Low-grade serous ovarian carcinoma (LGSOC) is a rare malignancy in pediatric populations, with most ovarian tumors in adolescents typically being of germ cell origin. LGSOC is a distinct subtype of serous ovarian carcinoma characterized by slow progression, frequent estrogen receptor (ER) positivity, and resistance to traditional chemotherapy. Despite its indolent nature, most patients ultimately experience disease recurrence, highlighting the need for alternative treatment approaches. This report presents the case of a 13-year-old female diagnosed with advanced-stage LGSOC following menarche. She initially presented with unintentional weight loss, constipation, and early satiety, and imaging revealed extensive pelvic disease. Given the tumor's limited sensitivity to chemotherapy, she was treated with neoadjuvant endocrine therapy (ET) using fulvestrant, palbociclib, and leuprolide. This regimen led to substantial tumor regression and normalization of CA-125 levels. Following 15 cycles, she underwent interval debulking surgery, achieving optimal cytoreduction while preserving the uterus. She continues adjuvant therapy with no signs of disease progression. Given the long-term effects of hormonal suppression in adolescents, careful monitoring is essential. Recent studies suggest that targeted therapies, including MEK and CDK4/6 inhibitors, may improve outcomes in LGSOC. Research has shown promising response rates and reduced toxicity compared to traditional chemotherapy. This case supports the potential role of endocrine-based targeted therapy in managing pediatric LGSOC, offering a viable alternative for patients with limited treatment options. Further research is needed to optimize treatment strategies and improve survival outcomes in this rare population.
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http://dx.doi.org/10.1016/j.gore.2025.101697 | DOI Listing |
Int J Gynecol Cancer
January 2025
Kaohsiung Chang Gung Memorial Hospital, Kaohsiung, Taiwan; Chang Gung University College of Medicine, Taoyuan City, Taiwan.
Objective: The HALO study (NCT04991051) determined the prevalence of homologous recombination deficiency and its associated factors in patients with high-grade serous/endometrioid ovarian, primary peritoneal, and/or fallopian tube cancers across Asia, the Middle East, and Russia.
Methods: This multinational, cross-sectional, real-world study enrolled adult women with newly diagnosed stage III or IV high-grade serous/endometrioid ovarian, primary peritoneal, and/or fallopian tube cancers. Formalin-fixed paraffin-embedded tumor blocks were collected within 120 days of enrollment.
Cancer Res
March 2025
Huntsman Cancer Institute, Salt Lake City, Utah, United States.
Black individuals experience worse survival after a diagnosis of high-grade serous ovarian carcinoma (HGSC) than White individuals and are underrepresented in ovarian cancer research. To date, the understanding of the molecular and genomic heterogeneity of HGSC is based primarily on the evaluation of tumors from White individuals. In the present study, we performed whole exome sequencing on HGSC samples from 211 Black patients to identify significantly mutated genes and characterize mutational signatures, assessing their distributions by gene expression subtypes.
View Article and Find Full Text PDFBrief Bioinform
March 2025
Department of Genetics and Genomic Sciences, Icahn School of Medicine at Mount Sinai, 1399 Park Ave, New York, NY 10029, United States.
To unravel the mechanism of immune activation and suppression within tumors, a critical step is to identify transcriptional signals governing cell-cell communication between tumor and immune/stromal cells in the tumor microenvironment. Central to this communication are interactions between secreted ligands and cell-surface receptors, creating a highly connected signaling network among cells. Recent advancements in in situ-omics profiling, particularly spatial transcriptomic (ST) technology, provide unique opportunities to directly characterize ligand-receptor signaling networks that power cell-cell communication.
View Article and Find Full Text PDFGynecol Oncol Rep
April 2025
Department of Gynecologic Oncology, Willamette Valley Cancer Institute and Research Center 520 Country Club, Eugene, OR 97401, United States.
Low-grade serous ovarian carcinoma (LGSOC) is a rare malignancy in pediatric populations, with most ovarian tumors in adolescents typically being of germ cell origin. LGSOC is a distinct subtype of serous ovarian carcinoma characterized by slow progression, frequent estrogen receptor (ER) positivity, and resistance to traditional chemotherapy. Despite its indolent nature, most patients ultimately experience disease recurrence, highlighting the need for alternative treatment approaches.
View Article and Find Full Text PDFOngoing mutagenesis in cancer drives genetic diversity throughout the natural history of cancers. As the activities of mutational processes are dynamic throughout evolution, distinguishing the mutational signatures of 'active' and 'historical' processes has important implications for studying how tumors evolve. This can aid in understanding mutagenic states at the time of presentation, and in associating active mutational process with therapeutic resistance.
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