{"product_id":"low-grade-serous-ovarian-cancer-what-experts-agree-on-in-2023","title":"Low-Grade Serous Ovarian Cancer: What Experts Agree On in 2023","description":"\u003cp\u003eLow-grade serous carcinoma (LGSC) is a rare type of ovarian cancer that behaves very differently from the more common high-grade serous carcinoma. It affects younger women, responds poorly to chemotherapy, and researchers are still working out the best ways to diagnose and treat it. An international panel of experts met in October 2022 to review the latest science and create a consensus document to guide doctors caring for women with this disease.\u003c\/p\u003e\n\n\u003ch1\u003eLow-Grade Serous Ovarian Cancer: What Experts Agree On in 2023\u003c\/h1\u003e\n\n\u003ch2\u003eTable of Contents\u003c\/h2\u003e\n\u003cul\u003e\n\u003cli\u003e\u003ca href=\"#ddn-key-points\"\u003eKey Points\u003c\/a\u003e\u003c\/li\u003e\n\n  \u003cli\u003e\u003ca href=\"#background\"\u003eBackground: Why This Disease Needs Its Own Playbook\u003c\/a\u003e\u003c\/li\u003e\n  \u003cli\u003e\u003ca href=\"#methods\"\u003eStudy Methods: How the Expert Panel Worked\u003c\/a\u003e\u003c\/li\u003e\n  \u003cli\u003e\u003ca href=\"#pathology\"\u003ePathology: Defining the Disease and Its Borderline Forms\u003c\/a\u003e\u003c\/li\u003e\n  \u003cli\u003e\u003ca href=\"#implants\"\u003eWhat Happens With Serous Borderline Tumors and Implants\u003c\/a\u003e\u003c\/li\u003e\n  \u003cli\u003e\u003ca href=\"#mixed\"\u003eRare Cases: Low-Grade and High-Grade Cancer Together\u003c\/a\u003e\u003c\/li\u003e\n  \u003cli\u003e\u003ca href=\"#translational\"\u003eTranslational Research: What Happens Inside the Tumor\u003c\/a\u003e\u003c\/li\u003e\n  \u003cli\u003e\u003ca href=\"#mek\"\u003eMEK Inhibitors: Which Patients Respond Best\u003c\/a\u003e\u003c\/li\u003e\n  \u003cli\u003e\u003ca href=\"#endocrine\"\u003eEndocrine Therapy: Hormone Treatment and Its Biomarkers\u003c\/a\u003e\u003c\/li\u003e\n  \u003cli\u003e\u003ca href=\"#epidemiology\"\u003eEpidemiology: How Common Is This Disease\u003c\/a\u003e\u003c\/li\u003e\n  \u003cli\u003e\u003ca href=\"#risk\"\u003eRisk Factors and the BRCA Question\u003c\/a\u003e\u003c\/li\u003e\n  \u003cli\u003e\u003ca href=\"#germline\"\u003eGenetic Testing: What Every Patient Should Know\u003c\/a\u003e\u003c\/li\u003e\n  \u003cli\u003e\u003ca href=\"#somatic\"\u003eTumor Testing: Looking Inside the Cancer Itself\u003c\/a\u003e\u003c\/li\u003e\n  \u003cli\u003e\u003ca href=\"#prognosis\"\u003ePrognostic Factors: What Predicts Outcomes\u003c\/a\u003e\u003c\/li\u003e\n  \u003cli\u003e\u003ca href=\"#imaging\"\u003eImaging: How the Disease Is Tracked\u003c\/a\u003e\u003c\/li\u003e\n  \u003cli\u003e\u003ca href=\"#limitations\"\u003eLimitations: What the Consensus Could Not Answer\u003c\/a\u003e\u003c\/li\u003e\n  \u003cli\u003e\u003ca href=\"#recommendations\"\u003eRecommendations for Patients\u003c\/a\u003e\u003c\/li\u003e\n  \u003cli\u003e\u003ca href=\"#ddn-faq\"\u003eFrequently Asked Questions\u003c\/a\u003e\u003c\/li\u003e\n\u003cli\u003e\u003ca href=\"#source\"\u003eSource Information\u003c\/a\u003e\u003c\/li\u003e\n\u003c\/ul\u003e\n\n\u003c!-- ddn:keypoints:start --\u003e\n\u003ch2 id=\"ddn-key-points\"\u003eKey Points\u003c\/h2\u003e\n\u003cul\u003e\n\u003cli\u003eLow-grade serous carcinoma is a rare ovarian cancer distinct from high-grade serous carcinoma.\u003c\/li\u003e\n\u003cli\u003eExpert pathologist review is important because borderline tumors and low-grade serous carcinoma can be confused.\u003c\/li\u003e\n\u003cli\u003eSerous borderline tumors with non-invasive implants: chemotherapy is not recommended; extended follow-up is needed.\u003c\/li\u003e\n\u003cli\u003eAll patients with ovarian cancer should undergo germline genetic testing, though LGSC is rarely BRCA-associated.\u003c\/li\u003e\n\u003cli\u003eResidual disease after surgery and age 35 or younger predict worse outcomes in LGSC.\u003c\/li\u003e\n\u003c\/ul\u003e\n\u003c!-- ddn:keypoints:end --\u003e\n\n\n\u003ch2 id=\"background\"\u003eBackground: Why This Disease Needs Its Own Playbook\u003c\/h2\u003e\n\n\u003cp\u003eLow-grade serous carcinoma (LGSC) of the ovary or peritoneum (the tissue lining the abdominal cavity) is a relatively rare type of epithelial ovarian cancer. Epithelial ovarian cancer is the most common form of ovarian cancer, starting in the cells on the surface of the ovary. LGSC represents \u003cstrong\u003eless than 10% of epithelial ovarian cancers\u003c\/strong\u003e.\u003c\/p\u003e\n\n\u003cp\u003eCompared with high-grade serous carcinoma (HGSC), LGSC carries a longer survival outlook. But the trade-off is harsh in another way. The disease is often diagnosed in younger women. Many of them endure years of ineffective treatments and suffer poor quality of life.\u003c\/p\u003e\n\n\u003cp\u003eLGSC is molecularly distinct from other ovarian cancers. That means the genetic machinery driving the disease is fundamentally different. Because of this, a unique approach to clinical management is required to maximize survival. Standard ovarian cancer approaches built for HGSC simply do not translate well.\u003c\/p\u003e\n\n\u003cp\u003eThe pathogenesis (the biological process that creates the disease) and its management remain incompletely understood. However, recent advances in molecular characterization have identified new targeted therapies with real activity in LGSC. These offer the promise of improved outcomes.\u003c\/p\u003e\n\n\u003cp\u003eIn 2019, a panel of experts convened at a state-of-the-science conference to address the unique needs of LGSC. That meeting produced a published consensus paper. Since then, research has progressed. In October 2022, experts gathered again, this time in New York, USA, for a one-day workshop. This article explains what they concluded and what it means for patients.\u003c\/p\u003e\n\n\u003ch2 id=\"methods\"\u003eStudy Methods: How the Expert Panel Worked\u003c\/h2\u003e\n\n\u003cp\u003eIn October 2022, investigators with expertise in the basic, translational, and clinical science of LGSC gathered in New York for a one-day workshop. Basic science looks at laboratory mechanisms. Translational research bridges lab discoveries and patient care. Clinical science studies treatments in people.\u003c\/p\u003e\n\n\u003cp\u003eA steering committee developed a series of questions before the workshop. The categories included pathology (the study of how the disease looks under a microscope), translational research, epidemiology (how the disease spreads through populations) and risk, clinical management, clinical trials, and future research directions.