage dysplasia (MDS-SLD, n=14, 5%), MDS with multi-lineage dysplasia (MDS-MLD, n=112, 42%), MDS with RS (including single lineage and multi-lineage dysplasia, MDS-RS, n=56, 21%); MDS-EB1, n=56 (21%), MDS-EB2, n=20 (8%) and MDS-U, n=6 (2%). Grading of fibrosis using reticulin/ trichrome stains showed absent-minimal fibrosis (grades 0-1) in 56/85 (66%) and moderate-severe fibrosis (2-3) in 29/85 (34%) cases. Mutation analysis for splicing factors was performed on 15 cases. Ten cases with 0-5% RS showed 2 cases each with SRSF2 and U2AF1 mutations. No cases had SF3B1 mutation. 5 cases with >5% RS showed SF3B1 mutations in 4 cases and 1 case each with SRSF2 and U2AF1 mutations. Over a median follow-up duration of 22.4 months (range, 0-156.8), 128 (48%) patients died. The median OS was 46.1 months (95% CI: 32.3, 58.4). Patients categorized as MDS-SLD by 2016 WHO had the best OS (156.8 months), followed by MDS-RS (58.7 months), MDS-MLD (46.3 months) and MDS-EB (21.2 months) (p<0.001). Older age, lower hemoglobin, lower ANC, lower platelet count, ≥5% BM blasts, MDS-EB1 and MDS-EB2 by 2016 WHO were significantly associated with worse OS (≤0.044). Accounting for all significant measures, age, hemoglobin, MDS-EB1 and MDS-EB2 remained significantly associated with OS.Sixteen patients transformed to AML; the median time-to-AML transformation was not reached; 5-year AML transformation rate was 88%. Patients with ≥ 5% BM blasts (p=0.056) and older age (p=0.063) tended to have a higher AML transformation rate. Conclusions: Morphological evaluation of BM/PB (for dysplasia, % BM blasts and RS) provides additional prognostic value and continues to be a critical component for evaluation of MDS patients. Molecular studies for splicing factor mutations are ongoing on all samples with >1% RS. Figure Figure. Disclosures Jabbour: ARIAD: Consultancy, Research Funding; Pfizer: Consultancy, Research Funding; Novartis: Research Funding; BMS: Consultancy.

scholarly journals Classification of renal cell tumors – current concepts and use of ancillary tests: recommendations of the Brazilian Society of Pathology

2021 ◽  
Vol 4 (1) ◽  
Author(s):  
Daniel Abensur Athanazio ◽  
Luciana Schultz Amorim ◽  
Isabela Werneck da Cunha ◽  
Katia Ramos Moreira Leite ◽  
Alexandre Rolim da Paz ◽  
...  
Keyword(s):  
Renal Cell ◽  
Who Classification ◽  
Recent Literature ◽  
Underlying Disease ◽  
Genetic Alterations ◽  
Renal Tumors ◽  
Specific Gene ◽  
Brazilian Society ◽  
Resource Limited

AbstractClassification of renal cell carcinomas has become more challenging. The 2016 WHO classification included 14 different subtypes and 4 emerging/provisional entities, and recent literature indicates new entities to be incorporated. Nomenclature is based on cytoplasmic appearance, architecture, combination of morphologies, anatomic location, underlying disease, familial syndromes, and specific genetic alterations. Immunohistochemistry is useful in selected cases while it can be insufficient in entities that require molecular confirmation of a specific gene alteration. The aim of these recommendations is to provide a reasonable and optimized approach for the use of ancillary tests in subtyping renal tumors, particularly in resource-limited settings.

The World Health Organization's Revised Classification of Tumours of the Larynx, Hypopharynx, and Tracheax

1993 ◽  
Vol 102 (9) ◽  
pp. 666-669 ◽  
Author(s):  
Alfio Ferlito
Keyword(s):  
Respiratory Tract ◽  
Who Classification ◽  
Current Knowledge ◽  
World Health ◽  
Histological Classification ◽  
Upper Respiratory Tract ◽  
Histological Typing ◽  
The World ◽  
Upper Respiratory

A second edition of the Histological Typing of Upper Respiratory Tract Tumours in the WHO series International Histological Classification of Tumours was published in 1991. The new edition has been entitled Histological Typing of Tumours of the Upper Respiratory Tract and Ear. The task of revising the first edition, which was published in 1978, was undertaken at the WHO Center for Upper Respiratory Tract Tumours by K. Shanmugaratnam in collaboration with L. H. Sobin and pathologists in 8 countries. Several tumour types have been added to the classification, and some have been redefined in light of current knowledge. This presentation outlines the changes in the revised WHO classification as regards tumours of the larynx, hypopharynx, and trachea and discusses the grounds for said revisions.

scholarly journals Low-Grade Rhabdoid Meningioma: Unusual Morphological Characteristics

