Epithelial carcinoma of the ovary, fallopian tube, and peritoneum: Clinical features and diagnosis

INTRODUCTION — Ovarian carcinoma is the second most common gynecologic malignancy (second to uterine carcinoma) and the most common cause of gynecologic cancer death in the United States and other resource-abundant countries. (See "Epithelial carcinoma of the ovary, fallopian tube, and peritoneum: Incidence and risk factors", section on 'Incidence'.)

The majority of ovarian malignancies (95 percent) are derived from epithelial cells (subtypes include high-grade serous, low-grade serous, endometrioid, clear cell, and mucinous); the remainder arise from other ovarian cell types (germ cell tumors, sex cord-stromal tumors (figure 1)) [1].

High-grade serous epithelial ovarian carcinoma (EOC), fallopian tube, and peritoneal carcinomas are considered a single clinical entity due to their shared clinical behavior and treatment. There is also accumulating evidence of a common pathogenesis for these carcinomas. We will use the term EOC to refer to this group of malignancies in the discussion that follows. Distinctions between these conditions, where present, will be addressed.

The clinical features and diagnosis of EOC are reviewed here with a focus on the most frequent subtype, epithelial serous malignancies.

Related topics are discussed in detail separately, including:

●An overview of ovarian carcinomas (see "Overview of epithelial carcinoma of the ovary, fallopian tube, and peritoneum")

●Screening of asymptomatic patients (see "Screening for ovarian cancer")

●Histopathology (see "Epithelial carcinoma of the ovary, fallopian tube, and peritoneum: Histopathology")

●Epidemiology and risk factors (see "Epithelial carcinoma of the ovary, fallopian tube, and peritoneum: Incidence and risk factors")

●Early symptoms (see "Early detection of epithelial ovarian cancer: Role of symptom recognition")

●Staging and surgical treatment (see "Epithelial carcinoma of the ovary, fallopian tube, and peritoneum: Surgical staging")

●Adjuvant therapy (see "Adjuvant therapy of early-stage (stage I and II) epithelial ovarian, fallopian tube, or peritoneal cancer" and "First-line chemotherapy for advanced (stage III or IV) epithelial ovarian, fallopian tube, and peritoneal cancer")

CLINICAL PRESENTATION — The clinical presentation of EOC may be acute, subacute, or in some cases, found incidentally during examination, imaging, or surgery for another indication.

Acute presentation — Patients presenting in an acute fashion are typically those with advanced disease who present with a condition that requires urgent evaluation and management.

Ascites — Malignancy-related ascites causes abdominal distension by two mechanisms: (1) tumor cells produce fluid in the peritoneal cavity and (2) metastatic disease on the diaphragm prevents fluid from flowing out of the peritoneal cavity. If imaging reveals ascites, paracentesis may be performed for diagnosis and therapeutic purposes. (See 'Pelvic and abdominal imaging' below and 'Fluid cytology or omental/pleural biopsy' below and "Evaluation of adults with ascites", section on 'Diagnosis'.)

Pleural effusion — Malignant pleural effusion causes shortness of breath (image 1); if chest imaging reveals a malignant pleural effusion, thoracentesis may be performed. (See "Pleural fluid analysis in adults with a pleural effusion" and 'Fluid cytology or omental/pleural biopsy' below and 'Pelvic and abdominal imaging' below.)

Bowel obstruction — Bowel obstruction often leads to severe nausea and vomiting. In patients with EOC, this is often accompanied by a finding of an abdominal mass on imaging (image 2). (See "Etiologies, clinical manifestations, and diagnosis of mechanical small bowel obstruction in adults" and 'Pelvic and abdominal imaging' below.)

Venous thromboembolism — Infrequently, patients with EOC initially present with venous thromboembolism (VTE (image 3)) [2,3]. In a retrospective study including over 12,000 patients with ovarian cancer, the incidence of VTE during the year preceding their cancer diagnosis was approximately three times higher than the general population (27 versus 9.5 cases per year; standard incidence rate 2.8, 95% CI 1.9-4.1) [2]. The role of screening for malignancy in patients with VTE is discussed separately. (See "Evaluating adult patients with established venous thromboembolism for acquired and inherited risk factors", section on 'Evaluation for occult malignancy'.)

