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Flashcards in Pathology: Ovaries Deck (23)
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What is polycystic ovarian syndrome? What is it also known as? What is the key driving factors underlying the pathophysiology? What is the syndrome associated with?

- PCOS is a condition of hyperandrogenism due to overactive theca cells and/or hyperinsulinemia
- it is also known Stein-Leventhal syndrome
- the major underlying factors are an elevated LH:FSH ratio (of 3 or more) and hyperinsulinemia
- the elevated LH stimulates the theca cells to produce excess androgens, while the relative decrease in FSH leads to decreased activity of granulosa cells (so decreased aromatization of the androgens into estrogen)
- the excess insulin actually decreases steroid-hormone binding globulins (SHBGs), which preferentially bind androgens, resulting in elevated levels of free androgens
- PCOS is associated with obesity and T2DM


How do patients with PCOS present? PCOS is the most common cause of what? What are other potential complications?

- patients with PCOS are usually obese and diabetic
- they present with hirsutism, amenorrhea/*oligomenorrhea*, and infertility
- imaging reveals bilaterally enlarged cystic ovaries
- PCOS is the most common cause of infertility in women
- other major complication is endometrial carcinoma


Why do patients with PCOS have an increased risk of endometrial carcinoma when they have lower levels of estradiol?

- although patients have lower levels of estradiol (because the relatively low levels of FSH decrease aromatization in the granulosa cells) they still have an increased risk of developing endometrial carcinoma because of the peripheral aromatization in adipose tissue
- the excess free androgens (because of high LH and high insulin) get converted into estrone in adipose cells
- thus, although estradiol is lowered, overall estrogen levels are raised, leading to an increased risk of endometrial carcinoma


What are the three categories of ovarian tumors? How common is each?

- ovaries contain oocytes, granulosa and theca cells/stroma, and surface epithelium
- therefore, the 3 types are germ cell tumors, sex cord-stromal tumors, and surface epithelial tumors
- surface epithelial tumors are the most common (70%), followed by germ cell tumors, followed by sex cord-stromal tumors
- (note that 90% of malignant tumors are surface epithelial tumors)


What are the four major types of surface epithelial ovarian tumors? Which are the most common?

- serous tumors: serous cystadenoma (MC benign ovarian neoplasm overall), serous cystadenocarcinoma (MC malignant ovarian neoplasm overall)
- mucinous tumors: mucinous cystadenoma, mucinous cystadenocarcinoma
- endometrioma (most are malignant)
- Brenner tumor (benign)


What are the four major types of germ cell ovarian tumors? Which are the most common?

- teratomas: mature/benign (MC germ cell tumor; MC ovarian tumor in 20-30 year olds), immature/malignant
- dysgerminoma (malignant; MC ovarian tumor in adolescents; MC malignant germ cell)
- endometrial sinus (yolk sac) tumor (malignant; usually seen in young children; MC tumor in MALE infants)
- choriocarcinoma (malignant)
- (note that germ cell tumors can arise in younger patients when compared to the other 2 types of ovarian tumors)
- (they are also MUCH more common in males)


What are the major types of sex cord-stromal cell ovarian tumors? Which are the most common?

- granulosa-theca cell tumor (malignant) (MC sex cord-stromal tumor)
- thecoma-fibromas (benign)
- Sertoli-Leydig cell tumor
- (these can produce sex hormones)


What are the differences between serous and mucinous cystadenomas and cystadenocarcinomas?

- these are all surface epithelial ovarian tumors (serous types being the most common ovarian tumors overall)
- these tumors are cystic and filled with either watery fluid (serous type) or mucous (mucinous type)
- cystadenoma (benign) usually presents in premenopausal women as a single simple cyst
- cystadenocarcinoma (malignant) usually presents in postmenopausal women as multiple complex cysts; serous type is frequently bilateral and also contains psammoma bodies; mucinous type is largely unilateral (if bilateral suspect Krukenberg tumor)


What are endometriomas associated with?

- endometrioma is a type of surface epithelial ovarian tumor
- they are associated with ectopic endometrial tissue in the ovary (from endometriosis)
- between 15-30% of patients will have concomitant endometrial carcinoma at the time of diagnosis
- most are malignant


What is a Brenner tumor? What type of epithelium characterizes this tumor?

- Brenner tumor is a benign type of surface epithelial ovarian tumor
- it is characterized by transitional-type epithelium (which is normally found in the urinary tract)


What is pseudomyxoma peritonei? What can cause it?