2013 ◽  
Vol 13 (2) ◽  
pp. 84-86
Author(s):  
Arvids Jakovlevs ◽  
Andrejs Vanags ◽  
Janis Gardovskis ◽  
Ilze Strumfa
Keyword(s):  
Who Classification ◽  
Morphological Characteristics ◽  
World Health ◽  
Low Grade ◽  
Unique Case ◽  
Rare Type ◽  
Rhabdoid Meningioma ◽  
The Central Nervous System ◽  
Health Organization

Abstract Rhabdoid meningioma (RM) is a rare type of meningioma. It is classified as a grade III tumour (anaplastic meningioma) in the recent World Health Organization (WHO) classification of the tumours of the central nervous system (CNS). Here we describe a unique case of RM lacking any features of malignancy. Few cases of low-grade RMs are described in the literature in contrast with the grading of this entity in WHO classification.

scholarly journals MPC-17 2021 WHO Classification of Tumors of the CNS, 5th ed

2021 ◽  
Vol 3 (Supplement_6) ◽  
pp. vi18-vi18
Author(s):  
Takashi Komori
Keyword(s):  
Molecular Markers ◽  
Risk Stratification ◽  
Who Classification ◽  
World Health ◽  
Grading System ◽  
Low Grade ◽  
Integrated Diagnosis ◽  
Classification Of Tumors ◽  
Tumor Types

Abstract The grading of gliomas based on histological features has been a subject of debate for several decades. While the traditional grading system has failed to stratify the risk of IDH-mutant astrocytoma, canonical histological and proliferative markers may be applicable to the risk stratification of IDH-wildtype astrocytoma. Numerous studies have examined molecular markers to obtain more clinically relevant information that will improve the risk stratification of gliomas. The CDKN2A/B homozygous deletion for IDH-mutant astrocytoma and the following three criteria for IDH-wildtype astrocytoma: the concurrent gain of whole chromosome 7 and loss of whole chromosome 10, TERT promoter mutations, and EGFR amplification, were identified as independent molecular markers of the worst clinical outcomes. Therefore, the 2021 World Health Organization (WHO) Classification of Tumors of the Central Nervous System adopted these molecular markers into the revised grading criteria of IDH-mutant and -wildtype astrocytoma respectively, as a grading system within tumor types. For diffuse gliomas in children, molecular alteration-based classification was adopted, dividing low-grade and high-grade subcategories. New tumor types and subtypes were introduced, some based on DNA methylation profiling. To achieve this novel classification in a resource-limited setting, an integrated diagnosis combining clinical, histological, and molecular information became more important.

scholarly journals Multilocular Cystic Renal Cell Carcinoma: A Rare Entity with Review of Literature

2014 ◽  
Vol 6 (01) ◽  
pp. 050-052 ◽  
Author(s):  
Shailja Puri Wahal ◽  
Kavita Mardi
Keyword(s):  
Renal Cell Carcinoma ◽  
Cell Carcinoma ◽  
Renal Cell ◽  
Who Classification ◽  
Clear Cell ◽  
World Health ◽  
Renal Tumors ◽  
Cystic Nephroma ◽  
Cell Renal Cell Carcinoma ◽  
Cystic Renal Cell Carcinoma

ABSTRACTMultilocular cystic renal cell carcinoma (MCRCC) represents a rare variant of clear cell renal cell carcinoma (RCC). MCRCC has been recognized as a separate subtype of RCC in the 2004 World Health Organization (WHO) classification of adult renal tumors. MCRCC is diagnosed on the basis of strict histological criteria according to 2004 WHO classification. The chief differentials diagnosis to be considered include cystic nephroma, cystic clear cell carcinoma, clear cell papillary renal cell carcinoma and tubulocystic carcinoma. Only few cases of MCRCC are reported in literature. This case is being highlighted for its rarity and so as to avoid a misdiagnosis as conventional RCC.

scholarly journals Tubulocystic Renal Cell Carcinoma: A Rare Renal Tumor

2014 ◽  
Vol 1 (5) ◽  
pp. 56-62 ◽  
Author(s):  
Jasneet Singh Bhullar ◽  
Sandiya Bindroo ◽  
Neha Varshney ◽  
Vijay Mittal
Keyword(s):  
Renal Cell Carcinoma ◽  
Cell Carcinoma ◽  
Renal Cell ◽  
Renal Tumor ◽  
Renal Tumors ◽  
Extensive Literature ◽  
Vancouver Classification ◽  
Distinct Entity ◽  
Extensive Literature Search

Tubulocystic renal cell carcinoma of the kidney is a rare entity with less than one hundred cases reported so far. It was previously considered to have some similarities to various other renal cancers although this tumor has distinct macroscopic, microscopic and immuno-histochemical features. It is now a well-established entity in renal neoplastic pathology and has been recognized as a distinct entity in the 2012 Vancouver classification of renal tumors. This review aims to give an overview of tubulocystic renal cell carcinoma after extensive literature search using PubMed and CrossRef. 

