Abstract
Low-grade serous ovarian carcinoma (LGSOC) is a distinct pathologic and clinical entity, characterized by less aggressive biological behavior, lower sensitivity to chemotherapy and longer survival compared with high-grade serous ovarian carcinoma. LGSOC often harbors activating mutations of genes involved in mitogen activated protein kinase (MAPK) pathway. Patients with disease confined to the gonad(s) should undergo bilateral salpingo-oophorectomy, total hysterectomy and comprehensive surgical staging, although fertility-sparing surgery can be considered in selected cases. Women with stage IA-IB disease should undergo observation alone after surgery, whereas observation, chemotherapy or endocrine therapy are all possible options for those with stage IC-IIA disease. Patients with advanced disease should undergo primary debulking surgery with the aim of removing all macroscopically detectable disease, whereas neoadjuvant chemotherapy followed by interval debuking surgery. After surgery, the patients can receive either carboplatin plus paclitaxel followed by endocrine therapy or endocrine therapy alone. Molecularly targeted agents, and especially MEK inhibitors and Cyclin-dependent kinase (CDK) inhibitors, are currently under evaluation. Additional research on the genomics of LGSOC and clinical trials on the combination of MEK inhibitors with hormonal agents, other molecularly targeted agents or metformin, are strongly warranted to improve the prognosis of patients with this malignancy.
Highlights
Low-grade serous ovarian carcinoma (LGSOC) is a distinct pathologic and clinical entity, accounting for 1.02–3.64% of all ovarian carcinomas and 4.66% of serous ovarian carcinomas, and characterized by younger age at presentation, less aggressive biological behavior, lower sensitivity to chemotherapy and longer overall survival (OS) compared with high-grade serous ovarian carcinoma (HGSOC) [1,2,3,4,5,6,7,8]
Surgery is the keystone of treatment, and considering that this malignancy is not very chemo-sensitive, an attempt of primary maximal cytoreduction is strongly warranted [40]
Patients with stage IIb-IV disease can receive either chemotherapy with CBDCA + PTX for six cycles followed by endocrine therapy, most commonly consisting of aromatase inhibitors, or endocrine therapy alone until disease progression or unacceptable toxicity
Summary
Low-grade serous ovarian carcinoma (LGSOC) is a distinct pathologic and clinical entity, accounting for 1.02–3.64% of all ovarian carcinomas and 4.66% of serous ovarian carcinomas, and characterized by younger age at presentation, less aggressive biological behavior, lower sensitivity to chemotherapy and longer overall survival (OS) compared with high-grade serous ovarian carcinoma (HGSOC) [1,2,3,4,5,6,7,8]. LGSOC is characterized by a monotonous proliferation of cuboidal, low columnar cells, mild to moderate atypia without nuclear pleomorphism, mitotic index up to 12 mitoses per 10 high power fields (HPF), and destructive invasion [1,8,9]. This last histological feature can show different architectural patterns; i.e., micropapillary and/or complex papillary; compact cell nests; inverted macropapillae; cribriform; glandular and/or cystic; solid sheets with slit-like spaces and single cells [9]. The lack of MRE11, RAD50 and NBS1 protein detection, suggestive of mismatch repair (MMR) deficiency, has been recently detected in LGSOCs [34]
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