\u003c\/p\u003e\n\n\u003cp\u003eAt the workshop, a qualified investigator presented the current evidence relating to each question. The entire group then discussed the findings. Experts developed consensus statements based on each question and the ensuing discussion. The panel also assembled a patient perspective from a social media-based survey, ensuring patient voices were part of the conversation.\u003c\/p\u003e\n\n\u003ch2 id=\"pathology\"\u003ePathology: Defining the Disease and Its Borderline Forms\u003c\/h2\u003e\n\n\u003cp\u003eIn 2020, the World Health Organization (WHO) defined LGSC as \u003cstrong\u003e\"an invasive serous neoplasm with low-grade malignant features.\"\u003c\/strong\u003e An invasive neoplasm is a tumor that has broken through the tissue where it started. Serous refers to the type of cell involved, one that produces a watery, serum-like fluid. Low-grade malignant features mean the cells look less aggressive under a microscope than high-grade cancer cells.\u003c\/p\u003e\n\n\u003cp\u003eThe WHO also defined ovarian serous borderline tumor (SBT) as \u003cstrong\u003e\"a non-invasive, low-grade, proliferative serous epithelial neoplasm.\"\u003c\/strong\u003e Borderline tumors sit between benign (non-cancerous) growths and fully invasive cancers. They have a low potential for malignancy but are not entirely harmless.\u003c\/p\u003e\n\n\u003cp\u003eThe WHO added an important clarification about implants. Implants are deposits of cells that spread to the peritoneum, the tissue lining the abdomen. The WHO stated that \"Implants of serous borderline are, by definition, non-invasive; if there is invasion, a diagnosis of low-grade serous carcinoma should be made.\" In other words, if the implant is actively invading tissue, the disease has crossed the line into cancer.\u003c\/p\u003e\n\n\u003cp\u003eThe old term \u003cstrong\u003e\"non-invasive low-grade serous carcinoma\"\u003c\/strong\u003e is no longer recommended. Previously, this phrase was used synonymously with micropapillary serous borderline tumor. Micropapillary refers to a specific finger-like microscopic pattern. A micropapillary serous borderline tumor is not to be treated as cancer, provided the tumor is well sampled to rule out any apparent invasion.\u003c\/p\u003e\n\n\u003cp\u003eThe diagnosis of \u003cstrong\u003e\"microinvasive low-grade serous carcinoma\"\u003c\/strong\u003e applies when there is a focus of ovarian stromal invasion measuring \u003cstrong\u003eless than 5 mm\u003c\/strong\u003e. The stroma is the supportive connective tissue of the ovary. This diagnosis should only be made after careful pathologic examination, preferably with additional sampling of the specimen, to exclude overtly (clearly) invasive LGSC.\u003c\/p\u003e\n\n\u003cp\u003eHere is some reassuring news: in most studies, ovarian SBT with microinvasive LGSC (or microinvasion) is \u003cstrong\u003enot associated with an increased risk of recurrence\u003c\/strong\u003e. Therefore, it should not be considered equivalent to overtly invasive LGSC, provided that extra-ovarian invasive implants are not present. Extra-ovarian means outside the ovary itself.\u003c\/p\u003e\n\n\u003ch3\u003eConsensus on Pathologic Definitions\u003c\/h3\u003e\n\n\u003cp\u003eThe expert panel accepted the WHO 2020 definition of LGSC (\"an invasive serous neoplasm with low-grade malignant features\"). They agreed the term \"non-invasive low-grade serous carcinoma\" should not be used. Ovarian SBT with microinvasive LGSC or microinvasion does not carry a concerning prognosis. It should be managed as a borderline tumor, not as full cancer.\u003c\/p\u003e\n\n\u003ch2 id=\"implants\"\u003eWhat Happens With Serous Borderline Tumors and Implants\u003c\/h2\u003e\n\n\u003cp\u003eOne major unresolved question is whether ovarian SBT with invasive peritoneal implants and advanced-stage ovarian LGSC behave the same way. Peritoneal implants are deposits of tumor cells on the peritoneum. \"Invasive\" means the deposits are pushing into the tissue beneath them.\u003c\/p\u003e\n\n\u003cp\u003eMost stage III\/IV ovarian LGSC tumors recur after primary therapy. Stage III means the cancer has spread within the abdomen or to lymph nodes. Stage IV means it has spread to distant organs. In contrast, the risk of malignant recurrence (recurrence as full cancer) is \u003cstrong\u003eat least 30%\u003c\/strong\u003e for advanced-stage ovarian SBT with invasive implants.\u003c\/p\u003e\n\n\u003cp\u003eThe actual recurrence risk varies across studies. This variability should be interpreted with caution, because diagnostic criteria for classifying implants have become more standardized over time. Older studies may have used different criteria than newer ones.\u003c\/p\u003e\n\n\u003cp\u003eA major terminology change occurred in 2014. The WHO renamed invasive peritoneal implants as \"metastatic low-grade serous carcinoma.\" This change generated controversy. The European Society of Gynecological Oncology (ESGO) retained the former term. According to ESGO, SBT with invasive implants should be considered separate from advanced LGSC. ESGO also recommended against adjuvant systemic therapy (chemotherapy given after surgery to prevent recurrence) for the primary treatment of SBT with extra-ovarian invasive or non-invasive implants.\u003c\/p\u003e\n\n\u003cp\u003eThe current expert panel agreed that the extent of invasive disease as a prognostic factor has not been well studied. This should be a focus of future research. For example, focal microscopic invasive implants associated with ovarian SBT likely do not behave the same as ovarian LGSC with widespread peritoneal carcinomatosis. Carcinomatosis means extensive spread across the peritoneal surfaces. Even though both conditions are grouped in the same diagnostic category, their behavior may be very different. It may be premature to consider invasive implants equivalent to metastatic LGSC. Further studies are needed to characterize which invasive implants are more likely to recur as LGSC.\u003c\/p\u003e\n\n\u003ch3\u003ePathologist Interpretation Can Vary\u003c\/h3\u003e\n\n\u003cp\u003eAnother layer of confusion is variable interpretation by pathologists. The morphologic criteria (what the tissue looks like under a microscope) for distinguishing invasive from non-invasive implants are well defined. However, some cases can be subjective. The panel recommends consultation with a pathologist experienced in evaluating these lesions for accurate diagnostic classification.