Department of Medicine, Medway NHS Foundation Trust, Windmill Road, Gillingham ME7 5NY, UK.
Department of Radiology, University Hospitals Plymouth NHS Trust, Plymouth PL6 8DH, UK.
Int J Environ Res Public Health. 2023 Jun 9;20(12):6089. doi: 10.3390/ijerph20126089.
Nonepithelial ovarian cancers (NEOC) are a group of rare malignancies, including germ cell tumours (GCT) and sex cord-stromal tumours (SCST), along with small-cell carcinomas and sarcomas. GCTs represent 2-5% of ovarian cancers, with a yearly incidence of 4:100,000, and they usually affect young women and adolescents. Precursory germ cells of the ovary form the basis of GCT. They are histologically classified into primitive GCT, teratomas, and monodermal and somatic-type tumours associated with dermoid cysts. A primitive GCT can be either a yolk sac tumour (YST), dysgerminoma, or mixed germ cell neoplasm. Teratomas are either mature (benign) or immature (malignant). Given that malignant GCTs occur rarely compared to epithelial ovarian tumours (EOC), greater focus is required in their diagnosis and treatment. In this article, we review the epidemiology, clinical manifestations, diagnosis, and molecular biology, along with the management and therapeutic challenges.
非上皮性卵巢癌(NEOC)是一组罕见的恶性肿瘤,包括生殖细胞肿瘤(GCT)和性索-间质肿瘤(SCST),以及小细胞癌和肉瘤。GCT 占卵巢癌的 2-5%,年发病率为 4:100000,通常影响年轻女性和青少年。卵巢的原始生殖细胞是 GCT 的基础。它们在组织学上分为原始 GCT、畸胎瘤以及与皮样囊肿相关的单胚层和体细胞型肿瘤。原始 GCT 可以是卵黄囊瘤(YST)、无性细胞瘤或混合性生殖细胞肿瘤。畸胎瘤要么是成熟的(良性的),要么是不成熟的(恶性的)。由于恶性 GCT 比上皮性卵巢肿瘤(EOC)少见,因此需要更加关注其诊断和治疗。本文综述了 GCT 的流行病学、临床表现、诊断和分子生物学,以及管理和治疗挑战。