Subacute presentation — More commonly, disease presents in a subacute fashion (eg, pelvic or abdominal pain, bloating, gastrointestinal symptoms). The type or severity of symptoms do not reliably correspond to disease stage. In addition, while EOC had historically been thought to be a silent disease, studies have found that symptoms may occur even at early stages of disease [4-11].

Pelvic and abdominal symptoms — Pelvic and abdominal symptoms may include:

●Bloating or abdominal distention (image 4).

●Urinary urgency or frequency.

●Difficulty eating, nausea, anorexia, or early satiety.

●Pelvic or abdominal pain – Anecdotal reports suggest patients with tubal carcinoma present with more intense pain than those with an ovarian carcinoma, possibly due to tubal distension [12].

In patients with advanced disease, these symptoms are typically due to the presence of ascites and omental or bowel metastases.

These symptoms are nonspecific and may also be caused by other conditions (eg, primary gastrointestinal or urologic disease (see 'Differential diagnosis' below)). Symptoms that warrant further evaluation for ovarian cancer are those that are of new onset, coexist with other symptoms, occur almost daily, and are more severe than expected. The Gynecologic Cancer Foundation, American Cancer Society, and Society of Gynecologic Oncologists issued a consensus statement regarding early symptoms in 2007 (table 1).

The role of pelvic or abdominal symptoms in the detection of ovarian cancer is discussed in detail separately. (See "Early detection of epithelial ovarian cancer: Role of symptom recognition".)

Vaginal bleeding — Some patients with EOC present with postmenopausal bleeding, although patients with postmenopausal bleeding should be first assessed for uterine pathology before proceeding with an evaluation for ovarian cancer [13,14]. (See 'Surveilling for other cancers' below and 'Differential diagnosis' below and "Approach to the patient with postmenopausal uterine bleeding", section on 'Etiology'.)

For fallopian tube carcinoma, while postmenopausal bleeding may occur [15], the classic triad of symptoms includes clear or blood-tinged vaginal discharge, pelvic pain, and a pelvic mass. The vaginal discharge, referred to as hydrops tubae profluens, has been regarded as pathognomonic for the disease [16]. However, vaginal discharge is not present in most patients with fallopian tube cancer (only 2 of 12 in one series [17]), and a complaint of vaginal discharge rarely results in the diagnosis of fallopian tube carcinoma.

Rare presentations

●Lymphadenopathy – Palpable inguinal or cervical lymphadenopathy is an uncommon presentation of EOC (image 5). (See "Evaluation of peripheral lymphadenopathy in adults", section on 'Localized lymphadenopathy'.)

●Rectal bleeding – Rectal bleeding is rare and is unlikely to be the only presenting symptom; further evaluation for EOC is only warranted if other clinical features of EOC are present (eg, adnexal mass, hereditary ovarian cancer syndrome) [13,14]. (See "Etiology of lower gastrointestinal bleeding in adults".)

●Paraneoplastic syndromes – Rarely, patients with EOC may present with, or develop, a paraneoplastic syndrome. Paraneoplastic syndromes associated with EOC include: cerebellar degeneration, polyneuritis, dermatomyositis, hemolytic anemia, disseminated intravascular coagulation, acanthosis, or nephrotic syndrome [18]. These syndromes are discussed in detail separately. (See "Overview of paraneoplastic syndromes of the nervous system" and "Malignancy and rheumatic disorders" and "Risk and prevention of venous thromboembolism in adults with cancer" and "Cutaneous manifestations of internal malignancy" and "Overview of kidney disease in patients with cancer" and "Cold agglutinin disease".)

Incidental finding

Adnexal mass — A diagnosis of EOC may result from finding an adnexal mass during a routine pelvic examination or imaging study performed for another indication. Patients with advanced disease may present with a pelvic mass that extends beyond the adnexa.

The clinical presentation of patients with an adnexal mass is discussed in detail separately. (See "Approach to the patient with an adnexal mass", section on 'Clinical presentation'.)

