Approximately half of all lesions of the ovary in childhood are neoplastic, yet only a small fraction of neoplastic lesions contain malignant elements. Overall, malignancies of the ovary account for 10% of all ovarian masses and 1% of childhood cancers. Primary ovarian malignancies fall into three broad categories based on the cell type of origin. Germ cell tumors predominate and may be further subdivided based on the differentiation of the malignant cells. Epithelial cell tumors, which are most common in adults, and sex-cord stromal tumors each make up 15% of the total in children. Rare malignant lesions and metastatic disease account for the small remaining group of tumors. Regardless of tumor type, the majority of patients present with localized disease, but proper surgical staging is important to determine the need for adjuvant therapy. The development of platinum-based chemotherapeutic regimens has resulted in significant improvements in long-term survival, even for children who present with advanced stage disease.
"On the basis of histological characteristics and age of onset, these tumors are divided into adult type (95% of cases) and juvenile type (5% of cases). Precocious pseudo-puberty, vaginal bleeding and irregular menstruation are a common presentation of these tumors associated with hormonal changes.1,2 We describe here a rare case of juvenile granulosa cell tumor (JGCT) in a girl with central precocious puberty. "
[Show abstract][Hide abstract] ABSTRACT: Ovarian tumors associated with hormonal changes of the peripheral iso-sexual precocious puberty are of common presentation. We describe here a rare case of juvenile granulosa cell tumor in a female with central precocious puberty (CPP). An 8-year old girl with CPP presented with vaginal bleeding four months after the diagnosis and before starting treatment with gonadotropin-releasing hormone (GnRH)-analogs. Suppression of basal follicle-stimulating hormone (FSH) level, elevation of serum estradiol, progesterone and Cancer Antigen-125 were documented. Abdominal ultrasound examination (US) and magnetic resonance imaging showed a pelvic mass affecting the left ovary. A left salpingo-oophorectomy was performed and the mass was totally resected. Juvenile granulosa cell ovarian tumor was diagnosed. One month post surgery, estradiol and progesterone decreased to values of the first evaluation and FSH increased; Cancer Antigen-125 resulted normal while ultrasound pelvic examination showed absence of pelvic masses. In our patient, the tumor had grown very quickly since hormonal data demonstrated a CPP without any evidence of ovarian mass on US only four months before diagnosis. The overstimulation of the FSH or aberrant activation of FSH receptors may have contributed to the development of the mass.
[Show abstract][Hide abstract] ABSTRACT: Adnexal masses are uncommon in the pediatric and adolescent population. The differential diagnosis includes ovarian lesions, tubal or paratubal lesions, nongynecologic lesions, and lesions related to infection or pregnancy. Presenting symptoms vary and may include acute abdominal pain, mass effect, and less commonly, precocious puberty and vaginal bleeding. Most of these lesions represent benign pathology, but malignant lesions are identified in a small subset of patients, and appropriate surgical and postoperative management is essential. It is important for clinicians caring for these patients to understand the differential diagnosis of an adnexal mass, to facilitate correct management (whether surgical or nonsurgical) and necessary referrals where appropriate. This review explores the etiologies, presenting symptoms, and appropriate management options for pediatric and adolescent patients with adnexal masses. There are few clinical trials guiding the management of these patients, and existing data comprise primarily case series and retrospective studies.
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