Reproductive 2 Flashcards
Lichen sclerosus
Thinning of epidermis with fibrosis/sclerosis of dermis.
Porcelain-white plaques with a red or violet border.
Skin fragility with erosions can be observed.
Postmenopausal women. Benign, but risk for SCC.
Lichen simplex chronicus
Hyperplasia of vuIvar squamous epithelium.
Leathery, thick vulvar skin with enhanced skin markings due to chronic rubbing or scratching.
Benign, no risk of SCC.
Vulvar carcinoma - Presentation
Carcinoma from squamous epithelial lining of vulvar - Rare.
Leukoplakia - biopsy often required to distinguish carcinoma from other causes.
Extramammary Paget disease
intraepithelial adenocarcinoma.
Carcinoma in situ, low risk of underlying carcinoma (vs Paget disease of the breast, which is always associated with underlying carcinoma).
Presents with pruritus, erythema, crusting, ulcers
imperforate hymen
Failure of hymen central epithelial cells to degenerate at birth.
Accumulation of vaginal mucus at birth -> self-resolving bulge in introitus.
If untreated, leads to 1° amenorrhea, cyclic abdominal pain, hematocolpos (accumulation of menstrual blood in vagina - bulging and bluish hymenal membrane).
Sarcoma botryoides
Embryonal rhabdomyosarcoma variant.
Affects girls< 4 years old; spindle-shaped cells; desmin (+)
Presents with clear, grape-like, polypoid mass emerging from the vagina.
Vaginal tumors
Vaginal squamous cell carcinoma - Usually 2° to cervical SCC
Clear cell adenocarcinoma - Affects women who had exposure to DES in utero.
Dysplasia and carcinoma in situ of cervix - pathology and Sx.
begins at the basal layer of squamocolumnar junction (transformation zone) and extends outward.
ClN 1, ClN 2, or ClN 3
Koilocytes are pathognomonic of HPV infection. May progress slowly to invasive carcinoma if left untreated.
Typically asymptomatic or vaginal bleeding (often postcoital).
Dysplasia and carcinoma in situ of cervix - risk factors
Associated with HPV-16 and HPV-18, which produce both the:
E6 gene product (inhibits TP53)
E7 gene product (inhibits pRb)
Risk factors: D - DES exposure I - immunocompromise (eg, Hl\/, transplant) S - smoking C - coitarche (early) S - sexual partners - multiple (#1)
Primary ovarian insufficiency
Also known as premature ovarian failure - Premature atresia of ovarian follicles in women of reproductive age.
Idiopathic - associated with chromosomal abnormalities (especially in females< 30 years) -> Need karyotype screening.
Patients present with signs of menopause after puberty but before age 40.
Dec. estrogen, Inc LH and FSH
Most common causes of anovulation
OB/GYN:
Pregnancy
Polycystic ovarian syndrome
Premature ovarian failure.
Endocrine: P - hyperProlactinemia A - adrenal insufficiency T - thyroid disorders C - Cushing syndrome H - HPO axis abnormalities/immaturity
Caloric/ chromosomal:
A - athletics
C - chromosomal abnormalities (eg, Turner syndrome)
E - eating disorders/obesity
Functional hypothalamic amenorrhea
exercise-induced amenorrhea.
Severe caloric restriction, Inc energy expenditure, and/or stress -> functional disruption of pulsatile GnRH secretion -> dec. LH, FSH, estrogen.
Dec. Leptin (dec fat)
Inc. Cortisol
Polycystic ovarian syndrome - Pathophysiology
Stein-Leventhal syndrome
Hyperinsulinemia and/or insulin resistance hypothesized to alter hypothalamic hormonal feedback response
- > Inc LH: FSH
- > androgens (eg, testosterone - theca interna cells)
- > Estrogen (from fat)
- > Dec rate of follicular maturation
- > unruptured follicles (cysts) + anovulation.
A common cause of dec fertility in women.
Polycystic ovarian syndrome - Presentation
A - amenorrhea/oligomenorrhea
F - fertility (dec.)
A - acanthosis nigricans
C - cystic ovaries (Enlarged bilateral)
H - hirsutism
A - acne
O - obesity
Polycystic ovarian syndrome - Presentation
A - amenorrhea/oligomenorrhea
F - fertility (dec.)
A - acanthosis nigricans
C - cystic ovaries (Enlarged bilateral)
H - hirsutism
A - acne
O - obesity
Polycystic ovarian syndrome - Tx:
C - clomiphene
o
W - weight reduction (cycle regulation via dec. peripheral estrone formation)
S - spironolactone (to treat hirsutism)
O - OCPs (prevent endometrial hyperplasia due to unopposed estrogen)
F - finasteride (to treat hirsutism)
F - flutamide (to treat hirsutism)
Ovarian cysts
Follicular cyst - Distention of unruptured Graafian follicle -> Most common ovarian mass in young women.
May be associated with hyperestrogenism, endometrial hyperplasia.
Theca-lutein cyst - Often bilateral/multiple (Due to gonadotropin stimulation).
