Abstract
Low-grade serous carcinoma (LGSC) of the ovary is a rare histological subtype of epithelial ovarian carcinoma. It has distinct clinical behavior and a specific molecular profile. Compared with high-grade serous carcinoma, this tumor presents at a younger age, has an indolent course, and is associated with prolonged survival. LGSC can arise de novo or originate following a serous borderline tumor (SBT). Pathological differentiation between LGSC and other ovarian carcinoma histological subtypes is fundamental. Several factors might influence the overall outcome, such as the age at diagnosis, current smoking, elevated body mass index, mutational status, hormonal receptors’ expression, and Ki-67 proliferation index. Surgery is the main treatment option in LGSC, and efforts must be maximized to achieve a microscopic residual in metastatic disease. Despite being relatively chemo-resistant, adjuvant platinum-based chemotherapy remains the standard of care in LGSC. Hormonal maintenance therapy after adjuvant chemotherapy results in improved outcomes. Treatment options for disease recurrence include secondary cytoreductive surgery, chemotherapy, hormonal therapy, targeted therapy, and clinical trials. Advancements in genomic studies and targeted therapies are expected to change the treatment landscape in LGSC.
Highlights
Ovarian cancer remains the most lethal gynecologic malignancy [1]
Psammocarcinoma is a rare variant of serous neoplasm arising from the ovary or peritoneum that is separate from low-grade serous carcinomas (LGSC)
They showed that LGSCs have a significantly lower median pretreatment CA-125 value, and that overall, there are fewer patients with an elevated level when compared with high-grade serous carcinomas (HGSC)
Summary
Ovarian cancer remains the most lethal gynecologic malignancy [1]. Epithelial ovarian carcinoma (EOC) is the most frequent histological subtype. Crispen et al evaluated 53 patients with progressive or recurrent SBTs of the ovary They observed that 73% of the relapses contained LGSC elements [10]. Patients with LGSC usually have an indolent clinical course They experience multiple recurrences and may eventually die from disease progression [13]. The pathogenesis begins from a serous cystadenoma or adenofibroma in a sequential and indolent manner, which develops into a SBT with either invasive or noninvasive implants, and reaches its full pathologic potential in the form of LGSC. Diagnostics 2022, 12, 458 ovarian epithelial inclusions, which are potentially the origin of serous cystadenoma, SBT, and LGSC [16,17]. A non-PTH tubal pathway may evolve these lesions, whereby the normal tubal epithelium may exfoliate and implant on the peritoneum or the ovary [17]
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