- pseudomyxoma peritonei is the intraperitoneal accumulation of excess mucous due to the implantation of mucinous tumor cells in the peritoneum
- most commonly arises from metastases from appendiceal carcinoma, but can also arise via mucinous cystadenocarcinoma


What is a mature cystic teratoma? What is it also known as? In which age group is this the most common ovarian tumor? What is struma ovarii and how do these patients present?

- a mature teratoma is a benign type of germ cell ovarian tumor; it is also known as a dermoid cyst
- it contains elements of all 3 germ layers; common components include hair, teeth, and sebum
- mature teratomas are the most common germ cell tumor; and are the most common ovarian tumor overall in patients aged 20-30
- struma ovarii: when the tumor contains hyperfunctional thyroid tissue and the patient presents with hyperthyroidism
- (90% of teratomas are benign/mature, 10% are malignant/immature)


What is an immature teratoma? What is the mean age of diagnosis?

- an immature teratoma is a malignant type of germ cell ovarian tumor (it is NOT the MC malignant germ cell, dysgerminoma is)
- it contains minimally differentiated cartilage, bone, muscle, nerve, and other tissues
- the mean age of diagnosis is 18 years
- (90% of teratomas are benign/mature, 10% are malignant)


What is the most common malignant germ cell tumor? In which age group is it most commonly seen in?

- dysgerminoma is the most common malignant germ cell tumor (most common benign is mature cystic teratoma)
- it is most commonly seen in adolescents


What is an endometrial sinus tumor? What is it also known as? Which patients is it more commonly seen in? What histological finding is seen in 50% of cases?

- endometrial sinus tumor AKA yolk sac tumor is a malignant type of germ cell ovarian tumors
- most commonly arises in young children
- yolk sac tumors are the most common tumor in MALE infants
- 50% have Schiller-Duval bodies, which resemble glomeruli


What is the major difference between placental choriocarcinoma and ovarian choriocarcinoma?

- placental (gestational) type responds very well to chemotherapy, but the ovarian type does NOT
- (ovarian choriocarcinoma is a type of germ cell ovarian tumor)


What is Meigs syndrome?

- Meigs syndrome is the triad of an ovarian fibroma (a type of benign sex cord-stromal cell ovarian tumor), ascites, and pleural effusion (hydrothorax)


What is the most common sex cord-stromal cell tumor? What sex hormone does it usually secrete? What do we see on histology?

- most common sex cord-stromal cell tumor is granulosa-theca cell tumor (malignant)
- these often produce estrogen: precocious puberty, heavy and irregular menses, postmenopausal bleeding
- histology reveals Call-Exner bodies (these resemble primordial follicles)


What are the risk factors for ovarian cancer? What is the overall 5-year prognosis for ovarian cancer?

- female sex, increased age, endometriosis, PCOS, nulliparity, early menarche, late menopause, genetic factors*, family history, history of breast cancer, obesity
- *genetic predispositions: BRCA1, BRCA2, Lynch (HNPCC), Turner syndrome, Peutz-Jeghers
- overall 5-year prognosis: 46% survival rate


How do patients with an ovarian tumor commonly present?

- most are asymptomatic until later stages
- presentations include: adnexal mass (especially in postmenopausal women, as these ovaries should be atrophic and non-palpable), abdominal distention, bowel obstruction, ascites, and pleural effusion; additionally, unprovoked DVTs may occur


How does ovarian cancer classically spread?

- ovarian cancer largely spreads via intraperitoneal dissemination (where the malignant cells implant anywhere in the peritoneal cavity)


Which tumor markers do we use for screening/monitoring of ovarian cancer?

- general marker is CA-125; it is used for monitoring treatment and recurrence, but is NOT recommended for screening
- AFP: tumor marker for yolk sac/endodermal sinus tumor
- beta-hCG and LDH: tumor markers for dysgerminoma
- (both yolk sac and dysgerminoma are malignant germ cell tumors)


Which ovarian cancers have characteristic morphologies? What are they?

- granulosa-theca cell tumor (MC sex cord stromal): Call-Exner bodies that resemble primordial follicles
- Brenner tumor (benign epithelial): transitional uroepithelium
- yolk sac/endodermal sinus tumor (germ cell): Schiller-Duval bodies that resemble glomeruli
- serous cystadenocarcinoma (MC malignant, epithelial): psammoma bodies