Neoplastic Diseases of the Female Reproductive System

Neoplastic Diseases of the Ovary, Fallopian Tube & Peritoneal Carcinoma

• The presentation focuses on neoplastic diseases affecting the female reproductive system, including the ovary, fallopian tubes, and peritoneum.
• Objectives include defining and classifying ovarian tumors, describing different types, examining epidemiology, gross and histological appearance, behavior, prognosis, and management.
• Staging classification, screening modalities, fallopian tube cancer, and peritoneal carcinoma are also discussed.
• Incidence of ovarian cancer is the second most common malignancy of the female genital tract and the leading cause of death from gynecologic neoplasms.
• It increases with age.
• US Cancer Statistics (2015) show 21,290 new cases and 14,180 deaths annually.
• Ovarian cancer is the 5th most prevalent cancer among females in the Philippines (2015).
• A clear-cut cause for ovarian cancer is not yet determined.
• Risk factors associated with increasing risk include age, diet, family history, industrialized countries, infertility, nulliparity, ovulation/ovulatory drugs, and talc.
• Risk factors associated with decreasing risk include breastfeeding, oral contraception, pregnancy, tubal ligation and hysterectomy with ovarian conservation.
• Reproductive factors, such as low parity and use of fertility drugs, contribute to an increased risk for epithelial ovarian carcinoma.
• Genetic factors, such as Breast-Ovarian cancer syndrome, Lynch type II, and site-specific ovarian cancer syndrome, are also risk factors.

Classification of Ovarian Neoplasms (WHO)

• Epithelial tumors comprise 65% of ovarian neoplasms, arising from inclusion cysts lined with coelomic epithelium.
• Germ cell tumors account for 25%.
• Sex cord-stromal tumors make up 6%.
• Lipid and gonadal blastoma are subcategories of sex cord-stromal tumors.

Epithelial Ovarian Neoplasms

• Serous tumors, originating from the endosalpinx, constitute 2/3 of all ovarian neoplasms (25-50%).
• 35-40% of all ovarian malignancies are serous tumors.
• They typically appear in postmenopausal women over 40, and are aggressive, often metastasizing to the ovarian surface and abdomen.

Benign Serous Tumors

• These tumors are typically unilateral and measure 1-10 cm in size, having a smooth and glistening external surface, and thin walls.
• Their internal surfaces may exhibit smooth structures or small papillary projections containing clear fluid.
• Cells are typically ciliated or columnar.

Malignant Serous Tumors

• These tumors are frequently bilateral (2/3 of cases), a combination of cystic and solid structures.
• Exhibit hemorrhagic papillary excrescences.
• Characterized by cribriform glands and tubules.
• The presence of stromal invasion is indicative of malignancy.

Mucinous Tumors

• Mucinous tumors make up 15-25% of all ovarian neoplasms, in women aged 30-60.
• They often are the largest ovarian tumors.
• Benign mucinous tumors are often unilateral, and have smooth inner linings filled with mucin.

Malignant Mucinous Tumors

• Malignant mucinous tumors are cystic with solid areas; they contain considerable necrosis and hemorrhage.
• Characterized by crowded glands and nuclear atypia with stromal invasion.

Endometrioid Tumors

• Endometrioid tumors (15-25% of ovarian malignancies) constitute 5% of all ovarian neoplasms.
• Primarily occur in women aged 50-60 and are often associated with endometriosis and endometriomas.

Endometrioid Adenocarcinoma

• Mostly unilateral and solid; containing bloody fluid inside the cyst.
• Epithelium mimics that of the endometrium.
• Characterized by stromal invasion
• Glands are often structured in a cribriform pattern

Clear Cell Tumors

• Clear cell tumors, make up 5% of ovarian neoplasms.
• Relatively rare among ovarian malignancies, and are highly aggressive.
• Primarily occur in women between 40 and 70.
• Not related to diethylstilbestrol (DES) exposure.

Clear Cell Carcinoma

• Cells contain abundant glycogen with a characteristic hobnail configuration.

Brenner Tumors

• These are rare tumors (2-3% of ovarian neoplasms).
• Occurs in women aged 50.
• The tumors are grossly small, well circumscribed, solid, and lobulated .
• Cells resemble transitional epithelium of the bladder.

