Journal
CANCER TREATMENT REVIEWS
Volume 97, Issue -, Pages -Publisher
ELSEVIER SCI LTD
DOI: 10.1016/j.ctrv.2021.102191
Keywords
Renal carcinoma; Non-clear cell carcinomas; Histological subtypes; Classification; Unclassified; Genetic and molecular alterations
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Funding
- Pfizer SAS
- Merck Sante S.A.S., an affiliate of Merck KGaA, Darmstadt, Germany
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Non-clear cell renal cell carcinomas (nccRCC) are a highly heterogeneous group of kidney tumors, often misclassified and challenging to accurately diagnose. Current therapeutic strategies for metastatic nccRCC remain poorly defined due to the lack of supportive evidence from clinical trials for each histological subtype.
Non-clear cell renal cell carcinomas (nccRCC) represent a highly heterogeneous group of kidney tumors, consisting of the following subtypes: papillary carcinomas, chromophobe renal cell carcinoma, so-called unclassified carcinomas or aggressive uncommon carcinomas such as Bellini carcinoma, renal cell carcinoma (RCC) with ALK rearrangement or fumarate hydratase-deficient RCC. Although non-clear cell cancers account for only 15 to 30% of renal tumors, they are often misclassified and accurate diagnosis continues to be an issue in clinical practice. Current therapeutic strategy of metastatic nccRCC is based primarily on guidelines established for clear cell tumors, the most common subtype, however this approach remains poorly defined. To date, published clinical trials for all histological nccRCC subtypes have been collectively characterized into one group, in contrast to clear cell RCC, and given the small numbers of cases, the interpretation of study results continues to be challenging. This review summarizes the available literature for each nccRCC subtype and highlights the lack of supportive evidence from prospective clinical trials and retrospective studies. Future trials should evaluate treatment approaches which focus on a specific histological subtype and progress in treating nccRCC will be contingent on understanding the unique biology of their individual histologies.
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