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Lec 6 Benign Ovary Flashcards

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1
Q

What are the 3 functions of ovary?

A
  • house gametes
  • secrete estrogen, progesterone, testosterone
  • produce egg during ovulation in response to hormonal stimulation
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2
Q

What are disorders of sexual differentiation?

A

discrepancy between external genitalia and internal

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3
Q

What are the 3 categories of disorders of sexual differentiation?

A
  • 46 XX [female pseudohermaphrodite]
  • 46, XY [male psueodhermaphrodite]
  • mixed sex chromosome [true hermaphrodite or gonadal dysngenesis]
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4
Q

What are characteristics of person with 46 XX DSD [= 46 XX with virilization]?

A
  • ovaries and normal uterus and fallopian tubes
  • external genitalia look ambiguous or male
  • labia fuse and clitoris appears enlarged like a penis
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5
Q

What are 3 causes of 46 XX DSD [virilization]?

A
  • congenital adrenal hyperplasia
  • gestational hyperandrogenism
  • testicular and ovotesticular DSD
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6
Q

What is most common cause of sexual ambiguity of newborn?

A

congenital adrenal hyperplasia

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7
Q

What is the internal duct system in females?

A

paramesonephric ducts –> give rise to uterus, fallopian tubes, upper vagina

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8
Q

What is the internal duct system in males?

A

mesonephric ducts –> give rise to vas deferens, epididymis, seminal vesicles

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9
Q

How does 21-alpha-hydroxylase deficiency present?

A
  • high 17-hydroxyprogesteron

- can have salt wasting –> hyponatremia, hyperkalemia, hypotension

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10
Q

What are some causes of gestational hyperandrogenism?

A
  • male hormones [testosterone or synthetic progestational agents] taken by mother during pregnancy
  • male hormone-producing tumor in mother
  • placental aromatase deficiency [normally converts testosterone to estradiol]
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11
Q

What is 46 XX ovotesticular DSD?

A

virilization with mixed ovarian and testicular tissue = either ovotestis or ovary and testis

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12
Q

What happens to development in absence of testosterone?

A

system programmed to develop internally and externally as a phenotypic female

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13
Q

What is action of MIF [mullerian inhibiting factor]?

A

stimulates development of male [wolffian] duct system and regression of female

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14
Q

What are 4 causes of 46 XY DSD?

A
  • congenital adrenal hyperplasia
  • abnormal testicular activity
  • abnormal androgen synthesis
  • abnormal response to androgen
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15
Q

What is presentation of 46 XY DSD?

A
  • external genitals incompletely formed, ambiguous, or clearly female
  • internally testes may be normal, malformed, or absent
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16
Q

What does serum conc of mullerian inhibiting substance tell you?

A

marks sertoli cell mass; it is normal when functional testicular tissue is present

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17
Q

What is effect of decreased MIS secretion/action early in development?

A

fully developed mullerian duct structures = gonadal dysgenesis or persistent mullerian duct syndrome

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18
Q

What is effect of decreased MIS secretion/action late in development?

A

partial regression of mullerian duct structure = testicular regression syndrome, vanishing testis syndrome, or congenital anorchia

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19
Q

What do low MIS levels in a 46, XY individual suggest?

A

gonadal dysgenesis = have femal external genitatlia, intact mullerian ducts, streak gonads

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20
Q

What is most common cause of abnormal androgen synthesis in 46 XY DSD?

A

17-beta-hydroxysteroid dehydrogenase type 3 deficiency –> low/normal serum testosterone, very high androstenedione

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21
Q

What happens in 5-alpha reductase type 2 deficiency?

A

autosomal recessive 46 XY with bilateral testes and normal testosterone but defect converting testosterone to more potent DHT

may have normal male genitalia or femal or ambiguous; the most changed to external male genitalia occurs in puberty with increased androgen production

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22
Q

What is the most common cause of 46 XY DSD?

A

androgen insensitivity syndrome –> normal testes and testosterone but receptors don’t function properly

get testicular feminization

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23
Q

What is the presentation of patients with androgen insensitivity syndrome?

A
  • have breasts
  • absent axillary and pubic hair
  • absent upper vagina/uterus/fallopian tubes/ovaries = has a blind vaginal pouch
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24
Q

What is mixed gonadal dysgenesis vs true hermaphroditism?

A

mixed gonadal dysgenesis = 1/3 raised as males; have ambiguous external genitalia and persistent mullerian duct regardless of adjacent gonad

true hermaphroditism = 3/4 rales as males; internal duct development corresponds to adjacent gonad

