CH13 - Female Genital System and Gestational Pathology Flashcards
What is the vulva?
Anatomically includes the skin and mucosa of the female genitalia external to the hymen (labia majora, labia minora, mons pubis, and vestibule)
What is the vulva lined by?
squamous epithelium
What is a bartholin cyst?
Cystic dilation of the Bartholin gland
What are the bartholin glands?
One Bartholin gland is present on each side of the vaginal canal and produces mucus-like fluid that drains via ducts into the lower vestibule.
Bartholin cyst arises due to what?
inflammation and obstruction of gland
In whom does bartholin cyst usually occur?
in women of reproductive age
How does bartholin cyst present?
as a unilateral, painful cystic lesion at the lower vestibule adjacent to the vaginal canal
What is condyloma?
Warty neoplasm of vulvar skin, often large
What is condyloma most commonly due to?
HPV types 6 or 11 (condyloma acuminatum) secondary syphilis (condyloma latum) is a less common cause. Both are sexually transmitted.
Histologically, how are HPV-associated condylomas characterized?
by koilocytes
What is the relationship between condylomas and carcinoma?
They rarely progress to carcinoma (6 and 11 are low-risk HPV types)
How is lichen sclerosis characterized?
by thinning of the epidermis and fibrosis (sclerosis) of the dermis
How does lichen sclerosis present?
as a white patch (leukoplakia) with parchment-1ike vulvar skin
What is lichen sclerosis most commonly seen in?
postmenopausal women
What is a possible etiology for lichen sclerosis?
Possibly autoimmune etiology
Is lichen sclerosis associated with carcinoma?
Benign, but associated with a slightly increased risk for squamous cell carcinoma
What is lichen simplex chronicus?
Characterized by hyperplasia of the vulvar squamous epithelium
How does lichen simplex chronicus present?
as leukoplakia with thick, leathery vulvar skin
What is lichen simplex chronicus associated with?
chronic irritation and scratching
Is lichen simplex chronicus associated with carcinoma?
Benign; no increased risk of squamous cell carcinoma
What is vulvar carcinoma?
Carcinoma arising from squamous epithelium lining the vulva
What is the frequency of vulvar carcinoma?
Relatively rare, accounting for only a small percentage of female genital cancers
How does vulvar carcinoma present?
as leukoplakia
In vulvar carcinoma what may be required and why?
biopsy to distinguish carcinoma from other causes of leukoplakia
What is the etiology for vulvar carcinoma?
may be HPV related or non-HPV related.
What is HPV-related vulvar carcinoma due to?
high-risk HPV types 16 and 18.
What are the risk factors for HPV-related vulvar carcinoma related to?
HPV exposure and include multiple partners and early first age of intercourse; generally occurs in women of reproductive age
From where does HPV related vulvar carcinoma arise?
It arises from vulvar intraepithelial neoplasia (VIN), a dysplastic precursor lesion characterized by koilocytic change, disordered cellular maturation, nuclear atypia, and increased mitotic activity
When does non-HPV related vulvar carcinoma arise?
most often, from long-standing lichen sclerosis,
What happens in non-HPV related vulvar carcinoma?
Chronic inflammation and irritation eventually lead to carcinoma
In whom is non-HPV related vulvar carcinoma generally seen?
in elderly women (average age is > 70 years)
How is extramammary paget disease characterized?
by malignant epithelial cells in the epidermis of the vulva
How does extramammary paget disease present?
as erythematous, pruritic, ulcerated vulvar skin
What does extramammary paget disease represent?
carcinoma in situ, usually with no underlying carcinoma
What is Paget disease of the nipple characterized by?
malignant epithelial cells in the epidermis of the nipple, but it is almost always associated with an underlying carcinoma.
What must paget disease be distinguished from?
melanoma, which rarely can occur on the vulva
How is pagets disease distinguished from melanoma?
1) Paget cells are PAS+. keratin+, and S100-. 2) Melanoma is PAS-, keratin-, and S100+.
What is the vagina?
Canal leading to the cervix
What lines the vagina?
Mucosa is lined by non-keratinizing squamous epithelium
What is adenosis?
