Department of Obstetrics and Gynecology, University Hospital, LMU Munich, Munich, Germany.
Department of Gynecologic Oncology and Reproductive Medicine, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA.
Int J Gynecol Cancer. 2021 Mar;31(3):432-441. doi: 10.1136/ijgc-2020-001775.
Although gynecologic cancers usually affect older women, a significant proportion of patients with rare ovarian tumors are of reproductive age. In a young patient who presents with a pelvic mass, a primary consideration should be the probability of a malignancy. If there is any suspicion of a cancer diagnosis, the patient should be referred to a gynecologic oncologist. Key factors in clinical management include assessment of preoperative studies (physical examination, tumor markers, and imaging) to determine the likelihood of a malignancy, appropriate preoperative counseling (including discussion of fertility preservation), choice of surgical approach (minimally invasive vs open), frozen section examination by a gynecologic pathologist, and intraoperative decision making. Fortunately, the clinical features of several rare ovarian tumors are compatible with fertility preservation. These characteristics include a high proportion of stage I disease and unilateral ovarian involvement for most rare histotypes. Once a final diagnosis of a rare ovarian tumor is determined, further clinical management may include the need for further studies, possible referral to a fertility expert, consideration of further surgery (if the initial surgery was incomplete), and recommendations for postoperative therapy. This article reviews the literature on fertility preservation in the context of the treatment of several rare ovarian tumor subtypes, including malignant germ cell tumors, sex cord-stromal tumors, borderline tumors, low grade serous carcinoma, clear cell carcinoma, mucinous carcinoma, and small cell carcinoma of the hypercalcemic type.
尽管妇科癌症通常影响老年女性，但相当一部分罕见卵巢肿瘤患者处于生育年龄。对于出现盆腔肿块的年轻患者，首要考虑因素应该是恶性肿瘤的可能性。如果怀疑癌症诊断，应将患者转介给妇科肿瘤学家。临床管理的关键因素包括评估术前检查（体格检查、肿瘤标志物和影像学）以确定恶性肿瘤的可能性、适当的术前咨询（包括讨论生育力保存）、手术方式的选择（微创与开放）、妇科病理学家进行的冰冻切片检查以及术中决策。幸运的是，几种罕见卵巢肿瘤的临床特征与生育力保存兼容。这些特征包括大多数罕见组织学类型的Ⅰ期疾病和单侧卵巢受累的高比例。一旦确定了罕见卵巢肿瘤的最终诊断，进一步的临床管理可能包括需要进一步研究、可能转介给生育专家、考虑进一步手术（如果初始手术不完整），以及推荐术后治疗。本文综述了在治疗几种罕见卵巢肿瘤亚型（包括恶性生殖细胞肿瘤、性索-间质肿瘤、交界性肿瘤、低级别浆液性癌、透明细胞癌、黏液性癌和高钙血症型小细胞癌）时生育力保存的文献。
