Cases reported "Enchondromatosis"

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1/3. Ovarian juvenile granulosa cell tumor associated with Maffucci's syndrome.

    A 15-year-old girl developed a juvenile granulosa cell tumor associated with Maffucci's syndrome (enchondromatosis hemangiomas). Clinical manifestations of the disease included an abdominal mass and progressive anemia. She underwent the removal of a Stage Ic juvenile granulosa cell tumor and subsequent adjuvant chemotherapy. On follow-up examination 4 years later, no recurrence of the ovarian tumor was noted. A review of the literature showed 10 previous cases of juvenile granulosa cell tumor associated with enchondromatosis, two associated with Maffucci's syndrome, and the rest with Ollier's disease (enchondromatosis). Ovarian juvenile granulosa cell tumor may occur not infrequently in female patients with enchondromatosis in the first or second decades, in contrast to the widely recognized sarcomatous changes of enchondromas that usually occur after the second decade. Data provided from these cases also emphasize the concept of a generalized mesodermal dysplasia.
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ranking = 1
keywords = granulosa cell, granulosa
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2/3. 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.
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ranking = 0.53553657151719
keywords = granulosa cell, granulosa
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3/3. Juvenile granulosa cell tumor in a 13-year-old girl with enchondromatosis (Ollier's disease): a case report.

    Juvenile granulosa cell tumor (JGCT) of the ovary has been reported to occur rarely in conjunction with Ollier's disease. We report a case of a 13-year-old girl who was noted to have a large abdominal mass at the time of excision of a symptomatic enchondroma of the right femur. Subsequent laparotomy at a tertiary care children's medical center identified a JGCT confined to the right ovary. review of the literature reveals eight previous cases of JGCT in patients with Ollier's disease. The pathophysiology and possible common causes of both disorders is discussed.
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ranking = 0.625
keywords = granulosa cell, granulosa
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