Focal persistence of columnar epithelium in the upper 1/3 of the vagina
What happens to the epithelium of the vagina during development?
squamous epithelium from the lower 2/3 of the vagina grows upward to replace the columnar epithelium lining of the upper 1/3 of the vagina
What is the lower 2/3 of the vagina derived from?
urogenital sinus
What is the upper 1/3 of the vagina derived from?
Mullerian ducts
In whom is there an increased incidence for adenosis?
in females who were exposed to diethylstilbestrol (DES) in utero
What is clear cell adenocarcinoma?
malignant proliferation of glands with clear cytoplasm
What is the frequency for clear cell adenocarcinoma?
Its rare, but feared, complication of DES-associated vaginal adenosis
What is a feared complication of DES-associated vaginal adenosis?
Clear cell adenocarcinoma
What led to the cessation of DES usage?
Discovery of the clear cell carcinoma complication (along with other DES-induced abnormalities of the gynecologic tract such as abnormal shape of the uterus)
What is embryonal rhabdomyosarcoma?
Malignant mesenchymal proliferation of immature skeletal muscle; rare
How does embryonal rhabdomyosarcoma present?
as bleeding and a grape-1ike mass protruding from the vagina or penis of a child (usually < 5 yrs of age)
What is embryonal rhabdomyosarcoma also known as?
sarcoma botryoides.
What is the characteristic cell for embryonal rhabdomyosarcoma?
Rhabdomyomablast
What does rhabdomyomablast exhibit?
cytoplasmic cross-striations and positive immunohistochemical staining for desmin and myogenin.
What is vaginal carcinoma?
Carcinoma arising from squamous epithelium lining the vaginal mucosa
What is vaginal carcinoma usually related to?
high-risk HPV
What is the precursor lesion for vaginal carcinoma?
it is vaginal intraepithelial neoplasia (VAIN)
What happens when vaginal carcinoma spreads to regional lymph nodes?
cancer from the lower 2/3 of vagina goes to inguinal nodes, and cancer from the upper 1/3 goes to regional iliac nodes.
What is the cervix?
Anatomically, comprises the neck of the uterus
What is the cervix divided into?
the exocervix (visible on vaginal exam) and endocervix
What is the exocervix lined by?
nonkeratinizing squamous epithelium
What is the endocervix lined by?
a single layer of columnar cells
What is the function between the exocervix and endocervix called?
the transformation zone
What is HPV?
Sexually transmitted DNA virus that infects the lower genital tract, especially the cervix in the transformation zone
What usually happens to HPV?
Infection is usually eradicated by acute inflammation;
In HPV what does persistent infection lead to?
an increased risk for cervical dysplasia (cervical intraepithelial neoplasia, CIN)
In HPV, what does the risk of CIN depend on?
the HPV type
How is the HPV type determined?
DNA sequencing.
What are the HPV types that are high risk for cervical intraepithelial dysplasia?
High-risk?HPV types 16, 18, 31, and 33
What are the low-risk types for cervical intraepithelial neoplasia?
HPV types 6 and 11
In high risk HPV, what is responsible for p53 destruction?
Production of E6
In high risk HPV, what is responsible for Rb destruction?
Production of E7
What do the high-risk HPV types produce that leads to the increased risk for CIN?
E6 and E7 proteins which result in increased destruction of p53 and Rb, respectively. Loss of these tumor suppressor proteins increases the risk for CIN.
What is cervical intraepithelial neoplasia characterized by?
koiloeytic change, disordered cellular maturation, nuclear atypia, and increased mitotic activity within the cervical epithelium.
What is cervical intraepithelial neoplasia divided into?
grades based on the extent of epithelial involvement by immature dysplastic cells(CIN I, II, III)
What does CIN I involve?
< 1/3 of the thickness of the epithelium
What does CIN II involve?
< 2/3 of the thickness of the epithelium,
What does CIN III involve?
slightly less than the entire thickness of the epithelium
What does carcinoma in situ (CIS) involve?
the entire thickness of the epithelium.
How does CIN classically progress?
in a stepwise fashion through CIN I, CIN II, CIN 111, and CIS to become invasive squamous cell carcinoma.
Can CIN progression be reversed?
Progression is not inevitable (CIN 3 often regresses)
What is the relationship between the grade of dysplasia and carcinoma?
the higher the grade of dysplasia, the more likely it is to progress to carcinoma and the less likely it is to regress to normal.
What happens in cervical carcinoma?
