Ovarian cancer is a group of diseases that originates in the ovaries, or in the related areas of the fallopian tubes and the peritoneum. Women have two ovaries that are located in the pelvis, one on each side of the uterus. The ovaries make female hormones and produce eggs. Women have two fallopian tubes that are a pair of long, slender tubes on each side of the uterus. Eggs pass from the ovaries through the fallopian tubes to the uterus. The peritoneum is the tissue lining that covers organs in the abdomen.
Worldwide, over 295,000 women were diagnosed with ovarian cancer in 2018 and nearly 185,000 died from this disease. In high-income/resource-rich countries, it is the third most common gynecologic cancer, after uterine and cervical cancer. In low-income/resource-limited countries, cervical cancer is the most common gynecologic cancer, followed by ovarian and uterine carcinoma.
The majority of ovarian malignancies (95 percent) are epithelial; the remainder arise from other ovarian cell types (germ cell tumors, sex cord-stromal tumors). The average age at diagnosis of ovarian cancer is 63 years old. The age at diagnosis of ovarian cancer is younger among patients with a hereditary ovarian cancer syndrome. The lifetime risk of developing ovarian cancer is 1.3 percent
An increased risk of EOC is associated with increasing age, infertility, endometriosis, polycystic ovarian syndrome, use of an intrauterine device, and cigarette smoking (for mucinous carcinomas). Factors that are associated with a decreased risk include previous pregnancy, history of breastfeeding, oral contraceptives, and tubal ligation.
Several ovarian cancer susceptibility genes, primarily BRCA1, BRCA2, as well as other genes in the homologous recombination pathway as well as mismatch repair genes associated with Lynch syndrome (hereditary nonpolyposis colorectal cancer) have been identified. cells.
Most commonly, ovarian cancer presents in a subacute fashion (eg, pelvic or abdominal pain, bloating, gastrointestinal symptoms) in patients with either early or advanced disease. Alternatively, an adnexal mass may be discovered incidentally at the time of imaging performed for another indication.
Up to 80 percent of patients with epithelial ovarian cancer will have an elevated CA125. However patients with germ cell ovarian tumour may have elevated levels of AFP, B-HCG and LDH, while patients with sex cord stromal tumours may have elevated Inhibin A or B, testosterone or estrogen levels.
Imaging studies like abdominal and pelvic CT or MRI can help assess for the presence of ascites and disease spread. Chest CT is often performed at the time of abdominal and pelvic CT to evaluate for pleural effusion, pulmonary metastases, and mediastinal lymphadenopathy. Positron emission tomography (PET) alone or combined with CT increases the detection of metastatic EOC.
In patients with ascites or pleural effusion, paracentesis or thoracentesis may be performed, respectively. Cytologic evaluation can establish the diagnosis of malignancy, but the overall sensitivity of these tests is variable. In patients with suspected omental or pleural metastases, biopsies may help guide diagnosis.
Image-guided biopsy of the ovary is not recommended; incising or rupturing the mass can lead to spillage of malignant cells, which may result in a more advanced stage of disease and adversely affects prognosis
All patients with a diagnosis of ovarian, fallopian tube, or peritoneal cancer should have a genetic risk evaluation, irrespective of their family history. Patients with epithelial carcinoma of the ovary should be offered testing for BRCA1 or BRCA2 mutations as well as other familial cancer syndromes (eg, Lynch syndrome). Patients with clear cell, endometrioid, or mucinous ovarian cancer should be offered testing for DNA mismatch repair deficiency.
1. Epithelial ovarian carcinomas: Based on histopathology, immunohistochemistry, and molecular genetic analysis, the five main subtypes of epithelial ovarian, fallopian tube, and peritoneal carcinomas and their relative proportions are:
2. Germ cell tumors: They begin in the reproductive cells. Germ cell tumors make up less than 2 percent of all ovarian cancers and have a high survival rate, with nine out of 10 patients surviving five years after diagnosis. Some of the most common subtypes of germ cell ovarian tumors are:
3. Stromal cell tumors: Even less common than ovarian germ cell tumors are ovarian stromal cell tumors (also called sex cord tumors and sex cord-gonadal stromal tumors), representing about 1 percent of all ovarian cancers. They develop from the stroma tissue cells that produce the female hormones of estrogen and progesterone
4. Stromal cell tumors: Even less common than ovarian germ cell tumors are ovarian stromal cell tumors (also called sex cord tumors and sex cord-gonadal stromal tumors), representing about 1 percent of all ovarian cancers. They develop from the stroma tissue cells that produce the female hormones of estrogen and progesterone
5. Ovarian sarcoma: Unlike their carcinoma counterparts, ovarian sarcoma tumors develop in the connective tissues of ovarian cells.
