Steroid cell tumors of the ovary are rare, functioning sex cord-stromal ovarian tumors.1 Previously, they were called lipid cell tumors because of their high lipid content.1,2 Two cases demonstrating occurrence of this rare ovarian tumor within the context of von Hippel-Lindau (VHL) disease have been reported.2,3 VHL disease is an autosomal dominant familial inherited syndrome, with varied manifestations depending on the specific mutation. Pancreatic neuroendocrine tumors in association with pheochromo-cytoma are a classic phenotype of VHL disease,3 but association with steroid tumor of the ovary is a very rare occurrence.
展开▼