Invasive carcinoma that arises from the cervical epithelium
In whom is cervical carcinoma most commonly seen?
in middle-aged women (average age is 40-50 years)
How does cervical carcinoma present?
It presents as vaginal bleeding, especially postcoital bleeding, or cervical discharge
What are the key risk factors for cervical carcinoma?
high-risk HPV infection; secondary risk factors include smoking and immunodeficiency (e.g cervical carcinoma with HIV is a potential AIDS-defining illness).
What are the most common subtypes of cervical carcinoma?
squamous cell carcinoma (80% of cases) and adenocarcinoma (15% of cases).
What are both of the most common types of cervical carcinoma related to?
Both types are related to HPV infection.
What happens to advanced tumors of cervical carcinoma?
they often invade through the anterior uterine wall into the bladder, blocking the ureters.
What is a common cause of death in advanced cervical carcinoma?
Hydronephrosis with postrenal failure
What is the goal of screening in cervical carcinoma?
it is to catch dysplasia (CIN) before it develops into carcinoma
How long does progression from CIN to carcinoma take?
on average, takes 10-20 years.
When does cervical carcinoma screening begin?
at age 21 and is initially performed yearly.
What is the gold standard for screening for cervical carcinoma?
pap smear
How is a pap smear used to screen for cervical carcinoma?
Cells are scraped from the transformation zone using a brush and analyzed under a microscope.
In a pap smear, how are the cells scraped from the transformation zone characterized?
Dysplastic cells are classified as low grade (CIN I) or high grade (CIN II and III)
What is high-grade dysplasia characterized by?
cells with hyperchromatic (dark) nuclei and high nuclear to cytoplasmic ratios
What is the most successful screening test for cervical carcinoma developed to date?
Pap smear
What effect has pap smear had on cervical carcinoma?
It is responsible for a significant reduction in the morbidity and mortality of cervical carcinoma (cervical carcinoma went from being the most common to one of the least common types of gynecologic carcinoma in the US).
What is usually the case for women who develop invasive cervical carcinoma?
they usually have not undergone screening
What is an abnormal pap smear followed by?
confirmatory colposcopy (visualization of cervix with a magnifying glass) and biopsy.
What are the limitations of the Pap smear?
they include inadequate sampling of the transformation zone (false negative screening) and limited efficacy in screening for adenocarcinoma
How has Pap smear screening affected the incidence of adenocarcinoma?
The incidence has not decreased significantly.
What is an effective way of preventing HPV infections?
immunization
What is the quadrivalent vaccine?
it covers HPV types 6,11,16, and 18,
What antibodies protect against condylomas?
Those generated against types 6 and 11
What antibodies protect against CIN, VAIN, VIN and carcinoma?
Those generated against types 16 and 18
For immunization, how long does protection last?
for 5 years.
Are pap smears necessary for someone who has been vaccinated?
Yes, due to the limited number of HPV types covered by the vaccine
What is the endometrium?
it is the mucosal lining of the uterine cavity
What is the myometrium?
it is the smooth muscle wall underlying the endometrium
Does the endometrium respond to hormones?
It is hormonally sensitive
What drives growth of the endometrium?
it is estrogen driven (proliferative phase).
What drives preparation of the endometrium for implantation?
it is progesterone driven (secretory phase)
When does shedding of the endometrium occur?
with loss of progesterone support (menstrual phase)
What is asherman syndrome?
It is secondary amenorrhea
What is asherman syndrome due to?
loss of the basalis and scarring
What is the basalis of the endometrium?
basalis
What does asherman syndrome result from?
overaggressive dilation and curettage (D&C)
What is the anovulatory cycle?
Lack of ovulation
What does the anovulatory cycle result in?
an estrogen-driven proliferative phase without a subsequent progesterone driven secretory phase
What happens in the anovulatory cycle?
Proliferative glands break down and shed resulting in uterine bleeding.
The anovulatory cycle represents a common cause of what?
dysfunctional uterine bleeding, especially during menarche and menopause
What is menarche?
The first menstrual period
What is acute endometritis?
Bacterial infection of the endometrium
What is acute endometritis usually due to?
retained products of conception (e.g., after delivery or miscarriage); retained products act as a nidus for infection.