\u003c\/p\u003e\n\n\u003cp\u003eThe medical literature has used various terms for low-grade serous neoplasms over the years. This has caused confusion among clinicians about appropriate treatment. Here is a summary of the terminology and treatment recommendations the panel endorsed:\u003c\/p\u003e\n\n\u003cul\u003e\n  \u003cli\u003e\n\u003cstrong\u003eSerous borderline tumor\u003c\/strong\u003e (formerly called atypical proliferative serous tumor or serous tumor of low malignant potential): chemotherapy is \u003cstrong\u003enot\u003c\/strong\u003e appropriate.\u003c\/li\u003e\n  \u003cli\u003e\n\u003cstrong\u003eMicropapillary serous borderline tumor\u003c\/strong\u003e (formerly called non-invasive low-grade serous carcinoma): chemotherapy is \u003cstrong\u003enot\u003c\/strong\u003e appropriate.\u003c\/li\u003e\n  \u003cli\u003e\n\u003cstrong\u003eSerous borderline tumor with microinvasive LGSC\u003c\/strong\u003e: chemotherapy is \u003cstrong\u003enot\u003c\/strong\u003e appropriate.\u003c\/li\u003e\n  \u003cli\u003e\n\u003cstrong\u003eSerous borderline tumor with non-invasive implant(s)\u003c\/strong\u003e: chemotherapy is \u003cstrong\u003enot\u003c\/strong\u003e appropriate.\u003c\/li\u003e\n  \u003cli\u003e\n\u003cstrong\u003eSerous borderline tumor with invasive implant(s) \/ metastatic LGSC\u003c\/strong\u003e: chemotherapy \u003cstrong\u003emay be considered\u003c\/strong\u003e.\u003c\/li\u003e\n  \u003cli\u003e\n\u003cstrong\u003eOvarian LGSC\u003c\/strong\u003e: chemotherapy \u003cstrong\u003emay be considered\u003c\/strong\u003e.\u003c\/li\u003e\n\u003c\/ul\u003e\n\n\u003ch3\u003eRecurrence Risk With Non-Invasive Implants\u003c\/h3\u003e\n\n\u003cp\u003eFor SBT with non-invasive implants, studies consistently show an increased risk of LGSC recurrence, though the exact number varies. That variation depends on the study. Researchers observed the highest risk of recurrence at a tertiary referral center in a study by Silva and colleagues: \u003cstrong\u003eoverall recurrence 44%, with malignant recurrence 34%\u003c\/strong\u003e. The median progression-free survival (the time until the disease worsens) was \u003cstrong\u003e7.1 years\u003c\/strong\u003e from initial diagnosis. Notably, \u003cstrong\u003e77% of recurrences occurred after 5 years\u003c\/strong\u003e.\u003c\/p\u003e\n\n\u003cp\u003eA population-based study by Vang and colleagues used two Danish cancer registries. It showed that the risk of LGSC recurrence with non-invasive implants is increased (\u003cstrong\u003e16%\u003c\/strong\u003e) but not as high as with invasive implants (\u003cstrong\u003e32%\u003c\/strong\u003e). Differences in study populations, length of follow-up, and how each study defined a non-invasive implant likely explain the variability in risk estimates.\u003c\/p\u003e\n\n\u003cp\u003eThe key takeaway for patients: regardless of the exact percentage, these patients require \u003cstrong\u003eextended clinical follow-up\u003c\/strong\u003e. Malignant recurrences can occur more than a decade after the initial diagnosis. The good news is that survival for patients with SBT and non-invasive implants has been reported as \u003cstrong\u003egreater than 90% at 10 years\u003c\/strong\u003e.\u003c\/p\u003e\n\n\u003cp\u003eAdjuvant chemotherapy is not recommended for these patients. Studies have reported a greater number of deaths from treatment complications than from the disease itself.\u003c\/p\u003e\n\n\u003ch3\u003eConsensus on Implants\u003c\/h3\u003e\n\n\u003cp\u003eThe panel concluded that non-invasive implants appear to confer at least a 15-20% increased risk of subsequent LGSC. Patients with no residual disease after surgery do not require adjuvant therapy. However, they do require extended clinical follow-up, because recurrences may happen 5 years or more after diagnosis. That long window matters. A patient who is disease-free after 3 years is not out of the woods yet.\u003c\/p\u003e\n\n\u003ch2 id=\"mixed\"\u003eRare Cases: Low-Grade and High-Grade Cancer Together\u003c\/h2\u003e\n\n\u003cp\u003eLGSC and HGSC are distinct pathologic entities with different spectra of underlying molecular genetic alterations. However, rare cases of SBT or LGSC coexisting with, or recurring as, HGSC or poorly differentiated carcinoma have been reported. Poorly differentiated means the cancer cells look very unlike normal cells and their tissue of origin is hard to identify.\u003c\/p\u003e\n\n\u003cp\u003eMost of these reported cases were associated with a poor prognosis. However, the numbers are small. The data are insufficient for drawing conclusions about the clinical behavior of this rare group of patients and how they should be treated.\u003c\/p\u003e\n\n\u003cp\u003eThe panel recommended that such cases should be \u003cstrong\u003eexcluded from LGSC clinical trials\u003c\/strong\u003e. These mixed tumors are not representative of the biology of most LGSC tumors, and including them could skew trial results.\u003c\/p\u003e\n\n\u003ch3\u003eConsensus on Mixed Tumors\u003c\/h3\u003e\n\n\u003cp\u003eLGSC coexisting with, or associated with, subsequent HGSC is rare. If HGSC is a substantial component of the cancer, the cancer should be managed according to HGSC treatment protocols. Further studies are needed to understand the biology and clinical behavior of these mixed tumors.\u003c\/p\u003e\n\n\u003ch2 id=\"translational\"\u003eTranslational Research: What Happens Inside the Tumor\u003c\/h2\u003e\n\n\u003cp\u003eCurrent evidence indicates that LGSC arises either de novo (on its own, without a precursor) or after a diagnosis of SBT. The mechanisms of LGSC tumorigenesis (how the tumor develops) are not well defined, particularly for tumors that do not involve the mitogen-activated protein kinase (MAPK) pathway. The MAPK pathway is a chain of proteins inside cells that controls growth and division. In LGSC, this pathway is frequently stuck in the \"on\" position, driving uncontrolled growth.\u003c\/p\u003e\n\n\u003cp\u003eMAPK pathway alterations are prominent in \u003cstrong\u003e50% of tumors\u003c\/strong\u003e. An alteration is a change in the genetic code of a gene. Besides the well-known KRAS and BRAF genes, other genetic alterations under investigation include:\u003c\/p\u003e\n\n\u003cul\u003e\n  \u003cli\u003eCDKN2A\/2B deletion (a loss of tumor suppressor genes that normally restrain cell growth)\u003c\/li\u003e\n  \u003cli\u003eNRAS alterations\u003c\/li\u003e\n  \u003cli\u003eERBB2 alterations\u003c\/li\u003e\n  \u003cli\u003ePIK3CA alterations (phosphatidylinositol-4,5-bisphosphate 3-kinase catalytic subunit alpha)\u003c\/li\u003e\n  \u003cli\u003eChromosome 1p36 deletion\u003c\/li\u003e\n  \u003cli\u003eNF1 (neurofibromin 1) alterations\u003c\/li\u003e\n  \u003cli\u003eERBB3 alterations (erbb2 receptor tyrosine kinase 3)\u003c\/li\u003e\n\u003c\/ul\u003e\n\n\u003cp\u003eIt is currently unknown which of these genes are truly driving the disease. That distinction matters, because a driver gene is a target for therapy. Passenger alterations are just along for the ride.