Abstract
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.
Highlights
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
Great majority of eosinophilic solid and cystic renal cell carcinoma (ESC RCC) are sporadic and are found in non-syndromic setting, a subset of identical tumors have been documented in patients with a tuberous sclerosis complex (TSC) [5,6]
high-grade oncocytic renal tumor (HOT) of kidney emerged from the spectrum of oncocytic tumours that include renal oncocytoma, chromophobe renal cell carcinoma (Chr RCC), as well as previously reported tumors with “hybrid” morphology of oncocytoma-Chr RCC, either as sporadic “hybrid-oncocytic” tumors, or tumors found in a syndromic setting, such as Birt–Hogg–Dubé (BHD) syndrome, renal oncocytosis and TSC [5,38,39,40,41,42]
Summary
LOT is typically a single and sporadic tumor, found in a non-syndromic setting and in older patients (median age 66 years). During the follow-up, these tumors behaved indolently, with no evidence of disease progression, this is based on a single study with a relatively limited follow-up (mean 31.8 months) [44]
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