What does acute endometritis present as?
fever, abnormal uterine bleeding, and pelvic pain
What is chronic endometritis?
Chronic inflammation of the endometrium
What is chronic endometritis characterized by?
lymphocytes and plasma cells
What are necessary for the diagnosis of chronic endometritis?
Plasma cells since lymphocytes are normally found in the endometrium.
What are the causes of chronic endometritis?
they include retained products of conception, chronic pelvic inflammatory disease (e.g., Chlamydia), IUD, and TB.
What does chronic endometritis present as?
abnormal uterine bleeding, pain, and infertility
What is an endometrial polyp?
Hyperplastic protrusion of endometrium
What does an endometrial polyp present as?
abnormal uterine bleeding
An endometrial polyp can arise as a side effect of what and why?
tamoxifen, which has anti-estrogenic effects on the breast but weak pro-estrogenic effects on the endometrium
What is endometriosis?
Endometrial glands and stroma outside of the uterine endometrial lining
What is endometriosis most likely due to?
retrograde menstruation with implantation at an ectopic site
What does endometriosis present as?
dysmenorrhea (pain during menstruation) and pelvic pain; may cause infertility
What happens to the cycle in endometriosis?
cycles just like normal endometrium
In endometriosis what is the most common site of involvement and what does this form?
the ovary, which classically results in formation o f a chocolate cyst
What are the other sites of involvement in endometriosis besides the ovary?
1) uterine ligaments 2) pouch of Douglas 3) bladder wall 4) bowel serosa 5) fallopian tube mucosa
In endometriosis how do the implants classically appear?
as yellow-brown [gun-powder] nodules
In endometriosis what indicates the involvement of the uterine ligaments?
pelvic pain
In endometriosis what indicates the involvement of the pouch of Douglas?
pain with defecation
In endometriosis what indicates the involvement of the bladder wall?
pain with urination
In endometriosis what indicates the involvement of the bowel serosa?
abdominal pain and adhesions
What does the involvement of the fallopian tube mucosa in endometriosis result in?
scarring that increases the risk for ectopic tubal pregnancy
For the involvement of the fallopian tube mucosa in endometriosis, how does the implants classically appear?
as yellow-brown gun-powder nodules
What is adenomyosis?
It is the involvement of the uterine myometrium in endometriosis
What does endometriosis increase the risk of?
there is an increased risk for carcinoma at the site of endometriosis, especially in the ovary.
What is endometrial hyperplasia?
Hyperplasia of the endometrial glands relative to stroma
What does endometrial hyperplasia occur as a consequence of?
unopposed estrogen (e.g., obesity, polycystic ovary syndrome, and estrogen replacement)
How does endometrial hyperplasia classically present?
as postmenopausal uterine bleeding
How is endometrial hyperplasia classified histologically?
It?s based on architectural growth pattern (simple or complex) and the presence or absence of cellular atypia
For endometrial hyperplasia, what is the most important predictor for progression to carcinoma (major complication)?
It is the presence of cellular atypia
What are the odds that endometrial hyperplasia will pregress to cancer?
simple hyperplasia with atypia often progresses to cancer (30%) whereas, complex hyperplasia without atypia rarely does (<5%)
What is endometrial carcinoma?
Malignant proliferation of endometrial glands
What is the most common invasive carcinoma of the female genital tract?
Endometrial carcinoma
How does endometrial carcinoma present?
as postmenopausal bleeding
How does endometrial carcinoma arise?
via two distinct pathways: hyperplasia and sporadic
What percentage of endometrial carcinoma cases arise from the hyperplasia pathway?
75% of carcinoma cases arise from endometrial hyperplasia
What are the risk factors for endometrial carcinoma?
They are related to estrogen exposure and include early menarche/late menopause, nulliparity, infertility with anovulatory cycles, and obesity.
What is the average age of presentation for endometrial carcinoma from endometrial hyperplasia?
it is 60 years.
What is the histology for endometrial carcinoma?
it is endometrioid (i.e., normal endometrium-like)
What percentage of endometrial carcinoma cases arise from the sporadic pathway?
25% of carcinoma cases arise from the sporadic pathway with an atrophic endometrium with no evident precursor lesion.
What is the average age at presentation for endometrial carcinoma from the sporadic pathway?
70 years.
What is the histology for endometrial carcinoma from the sporadic pathway?
