@article{3112755, title = "Granulosa cell tumor of the ovary: Tumor review", author = "Koukourakis, G.V. and Kouloulias, V.E. and Koukourakis, M.J. and Zacharias, G.A. and Papadimitriou, C. and Mystakidou, K. and Pistevou-Gompaki, K. and Kouvaris, J. and Gouliamos, A.", journal = "Integrative Cancer Therapies", year = "2008", volume = "7", number = "3", pages = "204-215", issn = "1534-7354, 1552-695X", doi = "10.1177/1534735408322845", keywords = "alkylating agent; antineoplastic agent; bleomycin; BRCA1 protein; BRCA2 protein; carboplatin; cisplatin; clomifene; cyclophosphamide; dactinomycin; doxorubicin; estradiol; etoposide; fluorouracil; follitropin; gemcitabine; gestagen; gonadotropin derivative; goserelin; inhibin; leuprorelin; medroxyprogesterone; oxaliplatin; paclitaxel; progesterone receptor; tamoxifen; vinblastine; vincristine, abdominal pain; cancer incidence; cancer relapse; cancer staging; cancer surgery; cancer survival; clinical trial; combination chemotherapy; computer assisted tomography; continuous infusion; disease association; drug efficacy; echography; endometrium carcinoma; endometrium hyperplasia; gene mutation; granulosa cell tumor; hemoperitoneum; hormonal therapy; hormone release; human; hysterectomy; oncogene; ovary cancer; ovary hyperstimulation; pneumonia; precocious puberty; priority journal; prognosis; radiation dose; review; risk factor; salpingooophorectomy; sepsis; treatment response; tumor recurrence; tumor volume; vagina bleeding, Antineoplastic Combined Chemotherapy Protocols; Chemotherapy, Adjuvant; Combined Modality Therapy; Disease-Free Survival; Female; Granulosa Cell Tumor; Humans; Neoplasm Staging; Ovarian Neoplasms; Prognosis; Radiotherapy, Adjuvant; Tumor Markers, Biological", abstract = "Granulosa cell tumors of the ovary are rare neoplasms that originate from sex-cord stromal cells. The long natural history of granulosa cell tumors and their tendency to recur years after the initial diagnosis are the most prominent of their characteristics. The secretion of estradiol is the reason for signs at presentation such as vaginal bleeding and precocious puberty. Abdominal pain and hemoperitoneum, which occasionally can occur, are attributable to tumor rupture. The most common finding in pelvic examination is a tumor mass, which is subsequently confirmed with imaging techniques. Surgery is the mainstay of initial management for histological diagnosis, appropriate staging, and debulking. A more conservative unilateral salpingo-oophorectomy is indicated in patients with stage I disease and patients of reproductive age. Total abdominal hysterectomy with bilateral salpingo-oophorectomy is the appropriate surgical treatment for postmenopausal women and those with more advanced disease. The stage of disease is the most important prognostic factor associated with the risk of relapse. There are no clear conclusions regarding the role of postoperative chemotherapy or radiotherapy in stage I disease and in those with completely resected tumor. The use of adjuvant chemotherapy or radiotherapy has sometimes been associated with prolonged disease-free survival and possibly overall survival. Chemotherapy is the treatment of choice for patients with advanced, recurrent, or metastatic disease, and BEP (bleomycin, etoposide, and cisplatin) is the preferred regimen. Although the overall rate of response to treatment is high, the impact of treatment on disease-free or overall survival is unknown. Prolonged surveillance is mandatory because tumors tend to recur years after the initial diagnosis. © 2008 Sage Publications." }