Clear cell renal cell carcinoma (ccRCC) with hemangioblastoma-like features is a recently described new entity, with only 4 cases reported in the English literature to date. It is characterized by the presence of a hemangioblastoma-like component which shares the same morphologic and phenotypic features with renal hemangioblastoma associated to an unequivocal ccRCC component. Transition between both components is gradual. The diagnosis is challenging, especially when ccRCC component is scattered, the tumor might be diagnosed as a hemangioblastoma. In fact, distinguishing the latter from ccRCC with hemangioblastoma-like features is essential for providing patients with the best available treatments. In this article, we describe a new case of ccRCC with hemangioblastoma in a 30-year-old woman to raise awareness of this rare entity presenting a challenging diagnosis.
Derqaoui et al. (Thu,) studied this question.