It is usually serous and is characterized by papillary structures with psammoma body formation;
For endometrial carcinoma from the sporadic pathway what mutation is common?
p53 and the tumor exhibits aggressive behavior
What is leiomyoma?
(fibroids) Benign neoplastic proliferation of smooth muscle arising from myometrium;
What is the most common tumor in females?
leiomyoma
What is leiomyoma related to?
estrogen exposure
What is leiomyoma common in?
premenopausal women
Is leiomyoma occur as single or multiple fibroids?
Often multiple
What happens to leiomyoma during pregnancy?
They enlage
When does leiomyoma shrink?
after menopause
What does the gross exam of leiomyoma show?
multiple, well-defined, white, whorled masses that may distort the uterus and impinge on pelvic structures
What are the symptoms of leiomyoma?
Usually asymptomatic; when present, symptoms include abnormal uterine bleeding, infertility, and a pelvic mass
What is leiomyosarcoma?
Malignant proliferation of smooth muscle arising from the myometrium
How does leiomyosarcoma arise?
de novo; leiomyosarcomas do not arise from leiomyomas
Are leiomyosarcomas related to leiomyomas?
leiomyosarcomas do not arise from leiomyomas
In whom is leiomyosarcoma usually seen?
in postmenopausal women
What does the gross exam often show for leiomyosarcoma?
a single lesion with areas of necrosis and hemorrhage;
What are the histological features for leiomyomasarcoma?
they include necrosis, mitotic activity, and cellular atypia
What is the ovary?
The functional unit of the ovary is the follicle.
What does a follicle consists of?
an oocyte surrounded by granulosa and theca cells
On what does LH act?
on theca cells to induce androgen production
What does FSH do?
stimulates granulosa cells to convert androgen to estradiol (drives the proliferative phase of the endometrial cycle)
What does the estradiol surge induce?
an LH surge, which leads to ovulation (marking the beginning of the secretory phase of the endometrial cycle)
What happens after ovulation?
the residual follicle becomes a corpus luteum,
What does the corpus luteum do?
it primarily secretes progesterone (drives the secretory phase which prepares the endometrium for a possible pregnancy)
What is a hemorrhagic corpus luteal cyst?
Hemorrhage into a corpus luteum can result in a hemorrhagic corpus luteal cyst, especially during early pregnancy.
What does degeneration of follicles result in?
follicular cysts.
What is the clinical significance of follicular cysts?
Small numbers of follicular cysts are common in women and have no clinical significance
What is polycystic ovarian disease (PCOD)?
Multiple ovarian follicular cysts due to hormone imbalance
What is the frequency of PCOD?
Affects roughly 5% of women of reproductive age
What is PCOD characterized by?
increased LH and low FSH (LH:FSH > 2)
What does increased LH induce?
excess androgen production (from theca cells) resulting in hirsutism (excess hair in a male distribution).
Within PCOD what happens to androgen?
it is converted to estrone in adipose tissue
What does estrone feedback result in PCOD?
it decreases FSH resulting in cystic degeneration of follicles.
What do the high levels of circulating estrone do in PCOD?
increase risk for endometrial carcinoma
What is the classic presentation for PCOD?
is an obese young woman with infertility, oligomenorrhea, and hirsutism;
What may happen to some patients with PCOD?
they have insulin resistance and may develop type 2 diabetes mellitus 10-15 years later.
What are the cell types of the ovary?
Ovary is composed of three cell types: surface epithelium, germ cells, and sex cordstroma.
From which of these cell types can a tumor arise?
from any of these cell types or from metastases
What is the most common type of ovarian tumor?
Occurs in 70% of ovarian tumor cases
What are the surface epithelial cells derived from?
coelomic epithelium that lines the ovary;
What does the coelomic epithelium embryologically produce?
the epithelial lining of the fallopian tube (serous cells), endometrium, and endocervix (mucinous cells).
What are the two most common subtypes of surface epithelial tumors?
serous and mucinous; both are usually cystic.
What are serous tumors filled with?
They are full of watery fluid.
What are mucinous tumors filled with?
they are full of mucus-like fluid.
