低级别浆液性卵巢癌以前被认为是高级别浆液性卵巢癌的一种亚型，但现在它被认为是一种具有独特临床和分子行为的独特疾病。该疾病可能从头出现或由浆液性交界性卵巢肿瘤发展而来。尽管它比高级别浆液性卵巢癌更惰性，但大多数患者在诊断时已患有晚期转移性疾病，并且复发很常见。复发性低级别浆液性卵巢癌通常对标准铂类紫杉烷化疗具有耐药性，因此很难用现有的治疗方案进行治疗。需要新的靶向疗法，但它们的开发取决于对该疾病的特定生物学的更深入了解。低级别肿瘤的已知分子驱动因素是强激素受体表达、丝裂原激活蛋白激酶 (MAPK) 通路（KRAS、BRAF 和 NRAS）以及与 MAPK 通路相关的基因（NF1/2、EIF1AX）的突变。和 ERBB2)。然而，MAPK 抑制剂仅表现出适度的临床反应。基于低级别浆液性卵巢癌中 CDKN2A 突变的发现，细胞周期蛋白依赖性激酶 4 和 6 (CDK4/6) 抑制剂目前正在临床试验中与激素治疗相结合进行测试。在较小群体的低级别肿瘤中发现的其他突变包括 USP9X、ARID1A 和 PIK3CA，但尚未针对这些突变进行临床测试。本综述总结了目前已知的低级别浆液性卵巢癌的临床、病理和分子特征，并介绍了潜在的治疗靶点和新的研究途径。© IGCS 和 ESGO 2024。在 CC BY-NC 下允许重复使用。禁止商业再利用。由英国医学杂志出版。

Low-grade serous ovarian cancer was previously thought to be a subtype of high-grade serous ovarian cancer, but it is now recognized as a distinct disease with unique clinical and molecular behaviors. The disease may arise de novo or develop from a serous borderline ovarian tumor. Although it is more indolent than high-grade serous ovarian cancer, most patients have advanced metastatic disease at diagnosis and recurrence is common. Recurrent low-grade serous ovarian cancer is often resistant to standard platinum-taxane chemotherapy, making it difficult to treat with the options currently available. New targeted therapies are needed, but their development is contingent on a deeper understanding of the specific biology of the disease. The known molecular drivers of low-grade tumors are strong hormone receptor expression, mutations in the mitogen-activated protein kinase (MAPK) pathway (KRAS, BRAF, and NRAS), and in genes related to the MAPK pathway (NF1/2, EIF1AX, and ERBB2). However, MAPK inhibitors have shown only modest clinical responses. Based on the discovery of CDKN2A mutations in low-grade serous ovarian cancer, cyclin-dependent kinases 4 and 6 (CDK4/6) inhibitors are now being tested in clinical trials in combination with hormone therapy. Additional mutations seen in a smaller population of low-grade tumors include USP9X, ARID1A, and PIK3CA, but no specific therapies targeting them have been tested clinically. This review summarizes the clinical, pathologic, and molecular features of low-grade serous ovarian cancer as they are now understood and introduces potential therapeutic targets and new avenues for research.© IGCS and ESGO 2024. Re-use permitted under CC BY-NC. No commercial re-use. Published by BMJ.