摘要
卵巢环状小管性索肿瘤是一种罕见的卵巢性索间质肿瘤,多见于年轻女性,约1/3伴有常染色体显性遗传的Peutz-Jeghers综合征。阴道不规则流血是其最常见的临床表现,血清雌二醇、孕激素等血清学标志物可协助诊断,治疗方式包括手术、放化疗、内分泌治疗及靶向治疗,年轻女性治疗需兼顾生育能力的保护。本共识参考国内外相关研究,阐述卵巢环状小管性索肿瘤的流行病学、病理学特征、生物学行为、转移途径、临床表现、诊断以及不同生育需求的治疗,以期为临床工作者提供临床参考。
Ovarian sex cord tumor with annular tubules is a rare ovarian sex cord-stromal tumor,which is predominantly observed in young women.Approximately one-third of cases are associated with the autosomal dominant Peutz-Jeghers syndrome.Irregular vaginal bleeding is most common clinical manifestation and serum markers such as estradiol and progesterone can aid in diagnosis.Treatment modalities include surgery,radiotherapy,chemotherapy,endocrine therapy,and targeted therapy.Management in young women must include fertility preservation considerations.This consensus,with reference to relevant domestic and foreign research,elaborates on the epidemiology,pathological characteristics,biological behavior,metastatic pathways,clinical manifestations,diagnosis,and treatment options catering to different fertility needs of ovarian sex cord tumor with annular tubules.It aims to provide clinical practitioners with a clinical reference.
出处
《癌症进展》
2025年第17期1985-1993,2012,共10页
Oncology Progress
基金
辽宁省重点研发项目(2024JH2/102500019)
辽宁省“兴辽英才计划”项目(XLYC2412037)
2022年沈阳市科技计划项目(22-321-33-08)
沈阳市科技局2023年助力中国医科大学高质量发展专项(23-506-3-01-10)。