Atypical glandular cells on cervical cytology — Infrequently, patients with EOC present with atypical glandular cells (AGC) on cervical cytology. In a systematic review including almost 7000 patients with a Pap smear with AGC, 5.2 percent had a malignancy; among patients with malignancy, 5.4 percent were ovarian and 1 percent were fallopian tube carcinoma [19]. Patients with a finding of AGC should be first evaluated for cervical and endometrial carcinoma, but if these evaluations are negative, further evaluation for EOC should be performed. (See "Cervical cytology: Evaluation of atypical and malignant glandular cells", section on 'Patients with persistent AGC and negative colposcopy, endometrial biopsy, and conization'.)

Findings during surgery for another indication — In some cases, EOC is discovered incidentally at the time of surgery for another indication. For example, patients undergoing risk-reducing salpingo-oophorectomy surgery may be diagnosed with an underlying fallopian tube or ovarian cancer at the time of risk-reducing surgery or with an occult cancer on serial sectioning of the pathology specimens. (See "Risk-reducing salpingo-oophorectomy in patients at high risk of epithelial ovarian and fallopian tube cancer", section on 'Occult malignancy on pathology'.)

If malignancy is recognized intraoperatively, intraoperative consultation from a gynecologic oncologist should be requested, if available. If a gynecologic oncologist is not available and the surgeon is not experienced in operative management of ovarian cancer (eg, lymphadenectomy), it is prudent to terminate the surgery and arrange for prompt consultation with a specialist for a second procedure. (See 'Referral to a specialist' below.)

If ovarian cancer is discovered only upon histologic evaluation following surgery, the patient should be referred to a gynecologic oncologist.

DIAGNOSTIC EVALUATION

Overview — Evaluation of patients with features suggestive of EOC is typically a multiphase process:

●An initial evaluation, including complete physical examination, imaging (typically pelvic ultrasound), and laboratory studies, to determine whether an adnexal mass or elevated tumor markers are present; this will dictate whether further evaluation and surgery are indicated. (See 'Initial evaluation' below.)

●Subsequent evaluation, including further imaging (eg, computed tomography [CT] or magnetic resonance imaging [MRI]) and possible fluid or tissue sampling to exclude metastatic disease, an extraovarian (or extratubal) primary cancer, or a synchronous endometrial cancer. This evaluation may result in the decision to treat with neoadjuvant chemotherapy prior to surgery. (See 'Subsequent evaluation' below and "First-line chemotherapy for advanced (stage III or IV) epithelial ovarian, fallopian tube, and peritoneal cancer", section on 'Neoadjuvant chemotherapy'.)

●Surgical exploration to remove the specimen, make a histologic diagnosis, and perform staging. (See 'Surgical evaluation' below and 'Diagnosis' below.)

Initial evaluation — The primary goal of the initial evaluation for EOC is to determine the degree of clinical suspicion of malignancy. If there is a high likelihood of malignancy, the patient should be referred to a gynecologic oncologist. (See 'Referral to a specialist' below.)

Physical examination and pelvic imaging — The most important element of the evaluation of EOC is the finding of an adnexal mass on pelvic examination and imaging. Pelvic ultrasound is typically the preferred initial imaging study, though patients with an acute presentation may be initially imaged with CT. (See 'Acute presentation' above.)

Findings concerning for malignancy include, but are not limited to, an adnexal mass with a solid component that is not hyperechoic and is often nodular or papillary, Doppler demonstration of flow in the solid component, irregularly thick septations, and ascites. (See "Approach to the patient with an adnexal mass", section on 'Assessing the risk of malignancy'.)

Once a suspicious adnexal mass has been identified, laboratory evaluation for tumor markers and additional imaging studies may be performed. (See 'Serum biomarkers' below and 'Pelvic and abdominal imaging' below.)

For patients without an adnexal mass, but with symptoms of EOC (eg, abdominal bloating), the decision to proceed with surgery is based on either the combination of EOC-associated symptoms, an elevated tumor marker, and/or imaging findings consistent with peritoneal carcinomatosis. (See 'Pelvic and abdominal symptoms' above and 'Serum biomarkers' below and 'Pelvic and abdominal imaging' below.)

