Schumer Susan Tinsley, Cannistra Stephen A
Program in Gynecologic Medical Oncology, Beth Israel Deaconess Medical Center, Boston, MA 02215, USA.
J Clin Oncol. 2003 Mar 15;21(6):1180-9. doi: 10.1200/JCO.2003.10.019.
Adult granulosa cell tumor (GCT) of the ovary is oftentimes a hormonally active, stromal cell neoplasm that is distinguished by its ability to secrete sex steroids such as estrogen. Patients may present with vaginal bleeding caused by endometrial hyperplasia or uterine cancer as a result of prolonged exposure to tumor-derived estrogen. In addition, GCT is a vascular tumor that may occasionally rupture and result in abdominal pain, hemoperitoneum, and hypotension, mimicking an ectopic pregnancy in younger patients. GCT is usually associated with a mass on pelvic examination that is subsequently confirmed on ultrasonography. Surgery is required for definitive tissue diagnosis, staging, and tumor debulking. In older women, a total abdominal hysterectomy and bilateral salpingooophorectomy are typically performed. In women of childbearing age, a more conservative unilateral salpingo-oophorectomy may be performed, assuming that careful staging reveals that the disease has not extended outside of the involved ovary and that a concomitant uterine cancer has been excluded. Survival of patients with GCT is generally excellent because most patients present with early-stage disease, although certain high-risk patient groups may be identified. Stage is the most important prognostic factor, with a higher risk of relapse being associated with stages II through IV disease. In addition, patients with stage I disease associated with features such as large tumor size, high mitotic index, or tumor rupture may also be at higher risk in some series. The value of postoperative adjuvant therapy for high-risk patients has not been investigated by prospective randomized trials, which are difficult to perform because of the rarity of this tumor. Nonetheless, the use of adjuvant chemotherapy or radiation has sometimes been associated with prolonged disease-free survival in patients with high-risk features. Because of the propensity of GCT to recur years after initial diagnosis, prolonged surveillance with serial physical examination and serum tumor markers such as estradiol and inhibin is reasonable.
卵巢成人颗粒细胞瘤（GCT）通常是一种具有激素活性的间质细胞瘤，其特点是能够分泌雌激素等性类固醇。由于长期暴露于肿瘤分泌的雌激素，患者可能会出现子宫内膜增生或子宫癌导致的阴道出血。此外，GCT是一种血管性肿瘤，偶尔可能破裂，导致腹痛、腹腔内出血和低血压，在年轻患者中类似异位妊娠。盆腔检查时通常可触及肿块，随后超声检查得以证实。明确的组织诊断、分期及肿瘤减瘤需要手术治疗。对于老年女性，通常进行全腹子宫切除术和双侧输卵管卵巢切除术。对于育龄期女性，若仔细分期显示疾病未超出受累卵巢且排除了合并子宫癌，可进行更保守的单侧输卵管卵巢切除术。GCT患者的生存率总体良好，因为大多数患者为早期疾病，不过可识别出某些高危患者群体。分期是最重要的预后因素，II至IV期疾病复发风险较高。此外，在一些系列研究中，I期疾病伴有肿瘤体积大、有丝分裂指数高或肿瘤破裂等特征的患者复发风险也可能较高。由于该肿瘤罕见，前瞻性随机试验尚未对高危患者术后辅助治疗的价值进行研究。尽管如此，辅助化疗或放疗有时与具有高危特征患者的无病生存期延长有关。由于GCT在初次诊断数年后仍有复发倾向，通过定期体格检查和检测血清肿瘤标志物如雌二醇和抑制素进行长期监测是合理的。
