Article ; Online: Update on Selected High-grade Renal Cell Carcinomas of the Kidney: FH-deficient, ALK-rearranged, and Medullary Carcinomas.
Advances in anatomic pathology
2023 Volume 31, Issue 2, Page(s) 118–125
Abstract: High-grade renal cell carcinoma (RCC), often diagnosed at advanced stages, significantly contributes to renal cancer-related mortality. This review explores the progress in understanding specific subtypes of high-grade RCC, namely fumarate hydratase (FH)- ...
Abstract | High-grade renal cell carcinoma (RCC), often diagnosed at advanced stages, significantly contributes to renal cancer-related mortality. This review explores the progress in understanding specific subtypes of high-grade RCC, namely fumarate hydratase (FH)-deficient RCC, anaplastic lymphoma kinase (ALK)-rearranged RCC, and SMARCB1-deficient renal medullary carcinoma, all of which are now recognized as molecularly defined entities in the WHO classification system (2022). While these entities each exhibit a morphologic spectrum that overlaps with other high-grade RCC, ancillary tools developed based on their distinctive molecular alterations can help establish a specific diagnosis, underscoring the importance of integrating molecular findings into diagnostic paradigms. It is important to exclude these specific tumor types in cases with similar morphologic spectrum before rendering a diagnosis of high-grade papillary RCC, collecting duct carcinoma, or RCC, NOS. Several gray areas exist within the spectrum of high-grade uncommon types of RCC, necessitating continued research to enhance diagnostic precision and therapeutic options. |
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MeSH term(s) | Humans ; Carcinoma, Renal Cell/pathology ; Carcinoma, Medullary ; Anaplastic Lymphoma Kinase ; Fumarate Hydratase ; Kidney/pathology ; Kidney Neoplasms/pathology |
Chemical Substances | Anaplastic Lymphoma Kinase (EC 2.7.10.1) ; Fumarate Hydratase (EC 4.2.1.2) |
Language | English |
Publishing date | 2023-12-25 |
Publishing country | United States |
Document type | Review ; Journal Article |
ZDB-ID | 1212493-x |
ISSN | 1533-4031 ; 1072-4109 |
ISSN (online) | 1533-4031 |
ISSN | 1072-4109 |
DOI | 10.1097/PAP.0000000000000426 |
Database | MEDical Literature Analysis and Retrieval System OnLINE |
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