IMPE Abstracts

Introduction: Ovarian neoplasms are occurring in 2.6/100,000 girls per year. Juvenile Granulosa Tumor (JGCT) belongs to the category of sex cord-stromal tumors. JGCTs usually present in premenarcheal girls and young women with a mean age at diagnosis of 20 years of age and a range between 8-45 years. The tumor produces symptoms derived from the secretion of the estradiol. It has a favorable prognosis and is usually limited to one ovary. In girls, it can cause precocious pseudopuberty vaginal bleeding, abdominal pain and abdominal distention. The surgery is the main diagnostic approach. Late recurrences with 80% mortality rate have also been reported. Serum levels of AMH, inhibin B and estradiol are useful to evaluate the efficacy of treatment and early detection of recurrences. Our objective is to present three clinical cases of a rare pathology in girls under 8 years old.

Cases Description: Case 1: A 7.8-year-old girl with precocious puberty, presented with an acute abdomen, interpreting like right ovarian torsion. Tumor was removed and, subsequently, a right oophorectomy was indicated. Laboratory tests showed gonadotrophins and estradiol in the pubertal range. Case 2: A 3.2-year-old girl with precocious thelarche and vaginal bleeding, laboratory tests showed normal gonadotrophins and high estradiol, advanced bone age, and gynecological ultrasound with a pubertal uterus and a mass in the right ovary, verified on CT scan of the abdomen and pelvis. Case 3: A 5-year-old girl who presented little red/brown vaginal bleeding of 5 days of evolution associated with constipation, asthenia, adynamia and weight gain in the last month. Surgical resection (left oophorectomy) was indicated. In the 3 cases, the pathological anatomy study revealed JGCT, in case 1, the tumor could not be staged and in cases 2 and 3, they were staged 1 (Children's Oncology Group). The patients are in regular clinical follow-up, with imaging (gynecological ultrasound) and serum level tests: antimüllerian hormone (AMH), gonadotrophins and estradiol.

Final comments: Granulosa tumors are rare and have a low malignant potential. The clinical presentation, staging, and prognosis are different according to the age and site at diagnosis. The recognition of this pathology is important to optimize the treatment, selecting the appropriate surgical procedure in girls, transcendental for the preservation of fertility.