Polycystic ovarian disease (Stein-Leventhal syndrome) is an endocrine disorder of unknown etiology showing signs of androgen excess (clinical or biochemical), oligoovulation and/or anovulation, and polycystic ovaries.
Accurate diagnosis requires exclusion of other endocrine disorders that might affect reproduction. Patients are usually young women of reproductive age who present with oligomenorrhea or secondary amenorrhea, hirsutism, infertility, or obesity. Treatment is lifestyle change and hormone therapy.
Lab studies show elevated luteinizing hormone (LH), low follicle-stimulating hor-mone (FSH), and elevated testosterone. Gross examination is notable for bilaterally enlarged ovaries with multiple cysts; microscopic examination shows multiple cystic follicles.
Epithelial ovarian tumors are the most common form of ovarian tumor. Risk factors include nulliparity, family history, and germline mutations.
Previously, epithelial tumors were characterized by histology into the categories cystadenoma (benign), borderline, and cystadenocarcinoma. Now, serous tumors are classified as low grade and high grade for prognostic significance.
• Low grade serous tumors are associated with KRAS, BRAF, or ERB2 mutations.
• Most high grade serous tumors have TP53 mutations.
The most common malignant ovarian tumor is serous cystadenocarcinoma. Hereditary risk factors include BRCA1 (breast and ovarian cancers) and Lynch syndrome.
• Well-differentiated serous tumors show psammoma bodies and a lining simi-lar to that of the fallopian tube.
Mucinous tumors commonly have goblet cells like intestinal mucosal cells.
CA 125 can be used to follow treatment.
• Teratoma (dermoid cyst)
°° Vast majority (>95%) of ovarian (but not testicular) teratomas are benign; commonly occurs in early reproductive years
°° Include elements from all 3 germ cell layers: ectoderm (skin, hair, adnexa, neural tissue), mesoderm (bone, cartilage), and endoderm (thyroid, bronchial tissue)
°° Complications include torsion, rupture, and malignant transformation
°° Can contain hair, teeth, and sebaceous material
°° The term struma ovarii is used when there is a preponderance of thyroid tissue
°° Immature teratoma is characterized by histologically immature tissue
°° Malignant; commonly occurs in children and young adults
°° Risk factors include Turner syndrome and disorders of sexual develop-ment
°° Gross and microscopic features are similar to seminomas
°° Are radiosensitive; prognosis is good
• Ovarian fibroma
ü Meigs syndrome refers to the combination of fibroma, ascities, and pleu-ral effusion.
• Granulosa cell tumor
ü Potentially malignant, estrogen-producing tumorresentation depends on age:
ü Prepuberal patients with juvenile granulosa cell tumor present with precocious puberty
ü Reproductive age patients present with irregular menses
ü Postmenopausal patients present with vaginal bleeding
ü complications include endometrial hyperplasia and cancer
ü Tumor forms a yellow-white mass that microscopically shows polygonal tumor cells and formation of follicle-like structures (Call-Exner bodies)
• Sertoli-Leydig cell tumor (androblastoma) is an androgen-roducing tumor that presents with virilization, usually in young women.
Primary sites for metastatic tumor to the ovary include breast cancer, colon cancer, endometrial cancer, and gastric “signet-ring cell” cancer (Krukenberg tumor).
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