\u003c\/p\u003e\n\n\u003cp\u003eThere is also an open question about where LGSC originates. Whether LGSC arises from fallopian tube epithelial progenitor cells (early cells that later specialize) remains controversial. Typically, HGSC is thought to start in the fallopian tubes. For LGSC, the picture is less clear. The presence of AGR3-positive ciliated cells in LGSC, and the observation that \u003cstrong\u003e60% of LGSC tumors are associated with ciliated serous borderline tumors\u003c\/strong\u003e, might suggest an alternative cell origin. Ciliated cells have tiny hair-like projections called cilia. These findings point away from the non-ciliated secretory fallopian tube epithelial cells thought to give rise to HGSC.\u003c\/p\u003e\n\n\u003cp\u003eOne practical implication: no evidence currently supports opportunistic salpingectomy (removing the fallopian tubes during another surgery, such as a hysterectomy) specifically for the prevention of LGSC. For HGSC, this procedure has gained traction as a prevention strategy. For LGSC, the science has not caught up.\u003c\/p\u003e\n\n\u003ch2 id=\"mek\"\u003eMEK Inhibitors: Which Patients Respond Best\u003c\/h2\u003e\n\n\u003cp\u003eMEK inhibitors are targeted drugs that block a specific protein in the MAPK pathway. In patients with LGSC, MAPK alteration status may be associated with a higher response to mitogen-activated extracellular signal-regulated kinase (MEK) inhibitors. However, doctors also observe activity in patients without MAPK alterations. That nuance makes treatment decisions less straightforward.\u003c\/p\u003e\n\n\u003cp\u003eMonk and colleagues studied binimetinib, a MEK inhibitor. They observed that, compared with KRAS wild-type (WT) tumors (tumors with normal, unchanged KRAS genes), KRAS alteration was statistically significantly associated with:\u003c\/p\u003e\n\n\u003cul\u003e\n  \u003cli\u003eA greater objective response rate to binimetinib (odds ratio [OR] 3.4; 95% confidence interval [CI] 1.53 to 7.66; unadjusted p=0.003)\u003c\/li\u003e\n  \u003cli\u003eProlonged progression-free survival (median 17.7 months for KRAS mutant tumors vs 10.8 months for KRAS WT tumors; p=0.006)\u003c\/li\u003e\n\u003c\/ul\u003e\n\n\u003cp\u003eAn odds ratio of 3.4 means patients with a KRAS alteration had about 3.4 times the odds of responding to the drug compared with patients without it. The 95% confidence interval means researchers are 95% certain the true value falls between 1.53 and 7.66. Because the entire interval sits above 1, the finding is statistically significant. A p-value of 0.003 means there is only a 0.3% chance this result happened by random luck.\u003c\/p\u003e\n\n\u003cp\u003eGershenson and colleagues studied trametinib, another MEK inhibitor. They observed that RAS or BRAF alteration was associated with a greater objective response rate than wild-type status (\u003cstrong\u003e50% vs 8%\u003c\/strong\u003e). However, the test for interaction did not achieve statistical significance (p=0.11). In plain terms, researchers could not prove with confidence that the biomarker truly predicted response. Alteration status was also not a significant predictor of progression-free survival (p for interaction 0.72).\u003c\/p\u003e\n\n\u003cp\u003eBecause of these mixed signals, no clear consensus exists that MEK inhibitors should be limited to a single biomarker-defined population. The National Comprehensive Cancer Network (NCCN) guidelines reflect this uncertainty. They do not require biomarker positivity for MEK inhibitor use. In other words, a patient without a MAPK alteration can still receive a MEK inhibitor, and a patient with one might not respond as expected.\u003c\/p\u003e\n\n\u003ch3\u003eConsensus on MEK Biomarkers\u003c\/h3\u003e\n\n\u003cp\u003eThe optimal predictive biomarker for sensitivity of LGSC to MEK inhibitors is unknown. There is no blood test or tumor test yet that can reliably tell a doctor which patient will benefit from these drugs.\u003c\/p\u003e\n\n\u003ch2 id=\"endocrine\"\u003eEndocrine Therapy: Hormone Treatment and Its Biomarkers\u003c\/h2\u003e\n\n\u003cp\u003eEndocrine therapy uses hormones or hormone-blocking drugs to slow cancer growth. Because LGSC is often fueled by estrogen and progesterone, hormonal approaches matter. But whether hospitals routinely test for estrogen receptor (ER) or progesterone receptor (PR) positivity varies by institution.\u003c\/p\u003e\n\n\u003cp\u003eMost patients with LGSC have ER-positive or PR-positive disease. That raises a practical question: if nearly everyone is positive, should resources be spent testing at all? The panel found this debatable. Yet the testing question is not purely academic.\u003c\/p\u003e\n\n\u003cp\u003eWhether lack of ER or PR positivity should exclude patients from hormonal therapy in the primary maintenance setting is controversial. Maintenance therapy is treatment given after initial treatment to keep the cancer from returning.\u003c\/p\u003e\n\n\u003cp\u003eLow-PR status (Allred score \u0026lt;2) has been associated with increased copy number changes compared with high-PR tumors. An Allred score is a standard way of scoring hormone receptor positivity, combining the proportion of positive cells with the intensity of staining. Copy number changes are gains or losses of large DNA segments, often a sign of genomic instability. High-ER and high-PR status have been associated with improved overall survival.\u003c\/p\u003e\n\n\u003cp\u003eHowever, no study has reliably shown that immunohistochemistry levels correlate with response to endocrine therapy. Immunohistochemistry is the laboratory technique used to detect proteins like ER and PR on tissue slides. Identifying predictive biomarkers for endocrine therapy is challenging due to the inherently low response rates observed in clinical trials.