6. Borderline ovarian tumours: In the early 1970s, the histologic category of borderline ovarian epithelial neoplasms was introduced to describe a group of neoplasms that did not display overt malignant features (invasion), but that occasionally had intraperitoneal spread. Borderline neoplasm is the most widely used designation by pathologists, gynecologists, and oncologists, and has been adopted into the World Health Organization classification. Borderline neoplasms account for an estimated 14 to 15 percent of all primary ovarian neoplasms and 15 to 20 percent of ovarian serous neoplasms
Treatment for ovarian cancer usually involves a combination of surgery and chemotherapy. Treatment will depend on many factors, including:
Surgery is the main treatment for ovarian cancer.The goal of surgery is to remove the tumor. Total extrafascial hysterectomy and bilateral salpingo-oophorectomy with pelvic and paraaortic lymph node dissection is the standard staging procedure for epithelail ovarian cancer. Cytology is performed of pelvic washings and the surface of the diaphragm; omentectomy is also performed. Fertility preservation surgery is an acceptable option for young patients with ovarian germ cell/sex cord stromal tumors confined to the ovary.
Most patients with early-stage epithelial ovarian cancer should receive adjuvant chemotherapy, specifically those with stage IC or II disease, clear cell histology (any grade), or high tumor grade. Those with advanced disease (stage III or IV) should also receive adjuvant therapy if they underwent primary surgical cytoreduction. However, patients who are unlikely to become optimally cytoreduced (ie, <10 mm of residual disease at the end of surgery) or who are not good candidates for surgery due to medical comorbidities at the time of diagnosis may be considered for Neoadjuvant chemotherapy followed by surgery. For Germ cell tumour ovary, different chemotherapy protocol (BEP) is used.
Targeted therapies attack the cancer cells while doing little damage to normal cells in the body. Newer targeted therapies to treat advanced epithelial ovarian cancer include PARP inhibitors, which are drugs that block an enzyme used by cells to repair damage to their DNA. The three PARP inhibitors currently available include olaparib, niraparib and rucaparib. The addition of another drug, bevacizumab, has also been used with chemotherapy before as well as following surgery.
Approximately 80 percent of patients with early-stage disease are recurrence-free at five years. Younger patients are more likely to have a favorable prognosis because they are more likely to have tumors of less aggressive histology and lower grade, and better baseline performance status. Five-year survival rate is mentioned below in the table for different types of ovarian cancer.
| Stage | Invasive epithelial ovarian cancer | Ovarian stromal tumours | Germ Cell tumours of the overy |
|---|---|---|---|
| Localized | 92% | 99% | 98% |
| Regional | 72% | 89% | 95% |
| Distant | 30% | 61% | 75% |
Following all first-line treatment for epithelial ovarian cancer, monitoring should include routine history and physical, assessment of cancer antigen 125 (CA 125) (or other tumor markers if they were elevated on initial presentation), and other testing if clinically indicated.
The approach to treatment of recurrent ovarian cancer depends upon the amount of time that has elapsed between the completion of platinum-based treatment and the detection of relapse, known as the platinum-free interval (PFI). This is because the PFI correlates with progression-free survival (PFS), overall survival (OS), and response to subsequent treatment (both with platinum and nonplatinum agents as well as cytoreduction). Patients with a PFI of six months or longer are considered to have chemotherapy-sensitive disease (often also termed "platinum-sensitive"). Patients with a PFI of less than six months are considered to have chemotherapy-resistant disease (often also termed "platinum-resistant"). Of this group, those patients who progress while on platinum-based therapy are often referred to as having "platinum-refractory" disease. Treatment of recurrent ovarian cancer will depend on these above factors and the stage of disease.
The COVID-19 pandemic has increased the complexity of cancer care. Important issues include balancing the risk from treatment delay versus harm from COVID-19, ways to minimize negative impacts of social distancing during care delivery, and appropriately and fairly allocating limited health care resources. There is no "one size fits all" approach to delivering cancer care during the COVID-19 pandemic, and there are no international guidelines. Screening and treatment decisions must generally be made on a case-by-case basis, and are often dependent on the state of COVID-19 in an individual community and the availability of resources.
39 year old female patient had presented in August 2017 with abdominal distension. She was evaluated with CA125 which came as 585 IU/ml. Contrast enhanced CT abdomen pelvis done was suggestive of right ovarian mass with omental and peritoneal deposits and gross ascitis. Ascitic fluid analysis done was positive for malignancy. Patient was taken up for 3 cycles of neoadjuvant chemotherapy followed by surgery and then 3 cycles of adjuvant chemotherapy, completed the last cycle of chemotherapy on 30/1/2018. Patient is now on regular followup and recently done CA125 was normal. Imaging done during followup last year was normal.
