Virilizing Sclerosing-Stromal Tumor of the Ovary in a Young Woman With McCune Albright Syndrome: Clinical, Pathological, and Immunohistochemical Studies

Context: McCune-Albright syndrome (MAS) is characterized by polyostotic fibrous dysplasia, cafe-au-lait skin pigmentations, and gonadotropin-independent sexual precocious puberty, resulting from a somatic postzygotic activating mutation of the GNAS1 gene. Setting: We report a virilizing sclerosing-stromal tumor of the ovary in a young female with MAS. Patient: She presented polyostotic fibrous dysplasia of the left upper and lower limbs and a cafe-au-lait skin spot in the posterior area of the neck. She had a history of precocious puberty, diagnosed at the age of 6 years and treated with cyproterone acetate until the age of 10 years; then she developed central puberty with severe oligomenorrhea. At the age of 23 years, she was hospitalized for a virilization syndrome including hirsutism, acne, deepening of the voice, amenorrhea, and clitoromegaly. Serum levels of T were dramatically increased (1293 ng/dl; normal range, 10–80). The abdominal computed tomography scan revealed a solid mass located on the lef...