Emerging entities in renal cell neoplasia: thyroid-like follicular renal cell carcinoma and multifocal oncocytoma-like tumours associated with oncocytosis.

The list of accepted entities of renal cell neoplasia has burgeoned since the turn of the century through recognition of rare tumour types and the discovery of genetic mutations driving renal neoplasia syndromes. This growth has not finished and in this report we present examples of each of these types which were not included in the 2016 World Health Organization classification of renal neoplasia, but are candidates for inclusion in the next edition of the classification. Thyroid-like follicular renal cell carcinoma is a rare tumour type with a distinctive microscopic appearance resembling follicles of the thyroid gland. Thirty-nine cases have been described and the findings have been reasonably consistent. Oncocytoma-like tumours associated with oncocytosis arise as a result of somatic mutations in the mitochondrial genome. The differential diagnosis is mainly with the renal lesions of the Birt-Hogg-Dubé syndrome, which is the result of germline mutations in the folliculin gene. Patients with oncocytoma-like tumours associated with oncocytosis are at great risk of developing renal failure as the proliferating lesions replace the renal parenchyma. Oncocytoma-like tumours have never been found to metastasise.