Ollier's disease associated with ovarian juvenile granulosa cell tumor

A six-year-old girl had Ollier's disease (OD) associated with juvenile granulosa cell tumor (JGCT). The clinical manifestations were right hemicorporal deformity due to multiple skeletal enchondromas and precocious pseudopuberty. After the removal of a Stage Iai JGCT, hormonal symptoms disappeared. Neither the ovarian tumor recurred nor the enchondromas underwent sarcomatous change after a follow-up period of 7 years. A review of the literature showed five previous cases, three associated with OD and two with Mafucci's syndrome (MS). In these cases, patients were young and the ovarian tumors were homolateral to the hemicorporal side involved by enchondromatosis. Data provided from these cases emphasize the notion of a generalized mesodermal dysplasia. JGCT behave in association with the OD in its usual fashion of hormonal production and low-degree aggressiveness.