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25
What is turner syndrome?
45XO; phenotypically female but have physical abnormalities --> webbed neck, edema on dorsum of hands/feet internally have streak [nonfunction/fibrous] gonads
26
How does turner syndrome present?
short stature, streak ovary, amenorrhea, low hairline, shield chest, infantile uterus
27
What is klinefelter?
47 XXY due to nondisjunction of sex chromosomes in either parent
28
What is most common cause of primary amenorrhea?
turner syndrome
29
What is most commone cause of congenital abnormality causing primary hypogonadism?
klinefelter syndrome
30
How common in klinefeleter?
1 in 850
31
What is presentation of klinefelter?
small, firm testes with low sperm count, infertility, high FHS/LH low testosterone eunuchoid body shape, tll long extremities, gynecomastia, female hair distribution
32
What is mixed gonadal dysgenesis?
45X/46XY or 46 XY phenotypically ambiguous have persistent mullerian duct regardless of adjacent gonad [unlike true hermaphrodie] - have one streak gonad and a contralateral abnormal testit - sterile
33
What is appearance of ovotesticular DSD?
can be phenotypically ambiguous or male or female genotype 46XX or 46XY or mosaic by definition have both ovarian and testicular tissue [separate ovary and testis or combined ovotestis] internal duct system corresponds to the gonad [vs in gonadal dysgenesis fallopian tube adjacent to testis]
34
Is uterus present in true hermaphrodite?
yes
35
What percent of true hermaphrodites are raised males vs females?
3/4 male | 1/4 female
36
Are pts with ovotesticular DSD [true hermaphroditism] fertile?
some are fertile
37
What do the mesonephric ducts give rise to?
vas deferens epididymis seminal vesicles
38
What do the external genitalia and urethra develop fomr?
urogenital sinus and genital tuberlce
39
What does the urogenital sinus give rise to?
boys: prostate and prostatic urethra girls: urethra and lower portion of vagina
40
What does the genital tubercle become?
boys: glans penis girls: clitoris
41
What do you urogenital swellings become?
scrotum or labia majora
42
WHat do the genital folds become?
labia minora or fuse to form male urethra and shaft of penis
43
What is necessary for ovarian development?
all 46XX chromosomes --> 45XO turners will have atretic ovaries = fibrous non-functional
44
What is required for differentiation of indifferent gonad into testis?
testis determining factor [TDF] which is located in SRY region of Y chromosome
45
What happens if no TDF gene?
gonadal dysgenesis
46
Where does mullerian inhibiting factor come from? actions?
synthesized by sertoli cells and causes ipsilateral regression of mullerian ducts by week 8 also plays a role in testicular descent
47
What stimulates wolffian [mesonephric] duct growth?
testosterone from leydig cells
48
What develops in absence of MIF?
fallopian tubes, uterus, upper vagina
49
What happens if low levels of MAF?
partial development of wolffian ducts and partial regression of para-mesonephric ducts
50
When do the testis begin secreting testosterone?
around 8 weeks
51
Why is it important to have 5a reductase?
converts testosterone to DHT = more potent active hormone | causes ambiguous genitalia until puberty with normal internal genitalia
52
What are the 3 majors types of non-neoplastic ovarian cysts?
- functional [follicular] cysts - endometriotic cysts - polycystic ovarian syndrome [PCOS]
53
What is best way to assess a newly discovered mass?
ultrasound | --> thick septations and solid components in cyst are suspicious for malignancy
54
How long does it take for physiologic ovarian cysts to regress?
6-10 weeks
55
What is an ovarian cyst?
collection of fluid surrounded by thing wall within an ovary if smaller than 2 cm = cystic follicle = harmless
56
What is a follicular cyst vs corpus luteum cyst?
follicular = follicle fails to release egg; fluid not reabsorbed and cyst formed corpus luteum = after eggr released from follicle
57
What is size of functional cyst?
> 2cm
58
Who gets functional cyst?
women of reproductive age
59
What is clinical presentation of functional cyst?
``` usually asymptomatic sometimes - pelvic pain - abnormal uterine bleeding - dyspareunia ```
60
What lines follicular cyst internally?
follicle lining cells = granulosa and theca interna cells
61
What is effect of oral contraceptive use on ovarian cysts?
decreases risk of developing cyst b/c prevent development of follicles
62
What test should you check in woman with ovarian cyst and abnormal ultrasound or older age?
Ca-125 to look for cancer
63
What blood tests should you order in woman with ovarian test?
- Ca-125 for cancer - HCG for pregnancy - LH/FSH/estradiol/testosterone
64
What is treatment for functional cysts?
regress spontaneously in 60 days; can accelerate by treatment with high dose oral contraceptives
65
What is endometriosis?
cells from lining of uterus [endometrium] appeara and grow outside uterine cavity [commonly ovary]
66
How common is endometriosis?
10-15% of reproductive age women
67
What are symptoms of endometriosis?
- pelvic pain, dysmenorrhea, low back pain, dyspareunia [painful intercourse], dysuria that is worse around menses commonly causes infertility
68
What is etiology of endometriosis?
may be genetic predisposition; estrogen implicated
69
What is gross appearance of endometriotic cyst?
cyst containing turbid brown content = chocolate cysts
70
What is histo appearance of endometriotic cyst?
lining composed of endometrial-like glands and stroma and hemorrhage
71
How do you diagnose endometriotic cyst?
- MRI or ultrasound | - surgery = gold standard but not covered by insurance
72
What is treatment for endometriosis?
pain control, hormonal therapy
73
How common is polycystic ovarian syndrome?
5-10% of reproductive age women
74
What is finding on sonogram in PCOS?
string of pearls arrangement of cystic follicles and thick, sclerotic cortical rim
75
What is the pathogenesis of PCOS?
- release of excess LH by the anterior pituitary OR - high levels of blood insulin OR - reduced levels of sex hormone binding globulin --> excess free androgens --> results in ovaries producing excess androgens, high LH:FSH ratio
76
How is PCOS inherited?
autosomal dominant with high penetrance but variable expressivity
77
What is the appearance of a polycystic ovary?
ovary with abnormally large number of developing eggs visible near surface = looks like many small cysts or a string of pearls ovary covered by thick fibrotic overlying capsule
78
What is presentation of PCOS?
- oligomenorrhea or amenorrhea - infertility [lack of ovulation] - hyperandrogenemia --> hirsuitism and acne - metabolic syndrome: central obesity, insulin resistance
79
What is primary dysmenorrhea?
prostaglandins produced in uterus at menses resulting in menstrual cramp
80
What are sonographic feat of benign vs malignant cysts?
benign: single locule, thin septations, unilateral malignant: complex cyst, can be bilateral, thick septations, may have ascites
81
How do you diagnose endometriosis?
tissue biopsy
82
Why is laparoscopy better than laparotomy?
- shorter recovery - less pain - less blood loss - less overall cost compared to laparotomy - even large cysts can be removed

Reproductive (14 decks)

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