Are mucinous and serous tumors malignant?
they can be benign, borderline, or malignant
What are benign ovarian surface epithelial tumors?
cyst adenomas and are composed of a single cyst with a simple, flat lining
In whom does benign ovarian surface epithelial tumors most commonly arise?
in premenopausal women (30-40 years old)
What are malignant ovarian surface epithelial tumors?
They are cystadenocarcinomas and are composed of complex cysts with a thick, shaggy lining;
In whom do malignant ovarian surface epithelial tumors most commonly arise?
in postmenopausal women (60-70 years old)
What are boderline ovarian surface epithelial tumors?
Borderline tumors have features in between benign and malignant tumors, better prognosis than clearly malignant tumors, but still carry metastatic potential
Carriers of what mutation have an increased risk for serous carcinoma of the ovary and fallopian tube?
BRCA1
What do BRCA1 carriers often elect to have?
a prophylactic salpingo-oophorectomy (along with prophylactic mastectomy due to the increased risk for breast cancer)
What are some less common subtypes of [ovarian] surface epithelial tumors?
endometrioid and Brenner tumor.
What are endometrioid tumors composed of?
endometrial-like glands and are usually malignant
What may endometrioid tumors arise from?
endometriosis
What are 15% of endometrioid carcinomas of the ovary associated with?
An independent endometrial carcinoma (endometrioid type)
What are brenner tumors composed of?
bladder-like epithelium and are usually benign.
How do ovarian surface epithelial tumors clinically present?
late with vague abdominal symptoms (pain and fullness) or signs of compression (urinary frequency).
What is the prognosis for surface epithelial carcinoma?
Generally poor, worst prognosis of female genital tract cancers
Describe the spread of ovarial epithelial carcinomas?
they tend to spread locally, especially to the peritoneum.
What is CA-125?
it is a useful serum marker to monitor treatment response and screen for recurrence.
What are germ cell tumors?
They are the 2nd most common type of ovarian tumor (15% of cases)
In whom do germ cell tumors usually occur?
in women of reproductive age
What do germ cell tumor subtypes mimic?
tissues normally produced by germ cells.
What is the germ cell tumor subtype that mimics fetal tissue?
cystic teratoma and embryonal carcinoma
What is the germ cell tumor subtype that mimics oocytes?
dysgerminoma
What is the germ cell tumor subtype that mimics a yolk sac?
endodermal sinus tumor
What is the germ cell tumor subtype that mimic placental tissue?
choriocarcinoma
What is a cystic teratoma?
Cystic tumor composed of fetal tissue derived from two or three embryologic layers (e.g, skin, hair, bone, cartilage, gut, and thyroid)
What is the most common germ cell tumor in females (unilateral or bilateral)?
Cystic teratoma; bilateral in 10% of cases
Is a cystic teratoma benign or malignant?
Benign but presence of immature tissue (usually neural) or somatic malignancy (usually squamous cell carcinoma of skin) indicates malignant potential.
What would indicate malignant potential for a cystic teratoma?
presence of immature tissue (usually neural) or somatic malignancy (usually squamous cell carcinoma of skin) indicates malignant potential
What is struma ovarii?
it is a teratoma composed primarily of thyroid tissue.
What is dysgerminoma?
Tumor composed of large cells with clear cytoplasm and central nuclei;
What is the most common malignant germ cell tumor?
dysgerminoma
What is the testicular counterpart to dysgerminoma?
it is called seminoma, which is a relatively common germ cell tumor in males.
What is the prognosis for seminoma?
Good prognosis; responds to radiotherapy
In dysgerminoma, what levels may be elevated?
Serum LDH may be elevated
What is the endodermal sinus tumor?
It is a malignant tumor that mimics the yolk sac;
What is the most common germ cell tumor in children?
Endodermal sinus tumor
In an endodermal sinus tumor what levels are often elevated?
Serum AFP is often elevated.
What are classically seen on histology for endodermal sinus tumor?
Schiller-Duval bodies (glomerulus-like structures)
What is choriocarcinoma?
malignant tumor composed of trophoblasts and syncytlotrophoblasts;
What does choriocarcinoma mimic?
placental tissue, but villi are absent
Describe choriocarcinoma?
Small, hemorrhagic tumor with early hematogenous spread
High levels of what are characteristic of choriocarcinoma?
beta-hCG is characteristic (produced by syncytiotrophoblasts); may lead to thecal cysts in the ovary
How does choriocarcinoma respond to chemotherapy?