Management of benign-appearing adnexal masses is discussed separately. (See "Approach to the patient with an adnexal mass".)

Serum biomarkers — A baseline serum cancer antigen 125 (CA 125) should be drawn. Up to 85 percent of patients with EOC will have an elevated CA 125 [20], and posttreatment CA 125 testing is used to evaluate for response to treatment and recurrence [21].

Other tumor markers (eg, human epididymis protein 4, carcinoembryonic antigen, cancer antigen 19-9, lactate dehydrogenase, alpha fetoprotein) should be ordered as clinically indicated. This is discussed in detail elsewhere. (See "Adnexal mass: Role of serum biomarkers in diagnosing epithelial carcinoma of the ovary, fallopian tube, or peritoneum" and "Approach to the patient with an adnexal mass", section on 'Role of tumor markers and multimodal tests'.)

Subsequent evaluation

Assessing for metastatic disease — The medical history and physical examination of all patients with suspected EOC should include an assessment for symptoms and findings associated with metastatic disease (eg, gastrointestinal symptoms, abdominal distention, ascites, pleural effusion, inguinal or cervical lymphadenopathy). Preoperative assessment for metastatic disease helps the surgeon anticipate the need for cytoreduction and identify patients who are poor candidates for aggressive initial surgical cytoreduction due to imaging findings of extensive disease (eg, liver or pulmonary metastases, disease in the porta hepatis, massive ascites) and may be candidates for neoadjuvant chemotherapy. (See "First-line chemotherapy for advanced (stage III or IV) epithelial ovarian, fallopian tube, and peritoneal cancer", section on 'Neoadjuvant chemotherapy'.)

Pelvic and abdominal imaging — Imaging studies can help assess for the presence of ascites and disease spread. Abdominal and pelvic CT (image 6 and image 7) or MRI (image 8) are the most commonly used modalities. In our practice, we obtain an abdominal and pelvic CT because it is less expensive and more comfortable for the patient than MRI. If the patient has a contrast allergy that precludes use of CT, we order an MRI.

Some data suggest that positron emission tomography alone or combined with CT increases the detection of metastatic EOC compared with CT alone or MRI; further study of the preoperative use of this imaging modality is needed [22].

Other imaging studies — Chest CT is often performed at the time of abdominal and pelvic CT to evaluate for pleural effusion, pulmonary metastases, and mediastinal lymphadenopathy (image 9). Chest radiography, while not performed for staging, may also show evidence of disease spread, if present (image 10).

Bone scans and brain scans are unnecessary unless symptoms or signs suggest metastases to these sites.

Fluid cytology or omental/pleural biopsy — Some patients (eg, poor performance status, advanced disease that clinically appears unresectable) require neoadjuvant chemotherapy rather than primary surgery. For these patients, it is imperative a diagnosis be made prior to the start of medical treatment; this can be accomplished in several ways:

●Fluid cytology – 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, and more than one sample may be needed. This is discussed in more detail elsewhere. (See "Malignancy-related ascites", section on 'Cytology' and "Pleural fluid analysis in adults with a pleural effusion", section on 'Cytology, flow cytometry, and cancer-related biomarkers'.)

●Omental/pleural biopsies – In patients with suspected omental or pleural metastases, biopsies may help guide diagnosis as well as management decisions [23,24]. In a retrospective case series including 159 patients with peritoneal carcinomatosis, CT- or ultrasound-guided biopsy provided a site-specific diagnosis in 93 percent of patients [24]. The biopsy specimen should contain sufficient tissue so that immunostaining can be performed to help characterize a malignancy (ie, by type and grade). This is discussed in more detail elsewhere. (See "Malignancy-related ascites", section on 'Omental biopsy' and "Diagnostic evaluation of the hemodynamically stable adult with a pleural effusion", section on 'Pleural biopsy'.)

●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 (table 2) [25]. (See "Oophorectomy and ovarian cystectomy", section on 'Spillage of malignant cells'.)

