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Flashcards in The female genital tract Deck (105):

What diseases are specific to the vulva?

1) bartholin cyst
2) lichen sclerosus
3) squamous cell hyperplasia or lichen simplex chronicus


What does lichen sclerosus look like?

smooth white plaques that in time coalesce


What happens in lichen sclerosus when the entire vulva is affected?

the labia becomes atrophic and stiffened and the vaginal orifice is constricted


What group of women does lichen sclerosus more commonly occur in?

post menopausal women


What does lichen sclerosus look like on histology?

1) thinned epidermis with disappearance of rete pegs
2) dermal fibrosis with a scan perivascular mononuclear infiltrate


Is lichen sclerosus pre malignant?



What is the histology seen with squamous cell hyperplasia or lichen simplex chronicus?

1) marked epithelial thickening
2) expansion of the stratum granulosm
3) significant surface hyperkeratosis


Does squamous cell hyperplase or lichen simplex chronicus increase your predisposition to cancer?



What is a benign tumor of the vulva?

condyloma acuminatum


What are the malignant tumors of the vulva?

1) vulvar intraepithelial neoplasia and vulvar carcinoma
2) extra mammary paget disease
3) malignant melanoma


What is the cause of condyloma cuminatum?

1) sexually transmitted
2) HPV 6 and 11


What is the gross apperance of condyloma cuminatum?

1) verrocous
2) multifocal
3) looks like cauliflower and can be mistaken for laryngeal papiloma


What is the most common type of vulvar carcinoma?

squamous carcinoma


What squamous cell carcinoma is most related to high risk HPV infections?

basolid and warty carcinomas
VIN -----> SCC


What squamous cell carcinoma is not related to HPV?

keratinizing SCC


What cells do HPV infect?

immature basal cells


What cells does HPV replicate in?

1) squamous cells
2) results in a cytopathic effect "kolicytic atypia"


What is the progression of vulvar intraepithelial neoplasia (VIN) cause basaloid or warty carcinomas?

1) HPV infection
2) low grade dysplasia
3) moderate dysplasia
4) severe dysplasia (carcinoma in situ)
5) invasive squamous carcinoma


What causes non-HPV related VIN and what does it lead to?

probably caused by chronic irritation in the elderly. leads to keratinizing squamous carcinomas


What does extramammary pagets disease look like clinically?

1) pruritic
2) red
3) crusted
4) sharply demarcated
5) on the labia majora


What does extra mammary pagets disease look like on histology?

large tumor cells with a clear halo


What do patients with extra mammary pagets disease also have?

all have an underlying breast carcinoma


What product caused benign vaginal lesions in some babies?

DES - diethylstilbestrol


What are the tumors of the vagina?

1) vaginal intraepithelial neoplasia
2) squamous cell carcinoma
3) embryonal rhabdomyosarcoma


What is the most common malignant neoplasm of the vagina?

metastasis from the cervix


What is embryonal rhabdomyosarcoma also called and who does it occur in?

1) sarcoma botryoides
2) infants less than 5


What does embryonal rhabdomyosarcoma look like clinically?

1) polyploid, round bulk mass
2) protrudes out of vagina
3) bag of grapes


What does embryonal Rhabdomyosarcoma look like on histology?

1) small cells with oval nuclei
2) small protrusions of cytoplasm
3) striations within the cytoplasm


What is the more recent terminology for cervical intraepithelial neoplasia?

1) CIN I - mild
2) CIN II - moderate
3) CIN III - severe


What terminology are clinical decisions based on for CIN?

1) CIN I - low grade
2) CIN II and III - high grade


What are the characteristics of an SIL or squamous intraepithelial lesion?

1) nuclear atypia
2) enlargement
3) hyperchromasia (dark)
4) variation in nuclear size


What is koliocytosis?

1) involves mature squamous cells
2) bi nucleation and cytoplasmic halos


What are the differences in atypia found in the different stages of CIN?

1) CIN I - atypia in lower 1/3 of epithelium
2) CIN II - atypia 2/3 of epithelium
3) CIN III - full thickness atypia (Carcinoma in situ)


What is p16?

1) a gene that encodes for cyclin kinase inhibitor


What does cyclin kinase inhibitor do?

it is a cell cycle regulatory protein which inhibits the cell cycle


What is wrong with p16 in cells with HPV?

There is an over expression of p16 (cyclin kinase inhibitor)


What do ALL high grade squamous intraepithelial lesions have?

HPV 16 and HPV 18 infections


What are the recommendations for pap screening?

1) first pap - 21 y/o or within 3 years of onset of sexual activity and after on an anual basis
2) after age 30 - with 3 normal results - screened every 2-3 years


What are the two types of cervical carcinoma and how common are they respectively?