Poor response to chemotherapy
What is embryonal carcinoma?
malignant tumor composed of large primitive cells
Describe emryonal carcinoma?
Aggressive with early metastasis
What are sex cord stromal tumors?
Tumors that resemble sex cord-stromal tissues of the ovary
What is a granulosa-theca cell tumor?
Neoplastic proliferation of granulosa and theca cells
What does granulosa-theca cell tumors often produce?
estrogen; presents with signs of estrogen excess
What happens if a granulosa-theca cell tumor occurs prior to puberty?
precocious puberty
What happens if a granulosa-theca cell tumor occurs during reproductive age?
menorrhagia or metrorrhagia
What is the most common setting for granulosa-theca cell tumors?
Postmenopause
What happens with granulosa-theca cell tumors occurring in postmenopause?
endometrial hyperplasia with postmenopausal uterine bleeding
Are granulosa-theca cell tumors malignant or benign?
Malignant, but minimal risk for metastasis
What are sertoli-leydig cell tumors?
They are composed of Sertoli cells that form tubules and Leydig cells (between tubules) with characteristic Reinke crystals
What might sertoli-ledig cells produce?
May produce androgen; associated with hirsutism and virilization
What is fibroma in regard to sex cord stromal tumors?
Benign tumor of fibroblasts
What is fibroma in regard to sex cord stromal tumors associated with?
pleural effusions and ascites (Meigs syndrome); syndrome resolves with removal of tumor
What are examples of metastasis for ovarian surface epithelial tumors?
Krukenberg tumor and pseudomyxoma peritonei
What is a Krukenberg tumor?
it is a metastatic mucinous tumor that involves both ovaries
What is a Krukenberg tumor most commonly due to?
metastatic gastric carcinoma (diffuse type)
What helps distinguish metastases from primary mucinous carcinoma of the ovary?
Metastases (Krukenberg tumor) is bilateral as opposed to primary mucinous carcinoma which is unilateral.
What is pseudomyxoma peritonei?
It is massive amounts of mucus in the peritoneum.
What is pseudomyxoma peritonei due to?
a mucinous tumor of the appendix, usually with metastasis to the ovary
What is ectopic pregnancy?
Implantation of fertilized ovum at a site other than the uterine wall;
What is the most common site for ectopic pregnancy?
the lumen of the fallopian tube
What are the key risk factors for ectopic pregnancy?
scarring (e.g., secondary to pelvic inflammatory disease or endometriosis).
What is the classic presentation for ectopic pregnancy?
lower quadrant abdominal pain a few weeks after a missed period.
What is the treatment for ectopic pregnancy?
Surgical emergency; major complications are bleeding into fallopian tube (hematosalpinx) and rupture
What is spontaneous abortion?
Miscarriage of fetus occurring before 20 weeks gestation (usually during first trimester)
What is the frequency of spontaneous abortion?
Its common and occurs in up to 1/4 of recognizable pregnancies
How does spontaneous abortion present?
Presents as vaginal bleeding, cramp-like pain, and passage of fetal tissues
What is spontaneous abortion most often due to?
chromosomal anomalies (especially trisomy 16);
Aside from chromosomal anomalies what are some other causes for spontaneous abortion?
hypercoagulablc states (e.g antiphospholipid syndrome), congenital infection, and exposure to teratogens (especially during the first 2 weeks of embryogenesis).
What does the effect of teratogens generally depend on?
the dose, agent, and time of exposure
Teratogen exposure in the first two weeks of gestation results in what?
spontaneous abortion
Teratogen exposure in weeks 3-8 results in what?
risk of organ malformation
Tereatogen exposure in months 3-9 result in what?
risk of organ hypoplasia
What is placenta previa?
Implantation of the placenta in the lower uterine segment; placenta overlies cervical os (opening).
How does placenta previa present?
as third-trimester bleeding
What does placenta previa often require?
delivery of fetus by caesarian section
What is the decidua?
It is the term for the uterine lining during pregnancy which forms the maternal part of the placenta
What is placental abruption?
Separation of placenta from the decidua prior to delivery of the fetus
Placental abruption is a common cause of what?
still birth
What does placental abruption present with?
third-trimester bleeding and fetal insufficiency
What is placenta accreta?