Treatment with neoadjuvant chemotherapy is discussed in detail elsewhere. (See "First-line chemotherapy for advanced (stage III or IV) epithelial ovarian, fallopian tube, and peritoneal cancer", section on 'Neoadjuvant chemotherapy'.)

Surveilling for other cancers

●Although it is rare for a de novo metastatic breast or colon cancer to present similarly to EOC, those with a prior history of these cancers may present with metastatic disease to the ovary, or with symptoms that mimic ovarian cancer (image 11). In studies of 50 or more cases of metastatic neoplasms to the ovary, the sites of primary tumors included colon cancer (15 to 32 percent), breast (8 to 28 percent), gastric (6 to 22 percent), and appendix (2 to 20 percent) [26-28]. The clinical features and diagnosis of breast and gastrointestinal cancers are discussed in detail separately. (See 'Differential diagnosis' below and "Clinical presentation, diagnosis, and staging of colorectal cancer" and "Well-differentiated neuroendocrine tumors of the appendix" and "Clinical features, diagnosis, and staging of gastric cancer" and "Clinical features, diagnosis, and staging of newly diagnosed breast cancer".)

●Among gynecologic malignancies other than ovarian cancer, endometrial cancer is the most likely to present with an adnexal mass. Thus, patients with abnormal uterine bleeding or a uterine mass should undergo endometrial sampling prior to surgical exploration for presumed EOC. (See 'Differential diagnosis' below and "Overview of the evaluation of the endometrium for malignant or premalignant disease".)

Synchronous primary cancers of the ovary and endometrium can also occur and have been reported in approximately 10 percent of patients with ovarian cancer and 5 percent of patients with endometrial cancer [29]. Patients at an increased risk for both ovarian and endometrial cancer are those with Lynch syndrome (table 3) and those with an estrogen-secreting tumor (although these are sex cord-stromal tumors rather than epithelial carcinoma (table 4)). Other risk factors for synchronous cancers include younger age, obesity, and nulliparity, which suggest a hormonal effect [30]. (See "Endometrial carcinoma: Epidemiology, risk factors, and prevention", section on 'Estrogen-secreting tumors'.)

●Cervical, vaginal, or vulvar cancer are not commonly mistaken for ovarian cancer. These tumors may present with a pelvic mass but, rather than an isolated adnexal mass, these masses often occur at the site of origin of these cancers. (See "Invasive cervical cancer: Epidemiology, risk factors, clinical manifestations, and diagnosis" and "Vaginal cancer" and "Vulvar cancer: Epidemiology, diagnosis, histopathology, and treatment".)

Surgical evaluation — If symptoms associated with EOC and/or abdominal distention or ascites are present and tumor markers associated with EOC are elevated, a diagnostic laparoscopy should be considered as part of the evaluation and to assess cytoreductive feasibility (ie, Fagotti score). (See 'Diagnosis' below and "Cancer of the ovary, fallopian tube, and peritoneum: Surgical cytoreduction", section on 'Laparoscopy'.)

DIAGNOSIS — EOCs are histologic diagnoses. (See "Epithelial carcinoma of the ovary, fallopian tube, and peritoneum: Histopathology".)

For most patients, tissue is obtained during surgical removal of an ovary or fallopian tube or biopsies of the peritoneum. When the diagnosis is confirmed, staging and cytoreduction can be performed. Patients with early-stage disease (ie, no malignant cells in ascites or peritoneal cytology) benefit from removal of the adnexal mass intact since incising or rupturing the mass results in a more advanced stage of disease and adversely affects prognosis. In the case of metastatic disease, the goal of surgery is to resect the tumor to minimal (ideally zero) gross residual disease. (See "Epithelial carcinoma of the ovary, fallopian tube, and peritoneum: Surgical staging".)

In approximately one-third of patients with advanced disease, the diagnosis is based on tissue or fluid obtained via image-guided biopsy, paracentesis, or thoracentesis. This is discussed in more detail above. (See 'Fluid cytology or omental/pleural biopsy' above.)