\u003c\/p\u003e\n\n\u003cp\u003eHere are the numbers that frame the challenge: a benefit of endocrine therapy is observed, with \u003cstrong\u003estable disease rates of 50-62%\u003c\/strong\u003e. Stable disease means the cancer does not grow or shrink; it just holds steady. In contrast, objective response rates (where tumors measurably shrink) range from \u003cstrong\u003e9% to 14%\u003c\/strong\u003e in the recurrent setting. So endocrine therapy is more likely to hold the disease steady than to shrink it.\u003c\/p\u003e\n\n\u003cp\u003eAnother consideration involves ESR1 alterations. ESR1 is the gene that encodes the estrogen receptor. Based on evidence from breast and endometrial cancers, development of ESR1 alterations would be expected to confer resistance to aromatase inhibitor therapy. Aromatase inhibitors are drugs that block the enzyme converting androgens into estrogen, thereby lowering estrogen levels.\u003c\/p\u003e\n\n\u003ch3\u003eConsensus on Endocrine Biomarkers\u003c\/h3\u003e\n\n\u003cp\u003eThe optimal biomarker for sensitivity of LGSC to endocrine therapy is unknown. Perhaps the most striking statement from the panel: \u003cstrong\u003eER-positive disease does not correlate with efficacy of hormonal therapy\u003c\/strong\u003e. A tumor can be packed with estrogen receptors and still ignore hormone-blocking drugs.\u003c\/p\u003e\n\n\u003ch2 id=\"epidemiology\"\u003eEpidemiology: How Common Is This Disease\u003c\/h2\u003e\n\n\u003cp\u003eThe panel classifies LGSC as a distinct rare disease. Under the Orphan Drug Act, the US Food and Drug Administration (FDA) defines a rare disease as one affecting \u003cstrong\u003efewer than 200,000 people in the USA\u003c\/strong\u003e. This definition matters practically: orphan drug status creates financial incentives for pharmaceutical companies to develop treatments for small patient populations.\u003c\/p\u003e\n\n\u003cp\u003eCurrent prevalence estimates for LGSC (the total number of people living with the disease at a given time) are not available. However, it has been estimated that LGSC represents less than 10% of new epithelial ovarian cancer cases. Given that the prevalence of all ovarian cancer cases in the USA was estimated at \u003cstrong\u003e233,565 in 2019\u003c\/strong\u003e, even with an approximate doubling in life expectancy, the prevalence of LGSC would not be expected to exceed 200,000 cases in the USA.\u003c\/p\u003e\n\n\u003cp\u003eLGSC is also pathologically distinct from HGSC in an important way: it lacks BRCA-associated etiology. BRCA genes (BRCA1 and BRCA2) normally repair damaged DNA. When they are mutated, DNA errors accumulate and cancer can develop. LGSC is not driven by this mechanism, which sets it apart from many HGSC cases.\u003c\/p\u003e\n\n\u003ch3\u003eConsensus on Classification\u003c\/h3\u003e\n\n\u003cp\u003eIt is appropriate to classify LGSC as a distinct rare disease. This classification has implications for research funding, drug development, and patient advocacy.\u003c\/p\u003e\n\n\u003ch2 id=\"risk\"\u003eRisk Factors and the BRCA Question\u003c\/h2\u003e\n\n\u003cp\u003eLimited evidence from a Danish population-based case-control study helps identify factors affecting the risk of SBT, the precursor lesion to many LGSC cases. A case-control study compares people with the condition to similar people without it. The findings suggest:\u003c\/p\u003e\n\n\u003cul\u003e\n  \u003cli\u003e\n\u003cstrong\u003eLower risk of SBT\u003c\/strong\u003e associated with: parity (having given birth), older age at first birth, and oral contraceptive use\u003c\/li\u003e\n  \u003cli\u003e\n\u003cstrong\u003eGreater risk of SBT\u003c\/strong\u003e associated with: infertility and hormone replacement therapy\u003c\/li\u003e\n\u003c\/ul\u003e\n\n\u003cp\u003eWhether BRCA alteration is associated with LGSC is an important consideration for patients and their families. The worry is understandable: if LGSC ran in families through BRCA mutations, relatives would need genetic testing and heightened surveillance. The data say otherwise.\u003c\/p\u003e\n\n\u003cp\u003eMeager rates of BRCA mutation have been observed in LGSC cohorts, generally ranging from \u003cstrong\u003e0% to 5%\u003c\/strong\u003e. One notable study by Vineyard and colleagues evaluated personal and family histories of patients with ovarian cancer to elucidate factors suggestive of hereditary breast and ovarian cancer (HBOC) syndrome. They found that women with LGSC had a significantly lower risk estimate of hereditary breast and ovarian cancer than patients with HGSC. In plain terms, LGSC does not raise the same red flags for inherited cancer risk that HGSC does.\u003c\/p\u003e\n\n\u003cp\u003eA second study corroborated this finding. It employed a secondary pathologic review (a second expert look at the tissue) and found \u003cstrong\u003eno BRCA germline mutations among 79 patients with LGSC\u003c\/strong\u003e treated at a comprehensive cancer center in a geographic region enriched with patients of Ashkenazi Jewish descent. Ashkenazi Jewish women carry higher rates of specific BRCA mutations, making this population a sensitive test of the BRCA-LGSC connection. Even in that enriched population, no mutations appeared.\u003c\/p\u003e\n\n\u003cp\u003eOverall, LGSC is not considered to be BRCA-associated. Still, as discussed below, the panel recommends that all patients with epithelial ovarian cancer undergo germline testing anyway.\u003c\/p\u003e\n\n\u003ch3\u003eConsensus on Risk Factors\u003c\/h3\u003e\n\n\u003cp\u003eMost participants agreed that the current evidence does not suggest that LGSC is driven by BRCA mutation. The reassurance extends to family members: the inherited cancer risk profile for LGSC families looks different from that of HGSC families.\u003c\/p\u003e\n\n\u003ch2 id=\"germline\"\u003eGenetic Testing: What Every Patient Should Know\u003c\/h2\u003e\n\n\u003cp\u003eGermline testing examines the DNA you inherited from your parents. It looks for mutations present in every cell of your body. Germline testing is recommended for all patients with newly diagnosed ovarian cancer in guidelines from the American Society of Clinical Oncology (ASCO) and the NCCN. The recommendation exists for practical reasons that go beyond LGSC itself.\u003c\/p\u003e\n\n\u003cp\u003ePanelists outlined several reasons for routine testing:\u003c\/p\u003e\n\n\u003cul\u003e\n  \u003cli\u003e\n\u003cstrong\u003eHistologic uncertainty:\u003c\/strong\u003e Sometimes pathologists cannot be 100% certain whether a tumor is LGSC or HGSC. A germline BRCA result can provide a clue, since BRCA mutations are common in HGSC and rare in LGSC.