Kidney Tumors: New and Emerging Kidney Tumor Entities.

This review summarizes current knowledge on several novel and emerging renal entities, including eosinophilic solid and cystic renal cell carcinoma (RCC), RCC with fibromyomatous stroma, anaplastic lymphoma kinase-rearranged RCC, low-grade oncocytic renal tumor, eosinophilic vacuolated tumor, thyroidlike follicular RCC, and biphasic hyalinizing psammomatous RCC. Their clinical features, gross and microscopic morphology, immunohistochemistry, and molecular and genetic features are described. The diagnosis of most of them rests on recognizing their morphologic features using immunohistochemistry. Accurate diagnosis of these entitles will further reduce the category of "unclassifiable renal carcinomas/tumors" and will lead to better clinical management and improved patient prognostication.

Kidney Tumors: New and Emerging Kidney Tumor Entities.

This review summarizes current knowledge on several novel and emerging renal entities, including eosinophilic solid and cystic renal cell carcinoma (RCC), RCC with fibromyomatous stroma, anaplastic lymphoma kinase-rearranged RCC, low-grade oncocytic renal tumor, eosinophilic vacuolated tumor, thyroidlike follicular RCC, and biphasic hyalinizing psammomatous RCC. Their clinical features, gross and microscopic morphology, immunohistochemistry, and molecular and genetic features are described. The diagnosis of most of them rests on recognizing their morphologic features using immunohistochemistry. Accurate diagnosis of these entitles will further reduce the category of "unclassifiable renal carcinomas/tumors" and will lead to better clinical management and improved patient prognostication.

Extrarenal renal cell carcinoma arising in the kidney proximity but without an identifiable renal primary - an intriguing dilemma: report of three cases and review of the literature.

AIM: To raise awareness of the existence of extrarenal renal cell carcinoma (RCC). METHODS AND RESULTS: We report three patients with extrarenal RCC found in the renal proximity, but unattached to the kidney. None had a history of RCC or an identifiable primary renal neoplasm at the time of the diagnosis and on follow-up. The patients included two males and one female aged 57, 77, and 63 years, respectively. One carcinoma was found in the perirenal tissue adjacent to the adrenal, one involved the adrenal gland, and one was a retroperitoneal mass found within the lymph nodes. Two extrarenal RCCs represented clear-cell RCCs and one was an unclassifiable RCC. No patient had evidence of metastases at presentation and disease progression during the follow-up. This report adds to the literature on this unusual clinical scenario and further supports the concept of extrarenal RCC, which is not a well-recognized clinical phenomenon. We also reviewed other similar reports documenting the absence of identifiable renal primaries in the setting of either disseminated metastatic disease or isolated distant metastases of presumed renal origin. Similarly, some carcinomas of apparent renal derivation have been also identified during a work-up of metastatic carcinomas of unknown primary. CONCLUSIONS: There should be an awareness of this unusual and intriguing clinical scenario that currently lacks a definitive explanation and standardized therapy strategies. Establishing a correct diagnosis may allow treatment with specific targeted therapies in selected clinical cases.

Low-grade oncocytic tumor (LOT) - a new renal entity ready for a prime time: An updated review.

Low-grade oncocytic tumor (LOT) of kidney has been recently proposed as a new renal entity. LOT was identified in the spectrum of oncocytic renal tumors with overlapping features between oncocytoma and eosinophilic chromophobe renal cell carcinoma, or it has been labelled as one of those entities in prior studies and in practice. LOT is often a single, relatively small tumor, found in a non-syndromic setting, but rare examples of multiple LOTs or admixed with other tumors have been found in patients with tuberous sclerosis complex. LOT typically has solid architecture, and it is composed of eosinophilic cells, with round to oval 'low-grade' nuclei, lacking irregularities and showing focal perinuclear halos. Sharp transition into edematous stromal areas, with scattered or loosely arranged cells are frequently found. LOT has a consistent immunohistochemical profile with diffuse reactivity for cytokeratin 7 and absent (or rarely weak) expression for CD117, a profile different from oncocytoma and eosinophilic chromophobe renal cell carcinoma. Similarly, in contrast to those entities, it also lacks or shows only weak expression for FOXI1. Recent studies have shown that LOT has a molecular/genetic profile different from other renal tumors, with frequent alterations affecting the MTOR/TSC pathway genes. LOT demonstrates either disomic pattern or deletions of 19p13, 19q13 and 1p36, and lacks complete chromosomal losses or gains. In all published studies to date, LOT has shown benign behavior. In this review, we summarize the evidence from recently published studies, which strongly supports the conclusion that LOT is a distinct and unique renal entity.