Even though EOCs are considered a single clinical entity due to their shared clinical behavior and common pathogenesis, we attempt to distinguish between these malignancies and assign an apparent primary site (ie, ovary, tube, or peritoneum) as part of staging [21,31]. Treatment, however, is the same for all three sites. (See 'Introduction' above and "Adjuvant therapy of early-stage (stage I and II) epithelial ovarian, fallopian tube, or peritoneal cancer" and "First-line chemotherapy for advanced (stage III or IV) epithelial ovarian, fallopian tube, and peritoneal cancer".)

DIFFERENTIAL DIAGNOSIS — Because the diagnosis of EOC is based on histologic findings, and many of the symptoms associated with EOC (eg, abdominal pressure or pain, gastrointestinal symptoms, urologic symptoms) are nonspecific, it is important to carefully assess for other conditions. The differential diagnosis typically depends on the presence or absence of an adnexal mass and/or peritoneal carcinomatosis.

If an adnexal mass is present:

●The mass may be benign (table 5). (See "Adnexal mass: Differential diagnosis".)

●Malignant adnexal masses may be due to primary EOC, a nonepithelial primary ovarian cancer (eg, germ cell, sex-cord stromal, sarcoma [rare]), or metastatic from another site. (See "Ovarian germ cell tumors: Pathology, epidemiology, clinical manifestations, and diagnosis" and "Sex cord-stromal tumors of the ovary: Epidemiology, clinical features, and diagnosis in adults" and "Clinical presentation, histopathology, diagnostic evaluation, and staging of soft tissue sarcoma" and 'Surveilling for other cancers' above.)

In the absence of an adnexal mass, the differential diagnosis is broad and depends on other findings:

●If ascites or pleural effusion is present, the patient should be evaluated for other conditions associated with these conditions. (See "Evaluation of adults with ascites" and "Pleural fluid analysis in adults with a pleural effusion".)

●If postmenopausal bleeding is present, the patient should be assessed for uterine pathology. (See 'Surveilling for other cancers' above and "Approach to the patient with postmenopausal uterine bleeding" and "Overview of the evaluation of the endometrium for malignant or premalignant disease".)

If peritoneal carcinomatosis is found on imaging:

●There is a high index of suspicion for peritoneal carcinoma; however, findings suggestive of peritoneal carcinomatosis may result from other intraabdominal malignancies or metastasizing conditions associated with leiomyomas. (See "Adenocarcinoma of unknown primary site" and "Uterine fibroids (leiomyomas): Variants and smooth muscle tumors of uncertain malignant potential", section on 'Leiomyomas with disseminated, intravascular, or metastatic growth patterns'.)

TESTING FOR HEREDITARY CANCER SYNDROMES — All patients with a diagnosis of ovarian, fallopian tube, or peritoneal carcinoma should have genetic risk evaluation, irrespective of their family history, as the presence of a familial cancer syndrome (eg, BRCA1 or BRCA2, Lynch syndrome) may impact treatment, posttreatment care, and testing of other family members (ie, cascade testing). This is discussed in more detail separately. (See "Overview of epithelial carcinoma of the ovary, fallopian tube, and peritoneum", section on 'Testing for hereditary cancer syndromes' and "Genetic testing and management of individuals at risk of hereditary breast and ovarian cancer syndromes".)

REFERRAL TO A SPECIALIST — Patients in whom there is a high suspicion of EOC (eg, findings suggesting of metastatic EOC, elevated serum cancer antigen 125 level in a patient with a complex adnexal mass) should be referred to a gynecologic oncologist. There is evidence that prognosis is improved when EOC staging and cytoreduction is performed by a gynecologic oncologist.

Criteria for referral to a gynecologic oncologist are shown in the table (table 6); a computer algorithm (the Risk of Ovarian Cancer Algorithm [ROCA]) that incorporates age-specific incidence of cancer, absolute CA 125 level, and rate of CA 125 change over time can also be used to estimate ovarian cancer risk. (See "Approach to the patient with an adnexal mass" and "Screening for ovarian cancer", section on 'Multimodal (CA 125 and TVUS) tests'.)