1) squamous cell carcinoma - 80%
2) adenocarcinoma - 15%


What is an immediate precursor to squamous cell carcinoma?



What is endometriosis?

presence of endometrial tissue outside the uterus


What are the most common sites of endometriosis?

1) ovaries
2) uterine ligaments
3) rectovaginal septum
4) cul de sac
5) pelvic peritoneum


What is the clinical presentation of endometriosis?

1) infertility
2) dysmenorrhea (painful menses)
3) pelvic pain
4) women of reproductive age


What can endometriosis give rise to?



What are the two theories on how endometriosis occurs?

1) metastatic theory
2) metaplastic theory


What is the main thought behind metastatic theory and what is some proof behind it?

1) retrograde menstration
2) proof - shed of endometrium in peritoneal fluid, high incidence in women with retrograde flow


What is the main thought behind metaplastic theory and what is some proof behind it?

1) endometrium could arise from epithelial lining of pelvic peritoneum or mullerian remnant tissue
2) proof - endometriosis in men and females with turner syndrome


What two things are present on histological diagnosis of endometriosis?

1) endometrial glands
2) endometrial stroma


What causes endometrial hyperplasia?

1) due to prolonged estrogen stimulation
2) increased number of glands


What are the 4 categories of endometrial hyperplasia?

1) simple without atypia
2) simple with atypia
3) complex wtithout atypia
4) complex with atypia


What category of endometrial hyperplasia is most likely to progress to carcinoma?

complex with atypia: 23-48%


Who is most likely to get endometrial carcinoma?

1) post menopausal women (55-65)
2) post menopausal bleeding


What have mutations of PTEN tumor supressor gene been associated with?

1) endometrioid carcinomas
2) endometrial hyperplasia


What is the most common type of endometrial carcinoma?

endometrioid adenocarcinoma


What are the associations with endometriod type carcinoma?

1) estrogen
2) age 55-65
3) hyperplasia


What are the associations with non-endometrioid carcinoma?

1) older females: 65-75
2) p53 mutations
3) poor prognosis
4) not related to estrogen
5) includes serous, clear cell and mixed mullerian tumors


What are the benign and malignant tumors of the myometrium?

1) benign - leiomyomas
2) malignant - leiomyosarcomas


What is a leiomyoma?

1) most common benign tumor in women
2) originates from smooth muscle
3) most of the women are child bearing age


What is the gross appearance of a leiomyoma?

1) sharply circumscribed
2) round
3) firm
4) gray-white


What is the histological apperance of a leiomyoma?

1) bundles of smooth muscle cells
2) uniform in size and shape
3) rare mitotic figures


Who is most susceptible to a leiomyosarcoma?

1) equally common before and after menopause
2) peak age 40-60


What is the prognosis of leiomyosarcoma?

1) tendency to metastasize
2) 5 year survival of 40%


What is the gross appearance of a leiomyosarcoma?

1) bulky, fleshy masses invading the uterine wall
2) polypoid masses that project into the uterine lumen


What is the histology of a leiomyosarcoma?

1) bundles of smooth muscle cells with pleiomorphic and abundant mitotic figures
2) enlarged and irregular nucleus


What are the non-neoplastic/functional cysts of the ovaries?

1) follicle and luteal cysts
2) polycystic ovaries and stromal hyperthecosis


What are tumors of the ovaries?

1) surface epithelium tumors
2) sex cord stromal tumors
3) germ cell tumors
4) metastatic tumors


How common are cystic follices in the ovaries?

so common they are basically normal


What are cystic ovarian follicles?

1) originate in unruptured graafian follicles or in follicles that have ruptured and immediately sealed
2) usually multiple
2) up to 2cm in size


What is polycystic ovaries and stromal hyperthecosis?

1) numerous cystic follicles often associated with oligomenorrhea
2) high levels of LH (anovulation, hyperandrogenism)
3) persistent amenorrhea, obesity, hirsutism and rarely virilism


What is hirsutism?

presence of excessive terminal hair in androgen-dependent areas of a womans body


Why is ovarian cancer so bad?

only 3% of all female cancer BUT detected when they have metastasized and account for a very high number of deaths


What are 3 types of tumors of the surface epithelium of the ovary?

1) serous tumor
2) mucinous tumor
3) endometroid tumor


What are risk factors for serous epithelial tumor of the ovary?

1) nulliparity
2) family history
3) mutations - BRACA1 and BRACA2


What mutations are associated with malignant serous carcinoma of the ovary?

1) low grade - KRAS, BRAF
2) high grade (de novo) - p53, BRACA 1/2


What are risk factors for mucinous epithelial ovarian tumors?