Associated with choriocarcinoma and hydatidiform moles.
Ovarian neoplasms - Inc risk:
Risk: P - PCOS A - advanced age I - infertility G - genetic predisposition (eg, BRCA-1 or BRCA-2 mutation, strong family history}) E - endometriosis
Lynch - Lynch syndrome
Ovarian neoplasms - Dec risk:
B - breastfeeding
O - OCPs
T - tubal ligation
+ previous pregnancy
Ovarian neoplasms - presentation
Presents with an adnexal mass, abdominal distension, bowel obstruction, pleural effusion.
Monitor response to therapy/relapse by measuring CA 125 levels (not good for screening)
Ovarian neoplasms - Surface epithelium tumors (benign)
Serous cystadenoma - Most common ovarian neoplasm. Lined with fallopian tube-like epithelium. Often bilateral.
Mucinous cystadenoma - Multiloculated, large. Lined by mucus-secreting epithelium
Ovarian neoplasms - Germ cell tumors (benign)
Mature cystic teratoma (dermoid cyst)
Most common in I0- 30 years old.
Cystic mass w/ all 3 germ layers (eg, teeth, hair, sebum).
pain 2° to ovarian enlargement or torsion.
A monodermal form with thyroid tissue (struma ovarii) uncommonly presents with hyperthyroidism.
Malignant transformation rare (usually to squamous cell carcinoma).
Ovarian neoplasms - Sex cord-stromal tumor (benign)
Fibroma
Sertoli-Leydig cell tumor
Thecoma
Ovarian neoplasms - Other (benign)
Brenner tumor
Resembles bladder epithelium (transitional cell tumor).
Solid tumor that is pale yellow-tan and appears encapsulated.
“Coffee bean” nuclei on H&E stain.
Usually benign.
Fibroma
Fibroma - Bundles of spindle-shaped fibroblasts.
Meigs syndrome- triad of ovarian fibroma, ascites, pleural effusion.
“Pulling” sensation in groin.
Sertoli-Leydig cell tumor
May produce androgens - virilization (eg, hirsutism, male pattern baldness, breast atrophy, clitoral enlargement, oligomenorrhea/amenorrhea).
Small, grey to yellow-brown mass and resembles testicular histology.
Thecoma
Thecoma - Like granulosa cell tumors, may produce estrogen.
Usually presents as abnormal uterine bleeding in a postmenopausal woman.
Surface epithelium tumors (malignant)
Serous cystadenocarcinoma - Most common malignant ovarian neoplasm, frequently bilateral.
Psammoma bodies.
Mucinous cystadenocarcinoma - Rare malignant mucinous ovarian epithelial tumor. Maybe metastatic from appendiceal or other GI tumors.
Can result in pseudomyxoma peritonei- intraperitoneal accumulation of mucinous material.
Germ cell tumors (malignant)
Dysgerminoma
Immature teratoma
Yolk sac tumor
Germ cell tumors (malignant) - Dysgerminoma
Most common in adolescents.
Equivalent to male seminoma but rarer.
1 % of all ovarian tumors, 30% of germ cell tumors.
Sheets of uniform “fried egg” cells.
hCG, LDH = tumor markers.
Germ cell tumors (malignant) - Immature teratoma
Aggressive, contains fetal tissue, neuroectoderm.
Commonly diagnosed before age 20.
Typically represented by immature/embryonic-like neural tissue.
Germ cell tumors (malignant) - Yolk sac tumor
Also known as ovarian endodermal sinus tumor.
Aggressive, in ovaries or testes and sacrococcygeal area in young children.
Yellow, friable (hemorrhagic), solid mass.
50% have Schiller-Duval bodies (resemble glomeruli).
AFP =tumor marker.
Sex cord-stromal tumors (malignant) - Granulosa cell tumor
Most common malignant stromal tumor.
Women in their 50’s.
Often produces estrogen and/or progesterone -> presents with postmenopausal bleeding, endometrial hyperplasia, sexual precocity (in pre-adolescents), breast tenderness.
Histology shows Call-Exner bodies (granulosa cells arranged haphazardly around collections of eosinophilic fluid, resembling primordial follicles.
Krukenberg tumor
GI malignancy that metastasizes to ovaries -> mucin-secreting signet cell adenocarcinoma.
Commonly presents as bilateral ovarian masses.
Primary dysmenorrhea
Painful menses, caused by uterine contractions to dec blood loss -> ischemic pain.
Mediated by prostaglandins -> Treatment: NSAIDs.
Endometriosis Tx
G - GnRH agonists O - OCPs (continuous)/progestins N - NSAIDs D - danazol e L - laparoscopic removal
Endometritis Tx
gentamicin + clindamycin +/- ampicillin.
Endometrial carcinoma
Endometrioid - Histology shows abnormally arranged endometrial glands.
Early pathogenic events include loss of PTEN or mismatch repair proteins.
Serous - associated with endometrial atrophy in postmenopausal women. Aggressive.
Characterized by the formation of papillae and tufts. TP53 mutations common.