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卵巢恶性生殖细胞肿瘤:细胞分类及临床和影像特征。

Ovarian malignant germ cell tumors: cellular classification and clinical and imaging features.

机构信息

From the Department of Diagnostic Radiology (A.M.S., M.R.) and Department of Radiology, Primary Children's Medical Center (H.B.), University of Utah, 30 N 1900 E, Room 1A71, Salt Lake City, UT 84132; Department of Radiology, University of Texas MD Anderson Cancer Center, Houston, Tex (K.M.E.); Department of Diagnostic Radiology, VA Pittsburgh Healthcare System, Pittsburgh, Pa (A.M.); Department of Diagnostic Radiology, Oregon Health & Science University, Portland, Ore (B.R.F.); Department of Pathology, University of Utah School of Medicine, Salt Lake City, Utah (E.A.J.); and Department of Radiology, Mayo Clinic, Scottsdale, Ariz (C.O.M.).

出版信息

Radiographics. 2014 May-Jun;34(3):777-801. doi: 10.1148/rg.343130067.

Abstract

Ovarian malignant germ cell tumors (OMGCTs) are heterogeneous tumors that are derived from the primitive germ cells of the embryonic gonad. OMGCTs are rare, accounting for about 2.6% of all ovarian malignancies, and typically manifest in adolescence, usually with abdominal pain, a palpable mass, and elevated serum tumor marker levels, which may serve as an adjunct in the initial diagnosis, monitoring during therapy, and posttreatment surveillance. Dysgerminoma, the most common malignant germ cell tumor, usually manifests as a solid mass. Immature teratomas manifest as a solid mass with scattered foci of fat and calcifications. Yolk sac tumors usually manifest as a mixed solid and cystic mass. Capsular rupture or the bright dot sign, a result of increased vascularity and the formation of small vascular aneurysms, may be present. Embryonal carcinomas and polyembryomas rarely manifest in a pure form and are more commonly part of a mixed germ cell tumor. Some OMGCTs have characteristic features that allow a diagnosis to be confidently made, whereas others have nonspecific features, which make them difficult to diagnose. However, imaging features, the patient's age at presentation, and tumor markers may help establish a reasonable differential diagnosis. Malignant ovarian germ cell tumors spread in the same manner as epithelial ovarian neoplasms but are more likely to involve regional lymph nodes. Preoperative imaging may depict local extension, peritoneal disease, and distant metastases. Suspicious areas may be sampled during surgery. Because OMGCTs are almost always unilateral and are chemosensitive, fertility-sparing surgery is the standard of care.

摘要

卵巢恶性生殖细胞肿瘤(OMGCT)是源自胚胎性腺原始生殖细胞的异质性肿瘤。OMGCT 较为罕见,占所有卵巢恶性肿瘤的 2.6%左右,通常发生在青少年时期,常表现为腹痛、可触及的肿块和血清肿瘤标志物水平升高,这些可能有助于辅助初始诊断、治疗期间监测和治疗后随访。最常见的恶性生殖细胞肿瘤——无性细胞瘤,通常表现为实性肿块。未成熟畸胎瘤表现为实性肿块,伴有散在的脂肪和钙化灶。卵黄囊瘤通常表现为混合性实性和囊性肿块。可能存在包膜破裂或“亮点征”,这是由于血管增多和小血管动脉瘤形成所致。胚胎癌和多胚瘤很少以纯形式出现,更常见于混合性生殖细胞肿瘤的一部分。一些 OMGCT 具有特征性表现,可做出明确诊断,而另一些则表现不具特异性,难以诊断。然而,影像学特征、患者的发病年龄和肿瘤标志物有助于建立合理的鉴别诊断。恶性卵巢生殖细胞肿瘤的扩散方式与上皮性卵巢肿瘤相同,但更有可能累及区域淋巴结。术前影像学可显示局部扩散、腹膜疾病和远处转移。可疑部位可在手术期间取样。由于 OMGCT 几乎总是单侧发生且对化疗敏感,因此保留生育功能的手术是标准治疗方法。

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