\u003c\/li\u003e\n  \u003cli\u003e\n\u003cstrong\u003eChange in diagnosis over time:\u003c\/strong\u003e A tumor initially classified one way can be reclassified later. Knowing the germline status provides a stable reference point.\u003c\/li\u003e\n  \u003cli\u003e\n\u003cstrong\u003eRisk for both patient and family:\u003c\/strong\u003e Missing a BRCA alteration has serious consequences. Relatives could undergo unnecessary surveillance, or worse, miss opportunities for prevention and early detection.\u003c\/li\u003e\n  \u003cli\u003e\n\u003cstrong\u003eKnowledge generation:\u003c\/strong\u003e Routine testing will help provide additional knowledge about the true incidence of germline alterations in this population and whether any germline alterations are associated with LGSC. The more data collected, the better the science.\u003c\/li\u003e\n\u003c\/ul\u003e\n\n\u003ch3\u003eConsensus on Germline Testing\u003c\/h3\u003e\n\n\u003cp\u003eAll patients with LGSC should undergo germline testing, consistent with the testing recommendations for the overall population of patients with epithelial ovarian cancer.\u003c\/p\u003e\n\n\u003ch2 id=\"somatic\"\u003eTumor Testing: Looking Inside the Cancer Itself\u003c\/h2\u003e\n\n\u003cp\u003eSomatic tumor testing examines the DNA within the tumor cells themselves. These mutations are acquired, not inherited. The NCCN guidelines recommend a tumor molecular analysis panel in the upfront setting for both LGSC and HGSC. \"Upfront\" means at the time of initial diagnosis, not later when the disease recurs.\u003c\/p\u003e\n\n\u003cp\u003eThe somatic tumor testing panel should test for a minimum set of genes: \u003cstrong\u003eKRAS, HRAS, NRAS, BRAF, NF1, and BRCA\u003c\/strong\u003e. Testing for this panel serves multiple purposes. KRAS, NRAS, and BRAF mutations guide MEK inhibitor decisions. NF1 loss also activates the MAPK pathway. BRCA mutations in the tumor can guide PARP inhibitor treatment decisions, though their role in LGSC remains less established.\u003c\/p\u003e\n\n\u003cp\u003eSome studies demonstrate changes in somatic tumor alterations in patients over time. A tumor that starts out KRAS wild-type can acquire a KRAS mutation later, or vice versa. However, these studies were limited by small patient numbers and single-institution designs. The panel noted specific situations where repeating somatic tumor testing is justified:\u003c\/p\u003e\n\n\u003cul\u003e\n  \u003cli\u003eAberrant clinical behavior (the tumor is not behaving as expected for its type)\u003c\/li\u003e\n  \u003cli\u003eClinical trial eligibility (many trials require a fresh biopsy or current mutation status)\u003c\/li\u003e\n  \u003cli\u003eProlonged disease course (a decade-long disease may evolve genetically)\u003c\/li\u003e\n  \u003cli\u003eCases of mixed LGSC\/HGSC, to identify which component is active and driving the current clinical situation\u003c\/li\u003e\n\u003c\/ul\u003e\n\n\u003ch3\u003eConsensus on Somatic Testing\u003c\/h3\u003e\n\n\u003cp\u003eA somatic tumor testing panel should be conducted at diagnosis in patients with LGSC, consistent with current NCCN guidelines. Repeat somatic tumor testing may be justified in certain cases, particularly those listed above.\u003c\/p\u003e\n\n\u003ch2 id=\"prognosis\"\u003ePrognostic Factors: What Predicts Outcomes\u003c\/h2\u003e\n\n\u003cp\u003eTwo factors consistently predict worse outcomes in newly diagnosed LGSC: \u003cstrong\u003eresidual disease at the end of primary therapy\u003c\/strong\u003e (cancer left behind after surgery) and \u003cstrong\u003eage ≤35 years\u003c\/strong\u003e. Residual disease gives the cancer a foothold to regrow. Younger age predicting worse outcomes seems counterintuitive but has been observed repeatedly in LGSC research. Younger women may present with more aggressive tumor biology or different hormonal environments.\u003c\/p\u003e\n\n\u003cp\u003eMore recently, researchers have evaluated the prognostic implications of MAPK alterations. The results are intriguing:\u003c\/p\u003e\n\n\u003cul\u003e\n  \u003cli\u003eIn the MILO\/ENGOT-ov11 and GOG-0281\/LOGS clinical trials, MAPK pathway alterations were associated with prolonged progression-free survival in the standard-of-care arms. However, these differences were not statistically significant.\u003c\/li\u003e\n  \u003cli\u003eGershenson and colleagues observed statistically significantly prolonged overall survival among patients with MAPK pathway alterations versus MAPK wild-type: \u003cstrong\u003emedian 148 months vs 78 months, respectively (p=0.001)\u003c\/strong\u003e. That is a survival difference of roughly 12.3 years versus 6.5 years.\u003c\/li\u003e\n  \u003cli\u003eManning-Geist and colleagues found even more dramatic differences: \u003cstrong\u003emedian 339 months vs 125 months, respectively (p=0.02 in multivariate analysis)\u003c\/strong\u003e. That is about 28 years versus 10.4 years.\u003c\/li\u003e\n\u003c\/ul\u003e\n\n\u003cp\u003eA p-value of 0.001 means there is only a 0.1% probability the survival difference occurred by chance. A p-value of 0.02 in multivariate analysis means the effect held up even after accounting for other variables like age, stage, and residual disease.\u003c\/p\u003e\n\n\u003cp\u003eOther potential prognostic factors that require further evaluation include:\u003c\/p\u003e\n\n\u003cul\u003e\n  \u003cli\u003eObesity\u003c\/li\u003e\n  \u003cli\u003eCA-125 levels (pretreatment or normalization of this blood marker)\u003c\/li\u003e\n  \u003cli\u003eLymph node ratio (the proportion of lymph nodes containing cancer)\u003c\/li\u003e\n  \u003cli\u003eLymphovascular space invasion (cancer cells invading blood or lymph vessels)\u003c\/li\u003e\n  \u003cli\u003eOmental involvement (spread to the omentum, the fatty apron of tissue over the abdomen)\u003c\/li\u003e\n  \u003cli\u003emRNA expression of Ki67 and polo-like kinase-1 (Plk1), both markers of cell proliferation\u003c\/li\u003e\n\u003c\/ul\u003e\n\n\u003ch3\u003eConsensus on Prognostic Factors\u003c\/h3\u003e\n\n\u003cp\u003eResidual disease at the end of primary therapy and younger age are associated with poor prognosis in patients with LGSC. In general, MAPK alteration is associated with improved prognosis. This makes biological sense: tumors with MAPK mutations may be more indolent (slow-growing), while those without identifiable MAPK drivers may rely on other, more aggressive pathways.