Fallopian Tube Cancer

• Fallopian tube cancer is rare (0.2%).
• Typically arises from the epithelial lining of the fallopian tube fimbria.
• A BRCA mutation has been implicated as a risk factor.
• Risk factors include infertility, low parity, early menarche, and late menopause

Peritoneal Carcinoma

• First described by Swerdlow in 1959.
• Diffusely involves the peritoneal surfaces, but spares or mildly affects the ovaries and fallopian tubes
• Incidence is 0.46 per 100,000 in the US.
• Risk factors are similar to those of ovarian and fallopian tube cancer.
• Theories regarding its origins include malignant transformation of the germinal epithelium or field effect of coelomic and germinal epithelium.

Ovarian Cancer Screening

• The recommended approach involves a combination of CA-125 testing, transvaginal ultrasound, and a pelvic exam.
• No cost-effective screening program currently exists.

Risk Reduction

• Early age at first pregnancy, early menopause, use of oral contraceptive pills (OCP), increased parity, and breastfeeding reduce the risk of ovarian cancer.

CA-125

• Elevated CA-125 levels (in 80% of nonmucinous ovarian cancers) can indicate the extent of tumor spread.
• However, 20% of ovarian cancers show no elevated levels.
• The test is also elevated in benign conditions (endometriosis, myoma, PID, liver disease, or CHF) and experiences fluctuations during premenopause.

High-Risk Populations

• Family history (2 or more affected 1st degree relatives) of breast or ovarian cancer increases risk.
• Ashkenazi Jewish women with a 1st degree relative with ovarian cancer are also at increased risk.

BRCA1/BRCA2 Mutations

• Mutations in BRCA1/2 increase the risk of epithelial ovarian cancer (30-70% lifetime risk).

Screening for High-Risk Populations

• Member of a high-risk population should consult a genetic counselor.
• Receive genetic education and counseling.
• Family history of breast and/or ovarian cancer should be discussed

Staging for Ovarian Carcinoma

• The FIGO (International Federation of Gynecology and Obstetrics) 2014 staging system classifies ovarian carcinoma based on the extent of tumor spread from stages 1 to 4.
• Stage 1: Tumor confined to one or both ovaries.
• Stage 2: Tumor spread to nearby pelvic organs.
• Stage 3: Tumor spread beyond the pelvis.
• Stage 4: Tumor spread beyond the abdomen.

Treatment

• Surgery is a primary treatment approach, involving systematic abdominal exploration, sampling of peritoneal washings, careful inspection, biopsy and resection, and removal of tumor with intact capsule.
• Total abdominal hysterectomy + bilateral salpingo-oophorectomy (THBSO) is also an approach.
• Unilateral salpingo-oophorectomy and/or surgical staging can be an option, especially for young people and/or those with grade 1-2 tumors.
• Chemotherapy is often required as an adjuvant treatment.
• Possible further approaches include infracolic omentectomy, biopsy of normal peritoneal surfaces, and complete surgical staging

Germ Cell Tumors

• Consist of dysgerminoma, embryonal carcinoma, extraembryonic structures (endodermal sinus tumor, choriocarcinoma), teratomas (mature, immature), dermoid cysts, and mixed forms.
• Most prevalent are derived from germ cells of the ovary accounting for 20-25% of all ovarian tumors.
• Germ cell tumors are second most common ovarian neoplasm

Sex Cord-Stromal Tumors

• These are derived from specialized ovarian stroma accounting for 6% of ovarian neoplasms
• Some types produce hormones, whereas others are non-functional.
• Granulosa-theca cell tumors are comprised primarily of granulosa cells, with varying proportions of theca cells and may produce estrogen causing vaginal bleeding
• Thecoma and fibromas are benign tumors of ovarian stroma (theca cells)
• Sertoli-Leydig cell tumours are very rare and originate from sex cord cells; this type of tumour can cause masculinization/virilization of a young woman.

Fallopian Tube and Peritoneal Cancer

• Fallopian tube cancer is rare, accounting for only 0.2% of cancers in women.
• Peritoneal cancer is a diffuse involvement of the peritoneal surfaces.
• Pathologic diagnostic criteria for both conditions are described, defining characteristics and differences between them.
• Treatment approaches including surgery and chemotherapy are discussed, distinguishing the treatment for each condition, providing details, stages, etc.

This quiz delves into neoplastic diseases affecting the ovary, fallopian tubes, and peritoneum. Participants will learn about ovarian tumor classification, staging, epidemiology, and management strategies. The focus includes understanding the incidence rates and risk factors associated with these cancers.