1) smoking
2) KRAS mutations


What is the histology of mucinous epithelial ovarian tumors?

tall columnar epithelial cells with apical cilia


What mutations are associated with endometriod tumors of the ovary?

1) PTEN tumor suppressor gene
2) KRAS and b-catenin oncogenes
3) micro satellite instability


What is present in the serum of most patients with serous and endometrioid carcinomas and why is it not good for screening?

1) CA-125
2) can be elevated with non-specific irritation


What is the most common germ cell tumor?



What are the contents of a teratoma?

1) wall of cyst - squamous epithelium with hair shafts and sebaceous glands
2) cyst contents - hair, bone, cartilage, brain


What is dyserminoma and whatis its prognosis?

1) ovarian counterpart of seminoma of testis
2) large polyhedral tumor cells with central round nucleus, with surrounding lymphocytes
3) responsive to chemo - 80% survival


What is an endodermal sinus (yolk sac) tumor?

1) rare
2) rich in alpha fetoprotein and alpha 1 antitrypsin
3) schiller-duval body - central blood vessel surrounded by germ cells


What is a chriocarcinoma?

1) very aggressive but rare
2) exist mostly in combo with other germ cell tumors
3) identical to placental choriocarcinoma
4) high blood levels of hCG


What happens with a granulosa theca cell tumors in young females vs adult females?

1) young females - precocious puberty
2) adult females - endometrial hyperplasia, cystic disease of the breast


What are the characteristics of a granulosa theca cell tumors?

1) elevated inhibin serum levels
2) usually unilateral
3) yellow cut surface - lipids
4) call-exner body - gland like structure filled with central acidophilic


What are fibromas, thecomas and fibrothecomas?

1) fibromas - tumors composed of fibroblast
2) thecomas - plump spindle cells with lipids


What are the characteristics of fibroma, thecoma and fibrothecomas?

1) unilateral (90%)
2) majority are hormonally inactive and benign
3) pelvic mass + ascites + hydrothorax = meig's syndrome


What is a sertoli-leyding cell tumors (androblastomas)?

1) produce masculinization due to androgen production - atrophy of breasts, amenorrhea, sterility and loss of hair
2) progress to virilization (hirsutism) - male distribution of hair, hypertrophy of clitoris and voice changes


What does a sertoli-leyding cell tumor look like grossly?

solid, golden yellow (lipid)


What does a sertoli-leyding cell tumor look like on histology?

tubules composed of sertoli cells or leydig cells


What are the gestational trophoblastic diseases?

1) hydatidiform mole
2) invasive mole
3) tumors
a) choriocarcinoma
b) placental site trophoblastic tumor


What is a hydatidiform mole?

1) cystic swelling of the chorionic villi


What age groups are hydatidiform moles most common?

1) teens
2) 40-50


What is a complete hydatidiform mole?

1) fertilization of an egg that has lost its chromosomes - genetic material is parenterally derived
2) 46 XX (90%)
3) 10% fertilization of an egg by two sperm
4) not usually fetal parts
5) small risk of choriocarcinoma


What is a partial hydatidiform mole?

1) fertilization of an egg with two sperm
2) triploid karyotype (69 XXY)
3) tetrapolyd (92, XXXY)
4) more often fetal parts
5) no risk of choriocarcinoma


What does a complete mole show on histology?

1) show abnormalities that involve all or most of the villous tissue
2) enlarged chorionic villi and proliferation of trophoblast that involves the entire circumference of the villi


What does a partial mole show on histology?

1) abnormalities involve only a portion of the villi


What is p57KIP2?

a gene that is maternally transcribed but paternally imprinted and shows expression in maternal decidual tissue and cytotrophoblast and stromal cells when maternal genetic material is present


Is p57 expressed in hydatidiform moles?

1) complete mole = negative p57
2) partial mole = positive p57 (has maternally derived tissue)


What are the treatments for hydatidiform moles?

1) curettage
2) monitor serum concentrations of HCG (6 months to a year)


What is an invasive mole?

1) mole that penetrates or perforates uterine wall
2) persistent elevated serum HCG
3) treat with chemotherapy


What is a choriocarcinoma?

1) malignant neoplasm of trophoblastic cells derived from a previously normal or abnormal pregnancy


Where do choriocarcinomas arise from?

1) 50% - hydatidiform moles
2) 25% - previous abortions
3) 22% - normal pregnancy


What is seen on histology with choriocarcinomas?

1) does not contain chorionic villi - contains syncytiotrophoblast and cytotrophoblast
2) invades myometrium, has a rapid growth
3) metastasis to lung, vagina, brain, liver and kidney


What is the treatment for choriocarcinoma?

1) chemotherapy - 100% remission
2) high cure rate