\u003c\/p\u003e\n\n\u003ch2 id=\"imaging\"\u003eImaging: How the Disease Is Tracked\u003c\/h2\u003e\n\n\u003cp\u003eWhether a preferred imaging technique exists specifically for LGSC is unknown. Imaging techniques vary according to institution. The NCCN guidelines recommend computed tomography (CT), positron emission tomography (PET)-CT, PET head to thigh, or magnetic resonance imaging (MRI). Each technique has strengths and weaknesses, and none has proven superior for LGSC specifically.\u003c\/p\u003e\n\n\u003cp\u003ePanelists agreed that clinicians should follow the NCCN guidelines to the extent possible. They concurred that the chest, abdomen, and pelvis should all be included in CT imaging. Missing the chest could mean missing lung metastases. A majority of panelists prefer CT as the primary imaging modality for tracking this disease.\u003c\/p\u003e\n\n\u003ch2 id=\"limitations\"\u003eLimitations: What the Consensus Could Not Answer\u003c\/h2\u003e\n\n\u003cp\u003eThis consensus report, like any scientific document, has important limitations. The original text was cut short, but several limitations are explicit within the content provided:\u003c\/p\u003e\n\n\u003cul\u003e\n  \u003cli\u003e\n\u003cstrong\u003eRare disease, small studies:\u003c\/strong\u003e Many findings come from studies with small patient numbers and single-institution designs. The data on somatic tumor changes over time, for example, were limited by these factors.\u003c\/li\u003e\n  \u003cli\u003e\n\u003cstrong\u003eVariable definitions:\u003c\/strong\u003e The risk of recurrence with non-invasive implants varies widely across studies (16% to 44%). Differences in study populations, length of follow-up, and pathologic definitions of implants may explain this variability.\u003c\/li\u003e\n  \u003cli\u003e\n\u003cstrong\u003eUnvalidated biomarkers:\u003c\/strong\u003e No optimal predictive biomarker exists for MEK inhibitor sensitivity. The trametinib data showed a 50% vs 8% response difference that failed to reach statistical significance (p=0.11). The binimetinib data showed significance for KRAS, but not all patients without KRAS mutations lacked benefit.\u003c\/li\u003e\n  \u003cli\u003e\n\u003cstrong\u003eHormone receptor testing uncertainty:\u003c\/strong\u003e ER positivity does not correlate with hormonal therapy efficacy. No study has reliably shown that immunohistochemistry levels predict endocrine therapy response.\u003c\/li\u003e\n  \u003cli\u003e\n\u003cstrong\u003eRare mixed tumors:\u003c\/strong\u003e Cases of LGSC coexisting with HGSC are too few to draw conclusions about clinical behavior or optimal treatment.\u003c\/li\u003e\n  \u003cli\u003e\n\u003cstrong\u003eNo imaging standard:\u003c\/strong\u003e Whether any imaging technique is superior for LGSC remains unknown.\u003c\/li\u003e\n\u003c\/ul\u003e\n\n\u003cp\u003eThe panel itself acknowledged key unanswered questions. The extent of invasive disease as a prognostic factor has not been well studied. The mechanisms of tumorigenesis in MAPK wild-type tumors are not well defined. Whether LGSC arises from fallopian tube progenitor cells remains controversial. And no evidence supports opportunistic salpingectomy for LGSC prevention. Each of these gaps represents a target for future research.\u003c\/p\u003e\n\n\u003ch2 id=\"recommendations\"\u003eRecommendations for Patients\u003c\/h2\u003e\n\n\u003cp\u003eBased on this expert consensus, here is what patients with LGSC or SBT should understand and discuss with their care team:\u003c\/p\u003e\n\n\u003col\u003e\n  \u003cli\u003e\n\u003cstrong\u003eGet the diagnosis confirmed by an expert pathologist.\u003c\/strong\u003e LGSC can be confused with SBT and other serous neoplasms. Terminology has changed over the years, and some cases are subjective. A pathologist experienced in these lesions provides the most accurate classification. Ask whether your slides have been reviewed by a gynecologic pathology specialist.\u003c\/li\u003e\n  \u003cli\u003e\n\u003cstrong\u003eUnderstand what your diagnosis does and does not mean.\u003c\/strong\u003e If you have SBT with microinvasion or non-invasive implants, your prognosis is excellent (over 90% survival at 10 years), and chemotherapy is not recommended. Recurrences can happen late, so extended follow-up matters even years after diagnosis.\u003c\/li\u003e\n  \u003cli\u003e\n\u003cstrong\u003eUndergo germline genetic testing.\u003c\/strong\u003e This is recommended for all patients with newly diagnosed ovarian cancer. Although LGSC is not considered BRCA-associated (BRCA mutation rates are only 0-5%), testing provides certainty for you and your family. It also contributes to scientific knowledge.\u003c\/li\u003e\n  \u003cli\u003e\n\u003cstrong\u003eRequest somatic tumor testing at diagnosis.\u003c\/strong\u003e The NCCN recommends testing for KRAS, HRAS, NRAS, BRAF, NF1, and BRCA. Results can guide treatment decisions, particularly around MEK inhibitors. Repeat testing may be appropriate if your disease behaves unexpectedly, if you are considering a clinical trial, or if your disease course has been very long.\u003c\/li\u003e\n  \u003cli\u003e\n\u003cstrong\u003eKnow that hormone therapy works differently than chemotherapy.\u003c\/strong\u003e Endocrine therapy produces stable disease in 50-62% of patients but objective shrinkage in only 9-14%. That does not mean it is a weak treatment; holding the disease steady is a legitimate and valuable goal in a chronic, indolent cancer like LGSC.\u003c\/li\u003e\n  \u003cli\u003e\n\u003cstrong\u003eUnderstand the role of MAPK alterations in your prognosis.\u003c\/strong\u003e If your tumor has a MAPK pathway alteration, your overall survival may be significantly longer (median 148 vs 78 months in one study, and 339 vs 125 months in another). This information can help frame expectations.\u003c\/li\u003e\n  \u003cli\u003e\n\u003cstrong\u003eKnow that residual disease matters.\u003c\/strong\u003e Complete surgical resection at the time of primary therapy is associated with better outcomes. If you are facing initial surgery, discuss with your surgeon whether optimal debulking (removing all visible disease) is achievable and what that means for your plan.\u003c\/li\u003e\n  \u003cli\u003e\n\u003cstrong\u003eIf you are under 35, be aware of your risk profile.\u003c\/strong\u003e Younger age at diagnosis is associated with worse outcomes in LGSC. This does not mean your prognosis is dire, but it does mean your care team should be especially vigilant and proactive.\u003c\/li\u003e\n  \u003cli\u003e\n\u003cstrong\u003eFollow NCCN imaging guidelines.\u003c\/strong\u003e CT, PET-CT, PET head to thigh, or MRI are all acceptable. CT should include the chest, abdomen, and pelvis.\u003c\/li\u003e\n  \u003cli\u003e\n\u003cstrong\u003eSeek out clinical trials.