Improper implantation of placenta into the myometrium with little or no intervening decidua
How does the placenta accreta present?
with difficult delivery of the placenta and postpartum bleeding
What are some common teratogens?
Alcohol, cocaine, thalidomide, cigarette smoke, isotretinoin, tetracycline, warfarin, plienytoin
What is the teratogenic effect of alcohol?
Most common cause of mental retardation; also leads to facial abnormalities and microcephaly
What is the teratogenic effect of cocaine?
Intrauterine growth retardation and placental abruption
What is the teratogenic effect of thalidomide?
Limb defects
What is the teratogenic effect of cigarette smoke?
Intrauterine growth retardation
What is the teratogenic effect of isotretinoin?
Spontaneous abortion, hearing and visual impairment
What is the teratogenic effect of tetracycline?
Discolored teeth
What is the teratogenic effect of warfarin?
Fetal bleeding
What is the teratogenic effect of plienytoin?
Digit hypoplasia and cleft lip / palate
What does placenta acrreta often require?
hysterectomy
What is preeclampsia?
Pregnancy-induced hypertension, proteinuria, and edema, usually arising in the third trimester
How often is preeclampsia seen?
Its seen in approximately 5% of pregnancies
In preeclampsia, what is the severity of the hypertension?
it may be severe, leading to headaches and visual abnormalities
What is preeclampsia due to and how does it resolve?
abnormality of the maternal-fetal vascular interface in the placenta; resolves with delivery
What is eclampsia?
it is preeclampsia with seizures
What is HELLP?
it is preeclampsia with thrombotic microangiopathy involving the liver
What is HELLP characterized by?
Hemolysis, Elevated Liver enzymes, and Low Platelets
What do you typically do with both eclampsia and HELLP?
It usually warrants immediate delivery
What is sudden infant death syndrome?
Death of a healthy infant (1 month to 1 year old) without obvious cause
When does sudden infant death usually occur?
Infants usually expire during sleep
What are the risk factors for sudden infant death syndrome?
include sleeping on stomach, exposure to cigarette smoke, and prematurity
What is hydatidiform mole?
abnormal conception characterized by swollen and edematous villi with proliferation of trophoblasts
What is observed with hydatidiform mole?
uterus expands as if a normal pregnancy is present, but the uterus is much larger and beta-hCG much higher than expected for date of gestation.
How does hydatidiform mole classically present?
in the second trimester as passage of grape-like masses through the vaginal canal
How are hydatidiform moles diagnosed?
With prenatal care, moles are diagnosed by routine ultrasound in the early first trimester.
What is seen on ultrasound for hydatidiform mole?
Fetal heart sounds are absent, and a snowstorm appearance is classically seen on ultrasound
How is the hydatidiform mole classified?
as complete or partial
What is the treatment for hydatidiform moles?
It is dilatation and curettage.
What is dilation and curettage?
D&C - Dilation (widening / opening) of the cervix and surgical removal of part of the lining of the uterus
For hydatidiform moles what happens after the D&C?
subsequent beta-hCG monitoring is important to ensure adequate mole removal and to screen for the development of choriocarcinoma
What might result as a complication of gestation?
Choriocarcinoma may arise as a complication of gestation (spontaneous abortion, normal pregnancy, or hydatidiform mole) or as a spontaneous germ cell tumor
How does choriocarcinomas that arise from the gestational pathway respond to chemotherapy?
It responds well to chemotherapy, those that arise from the germ cell pathway do not.
What are the genetics for the partial mole?
normal ovum fertilized by two sperm (or one sperm that duplicates chromosomes): 69 chromosomes
What are the genetics for the complete mole?
Empty ovum fertilized by two sperm (or one sperm that duplicates chromosomes); 46 chromosomes
Is there fetal tissue in the partial mole?
yes
Is there fetal tissue in the complete mole?
No its absent
What is villous edema in the partial mole?
Some villi are hydropic and some are normal
What is villous edema for the complete mole?
Most villi are hydropic
What is trophoblastic proliferation for partial moles?
Focal proliferation present around hydropic villi
What is trophoblastic proliferation for complete moles?
Diffuse circumferential proliferation around hydropic villi
What is the risk for choriocarcinoma in the partial mole?
Minimal
What is the risk for choriocarcinoma in the complete mole?
2-3%