Despite the above guidance, Black patients with ovarian cancer are less likely to receive consultation with a gynecologic oncologist compared with White patients [32]; this is consistent with other health disparity patterns and is discussed in detail separately. (See "Racial and ethnic inequities in obstetric and gynecologic care and role of implicit biases".)

SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Ovarian, fallopian tube, and peritoneal cancer".)

INFORMATION FOR PATIENTS — UpToDate offers two types of patient education materials, "The Basics" and "Beyond the Basics." The Basics patient education pieces are written in plain language, at the 5^th to 6^th grade reading level, and they answer the four or five key questions a patient might have about a given condition. These articles are best for patients who want a general overview and who prefer short, easy-to-read materials. Beyond the Basics patient education pieces are longer, more sophisticated, and more detailed. These articles are written at the 10^th to 12^th grade reading level and are best for patients who want in-depth information and are comfortable with some medical jargon.

Here are the patient education articles that are relevant to this topic. We encourage you to print or e-mail these topics to your patients. (You can also locate patient education articles on a variety of subjects by searching on "patient info" and the keyword(s) of interest.)

●Basics topics (see "Patient education: Ovarian cancer (The Basics)")

●Beyond the Basics topics (see "Patient education: Treatment of ovarian cancer (Beyond the Basics)" and "Patient education: Ovarian cancer diagnosis and staging (Beyond the Basics)")

SUMMARY AND RECOMMENDATIONS

●Clinical significance – Epithelial ovarian carcinoma (EOC), fallopian tube, and peritoneal carcinomas are considered a single clinical entity due to their shared clinical behavior and treatment. There is also accumulating evidence of a common pathogenesis for these carcinomas. (See 'Introduction' above.)

Nonetheless, we attempt to distinguish between these malignancies and assign an apparent primary site (ie, ovary, tube, or peritoneum) as part of the staging process. (See 'Diagnosis' above.)

●Clinical presentation – The clinical presentation of EOC may be acute (eg, pleural effusion, bowel obstruction), subacute (eg, pelvic and abdominal symptoms), or in some cases, found incidentally during examination, imaging, or surgery for another indication (eg, adnexal mass, occult fallopian tube carcinoma after salpingectomy). (See 'Clinical presentation' above.)

●Diagnostic evaluation – Evaluation of patients with features suggestive of EOC is typically a multi-phase process and includes:

•An initial evaluation, including physical examination, imaging (typically pelvic ultrasound), and laboratory studies to determine whether an adnexal mass and/or serum biomarkers (table 4) are present. (See 'Initial evaluation' above.)

•Subsequent evaluation, including further imaging (eg, CT or MRI) and possible fluid or tissue sampling to exclude metastatic disease, an extraovarian (or extratubal) primary cancer, or a synchronous endometrial cancer. (See 'Subsequent evaluation' above.)

•Surgical evaluation for removal of the intact specimen, histologic diagnosis, and staging. (See 'Surgical evaluation' above and 'Diagnosis' above.)

●Diagnosis – EOC is a histologic diagnosis. For most patients, tissue is obtained during surgical removal of an ovary or fallopian tube. Less commonly, the diagnosis is based on tissue or fluid obtained via image-guided biopsy (eg, omental or pleural biopsy), paracentesis, or thoracentesis. (See 'Diagnosis' above.)

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. (See 'Fluid cytology or omental/pleural biopsy' above.)

●Differential Diagnosis – The differential diagnosis typically depends on the presence or absence of an adnexal mass and/or peritoneal carcinomatosis. If an adnexal mass is present, the differential diagnosis includes both benign and malignant conditions (table 5). (See 'Differential diagnosis' above.)

●Testing for hereditary cancer syndromes – All patients with a diagnosis of EOC, irrespective of their family history, should have testing for hereditary cancer syndromes (eg, BRCA1 or BRCA2, Lynch syndrome). (See 'Testing for hereditary cancer syndromes' above.)

●When to refer – Patients in whom there is a high suspicion of EOC should be referred to a gynecologic oncologist (table 6). (See 'Referral to a specialist' above.)