Emerging entities of renal cell neoplasia

Abstract

The current classification of renal cell carcinoma (RCC) was formulated at the meeting of the World Health Organization Renal Tumor Panel in 2015, with the results published in the fourth edition of the World Health Organization Classification of Tumours of the Urinary System and Male Genital Organs Bluebook in 2016. At that meeting a number of tumor types were designated as emerging or provisional entities as it was felt that they were insufficiently characterized to merit inclusion as a recognized type of RCC. One tumor type included in this designation was thyroid-like follicular RCC. Since the publication of the 2016 classification this tumor type has been further characterized and in addition to this, detailed studies on three other types of RCC (multifocal oncocytoma-like tumors associated with oncocytosis, eosinophilic solid and cystic RCC and biphasic squamoid alveolar RCC) have been published. It is now apparent that these four tumors are unique morphotypes and genotypes of RCC, and are likely to be included in the next edition of the World Health Organization classification of renal tumors. Multifocal oncocytoma-like tumors associated with oncocytosis is a benign process characterized by the presence of hundreds to thousands of oncocytic tumors in a single kidney. These tumors occur sporadically and are unrelated to the tumors of Birt-Hogg-Dubé syndrome. Eosinophilic solid and cystic RCC is characterized by a solid and cystic architecture with tumor cells consisting of bulky eosinophilic and granular cytoplasm with intracytoplasmic vacuolation. Thyroid-like follicular RCC occurs in younger patients with a female predominance. The tumor bears a striking resemblance to follicular carcinoma of the thyroid with follicles containing intraluminal proteineacous material resembling thyroglobulin. Immunostains for thyroid markers are negative. Finally, biphasic squamoid alveolar RCC consists of aggregates of large cells with pale eosinophilic cytoplasm usually arranged in a glomeruloid/alveolar pattern and surrounded by a border of basophilic cells with scanty cytoplasm. The genotype of the tumor, as well its recorded association with typical papillary RCC, has led to the suggestion that it is related to type 1 papillary RCC.