\u003c\/strong\u003e LGSC is a rare disease with specific biology. The panel strongly supports ongoing research into MEK inhibitors, endocrine therapy combinations, and novel targeted agents. Ask your doctor about trial eligibility, including whether repeat biopsy or somatic testing is needed for enrollment.\u003c\/li\u003e\n  \u003cli\u003e\n\u003cstrong\u003eIf you have SBT, do not rush into aggressive treatment.\u003c\/strong\u003e For SBT with non-invasive implants, chemotherapy offers no survival benefit and can cause harm. Studies have reported more deaths from treatment complications than from the disease itself. The panel recommends extended clinical follow-up instead.\u003c\/li\u003e\n  \u003cli\u003e\n\u003cstrong\u003eIf your tumor shows both LGSC and HGSC components, expect HGSC-guided management.\u003c\/strong\u003e The panel's consensus is that if HGSC is substantial, the cancer should be managed as HGSC. Mixed tumors should not be treated in LGSC-specific trials.\u003c\/li\u003e\n\u003c\/ol\u003e\n\n\u003c!-- ddn:faq:start --\u003e\n\u003ch2 id=\"ddn-faq\"\u003eFrequently Asked Questions\u003c\/h2\u003e\n\u003ch3\u003eI was just diagnosed with low-grade serous ovarian cancer. Should I ask for a second opinion on the pathology?\u003c\/h3\u003e\n\u003cp\u003eYes. Low-grade serous carcinoma can be confused with borderline tumors and other serous neoplasms, and terminology has changed over the years. Some cases are subjective. The expert panel recommends consultation with a pathologist experienced in evaluating these lesions to ensure accurate classification, which guides whether chemotherapy is appropriate or not.\u003c\/p\u003e\n\u003ch3\u003eI have a serous borderline tumor with non-invasive implants. Do I need chemotherapy?\u003c\/h3\u003e\n\u003cp\u003eNo. For serous borderline tumors with non-invasive implants, adjuvant chemotherapy is not recommended. Survival is greater than 90% at 10 years, and studies report more deaths from treatment complications than from the disease itself. However, extended clinical follow-up is needed because recurrences can happen 5 years or more after diagnosis.\u003c\/p\u003e\n\u003ch3\u003eShould I get genetic testing if I have low-grade serous ovarian cancer?\u003c\/h3\u003e\n\u003cp\u003eYes. Germline testing is recommended for all patients with newly diagnosed ovarian cancer. Although low-grade serous carcinoma is not considered BRCA-associated, with mutation rates of 0-5%, testing provides certainty for you and your family, helps clarify any histologic uncertainty, and contributes to scientific knowledge about this rare disease.\u003c\/p\u003e\n\u003ch3\u003eWhat does somatic tumor testing look for in low-grade serous ovarian cancer?\u003c\/h3\u003e\n\u003cp\u003eAt diagnosis, tumor testing should check at least KRAS, HRAS, NRAS, BRAF, NF1, and BRCA. Results can guide treatment decisions, particularly around MEK inhibitors. Repeat testing may be justified if the disease behaves unexpectedly, if you are considering a clinical trial, or if your disease course has been very long.\u003c\/p\u003e\n\u003ch3\u003eMy tumor has a MAPK pathway alteration. Does that affect my prognosis?\u003c\/h3\u003e\n\u003cp\u003eSome studies show that MAPK pathway alterations are associated with longer overall survival. In one study, median survival was 148 months versus 78 months; in another, 339 months versus 125 months. However, these findings come from specific studies, and individual outcomes vary. Discuss what this means for your care with your oncology team.\u003c\/p\u003e\n\u003ch3\u003eHow well does hormone therapy work for low-grade serous ovarian cancer?\u003c\/h3\u003e\n\u003cp\u003eEndocrine therapy holds the disease steady in 50-62% of patients and causes measurable tumor shrinkage in only 9-14% of patients in the recurrent setting. Stable disease is a valuable outcome for this chronic, slow-growing cancer. Notably, estrogen receptor positivity does not reliably predict whether hormone therapy will be effective.\u003c\/p\u003e\n\u003ch3\u003eIf I am under 35, is my prognosis worse with low-grade serous ovarian cancer?\u003c\/h3\u003e\n\u003cp\u003eYounger age at diagnosis, specifically age 35 or younger, is associated with worse outcomes in low-grade serous carcinoma, according to expert consensus. This does not mean your prognosis is dire, but your care team should be especially vigilant and proactive about treatment and follow-up. Discuss your individual risk factors and treatment plan with your doctor.\u003c\/p\u003e\n\u003c!-- ddn:faq:end --\u003e\n\n\u003ch2 id=\"source\"\u003eSource Information\u003c\/h2\u003e\n\n\u003cp\u003e\u003cstrong\u003eOriginal Article Title:\u003c\/strong\u003e Low-grade serous ovarian cancer- expert consensus report\u003c\/p\u003e\n\u003cp\u003e\u003cstrong\u003eDOI:\u003c\/strong\u003e \u003ca href=\"https:\/\/doi.org\/10.1136\/ijgc-2023-004610\" target=\"_blank\" rel=\"noopener\"\u003e10.1136\/ijgc-2023-004610\u003c\/a\u003e\u003c\/p\u003e\n\n\u003cp\u003e\u003cstrong\u003eAuthors:\u003c\/strong\u003e Rachel N Grisham, Brian M Slomovitz, Nicole Andrews, Susana Banerjee, Jubilee Brown, Mark S Carey, Herman Chui, Robert L Coleman, Amanda N Fader, Stephanie Gaillard, Charlie Gourley, Anil K Sood, Bradley J Monk, Kathleen N Moore, Isabelle Ray-Coquard, Ie-Ming Shih, Shannon N Westin, Kwong-Kwok Wong, David M Gershenson\u003c\/p\u003e\n\n\u003cp\u003e\u003cstrong\u003ePublication:\u003c\/strong\u003e International Journal of Gynecological Cancer, 2023; Volume 33, pages 1331-1344. Published Online First on 17 August 2023. DOI: 10.1136\/ijgc-2023-004610\u003c\/p\u003e\n\n\u003cp\u003e\u003cstrong\u003eNote:\u003c\/strong\u003e This patient-friendly article is based on peer-reviewed research published by the International Gynecologic Cancer Society (IGCS) and the European Society of Gynaecological Oncology (ESGO). It was translated for educational purposes. The consensus report was presented at a one-day expert workshop held in New York, USA, in October 2022. This patient summary preserves the scientific content, data, and conclusions of the original publication while explaining medical terminology in plain language.\u003c\/p\u003e","brand":"DiagnosticDetectives.Com","offers":[{"title":"Default Title","offer_id":47542002286748,"sku":null,"price":0.0,"currency_code":"RUB","in_stock":true}],"url":"https:\/\/diagnosticdetectives.ru\/products\/low-grade-serous-ovarian-cancer-what-experts-agree-on-in-2023","provider":"DiagnosticDetectives.Com","version":"1.0","type":"link"}