scholarly journals The DEAD/DEAH Box Helicase, DDX11, Is Essential for the Survival of Advanced Clear Cell Renal Cell Carcinoma and Is a Determinant of PARP Inhibitor Sensitivity

Cancers ◽  
2021 ◽  
Vol 13 (11) ◽  
pp. 2574
Author(s):  
Jee Soo Park ◽  
Myung Eun Lee ◽  
Won Sik Jang ◽  
Koon Ho Rha ◽  
Seung Hwan Lee ◽  
...  
Keyword(s):  
Renal Cell Carcinoma ◽  
Cell Carcinoma ◽  
Renal Cell ◽  
Quantitative Polymerase Chain Reaction ◽  
Parp Inhibitor ◽  
Renal Tumors ◽  
Low Grade ◽  
Normal Kidney ◽  
The Dead ◽  
The Impact

Genes associated with the DEAD-box helicase DDX11 are significant biomarkers of aggressive renal cell carcinoma (RCC), but their molecular function is poorly understood. We analyzed the molecular pathways through which DDX11 is involved in RCC cell survival and poly (ADP-ribose) polymerase (PARP) inhibitor sensitivity. Immunohistochemistry and immunoblotting determined DDX11 expression in normal kidney tissues, benign renal tumors, and RCC tissues and cell lines. Quantitative polymerase chain reaction validated the downregulation of DDX11 in response to transfection with DDX11-specific small interfering RNA. Proliferation analysis and apoptosis assays were performed to determine the impact of DDX11 knockdown on RCC cells, and the relevant effects of sunitinib, olaparib, and sunitinib plus olaparib were evaluated. DDX11 was upregulated in high-grade, advanced RCC compared to low-grade, localized RCC, and DDX11 was not expressed in normal kidney tissues or benign renal tumors. DDX11 knockdown resulted in the inhibition of RCC cell proliferation, segregation defects, and rapid apoptosis. DDX11-deficient RCC cells exhibited significantly increased sensitivity to olaparib compared to sunitinib alone or sunitinib plus olaparib combination treatments. Moreover, DDX11 could determine PARP inhibitor sensitivity in RCC. DDX11 could serve as a novel therapeutic biomarker for RCC patients who are refractory to conventional targeted therapies and immunotherapies.

The Diagnostic Accuracy of Percutaneous Renal Needle Core Biopsy and Its Potential Impact on the Clinical Management of Renal Cortical Neoplasms

2014 ◽  
Vol 138 (12) ◽  
pp. 1673-1679 ◽  
Author(s):  
Lan L. Gellert ◽  
Rohit Mehra ◽  
Ying-Bei Chen ◽  
Anuradha Gopalan ◽  
Samson W. Fine ◽  
...  
Keyword(s):  
Clinical Management ◽  
Renal Cell ◽  
Core Biopsy ◽  
Clear Cell ◽  
Renal Tumors ◽  
Low Grade ◽  
Renal Cell Carcinomas ◽  
Biopsy Diagnosis ◽  
Potential Impact ◽  
Renal Cortical Neoplasms

Context While biopsies are now increasingly being performed for the diagnosis of renal cortical neoplasms, the influence of the rendered pathological diagnoses on the clinical management is only rarely documented. Objectives To report our experience with consecutively performed renal biopsies and the potential impact of the diagnosis on subsequent clinical management. Design Material from needle biopsies performed consecutively at our institution between 2006 and 2011 was reviewed. The influence of the reported pathology results on the clinical management was determined from patient follow-up medical record review. Results In total, 218 percutaneous biopsies for renal masses were performed during this period. Among them, 181 (83%) yielded neoplastic tissue, including 81 clear cell renal cell carcinomas, 29 low-grade oncocytic neoplasms, 7 papillary renal cell carcinomas, 5 clear cell papillary renal cell carcinomas, 5 angiomyolipomas, and 14 urothelial carcinomas. Fourteen additional cases (6%) contained lesional material from clinically known nonneoplastic processes, for a total diagnostic yield of 89%. Twenty-three (11%) were nonrepresentative of lesional tissue. In 10 of these, repeat biopsies or resections established the diagnosis of renal tumors. Biopsy diagnosis was confirmed in 29 of 30 cases (97%) on subsequent nephrectomy. Following the biopsy diagnosis, there were significant differences in the clinical management; overall, 79% of clear cell renal cell carcinomas received therapeutic interventions, and 17% were put on active surveillance. In contrast, 77% of the benign or low-grade lesions were put on active surveillance. Conclusions Accurate and specific diagnosis can be rendered on renal core biopsy in most renal tumors, and the biopsy diagnosis can have a definitive role in their clinical management.

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