Female Genital Flashcards
Which of the following is LEAST accepted as a gonococcal complication?
- Urethral stricture in a male
- Acute suppurative salpingitis
- Myometrial abscess
- Pyosalpinx and / or hydrosalpinx
- Tubo-ovarian abscess
- *Myometrial abscess
A clinical request states “?Paget’s disease of the Vulva” . Which of the following is most correct?
- The request is in error and likely means Paget’s disease of “breast” or “bone”
- This is a condition in which most have an underlying invasive SCC of the vulva
- This is a condition in which most have an underlying invasive adenocarcinoma of the vulva
- This is a condition in which most have an underlying invasive adenocarcinoma of the the lower vagina
- This is not usually associated with underlying carcinoma and, while wide local excision is preferred, may persist for decades without invasion/metastasis
- *This is not usually associated with underlying carcinoma and, while wide local excision is preferred, may persist for decades without invasion/metastasis
An MRI shows an incidental 1.5cm fluid filled structure in the vaginal wall closely related to the lumen in a 40 year old, separate from the cervix. Which of the following is most correct?
- It most likely represents a Bartholin’s cyst
- It most likely represents a Gartner duct cyst
- It most likely represents an epidermoid cyst
- It most likely represents a Nabothian cyst
- It most likely represents a focus of vaginal adenosis
- *It most likely represents a Gartner duct cyst
Concerning patient with carcinoma of the cervix, which of the following statements is most correct?
- If a patient with high grade squamous intraepithelial lesion is “lost to followup” they would have a 10% risk of invasive cancer at 2 years
- The average age of diagnosis is 60 – 65 years
- Squamous cell carcinomas account for over 97+% of cervical neoplasia
- Benign polyps are not recognised as forming in the cervical canal – all are considered at least low-grade malignancies
- Neuroendocrine tumor is not a recognised form of cervical carcinoma
- *If a patient with high grade squamous intraepithelial lesion is “lost to followup” they would have a 10% risk of invasive cancer at 2 years
Concerning the staging of cervical carcinoma which of the following is most correct?
- Stage 0 is used for carcinomas less than or equal to 7mm width and no deep (<3mm) invasion
- Stage 1 refers to carcinoma confined to the superficial epithelial layer of the cervix
- Stage II refers to extension confined to the deep cervix but not beyond
- Stage III should be considered when the tumors extends to the pelvic side wall or lower third of the vagina
- Stage IV indicates there is distant visceral metastasis
- *Stage III should be considered when the tumors extends to the pelvic side wall or lower third of the vagina
Which of the following is LEAST recognised as a cause of anovulatory cycles?
- Thyroid disease
- Granulosa cell tumor
- Polycystic ovarian syndrome
- Obesity
- MEN II syndrome
- *MEN II syndrome
Concerning endometriosis which of the following is LEAST correct?
- 6-10 % of women have foci of endometriosis
- Endometriosis may be seen in patients with amenorrhea due to gonadal dysgenesis
- Endometriosis can seen in lung, brain and bone sites
- Endometriosis can be seen in the urogenital tract of males treated with high dose estrogens
- Endometriosis is not associated with malignancies, increased risk of malignancy
- *6-10 % of women have foci of endometriosis
Concerning carcinoma of the endometrium which of the following is LEAST correct?
- Recognised common presenting complaints include post menopausal bleeding and perimenopausal menorrhagia
- Type I (accounting for 80%) has associations with diabetes, obesity, prolonged estrogen stimulation and hypertension
- It can occur in a setting of endometrial atrophy – particularly in older patients
- Pelvic peritoneal deposits indicate direct spread through the myometrium
- Invasion of the cervix represents Stage II disease
- *Pelvic peritoneal deposits indicate direct spread through the myometrium
A patient has a CT staging request stating “Mixed Mullerian Tumor: heterogenous mesenchymal component”, which of the following statements is most correct?
- This is a tumor of primitive embryonal remnants with its origin in the adenexa, between ovary and fimbria
- It is a tumor most commonly seen in adolescent females
- The heterogenous mesenchymal component suggests a poorer prognosis
- 5 Year survival, is good and in the order of 80 -90% even for high stage disease due to chemosensitivity
- The presence of bone / fat / muscle or cartilaginous elements should suggest a mature teratoma instead
- *The heterogenous mesenchymal component suggests a poorer prognosis
Concerning uterine leiomyomas, which of the following statements is most correct?
- A leiomyoma may spread unto uterine veins and even to lungs and still be considered benign
- The neoplasms are strongly EBV associated
- Rapid increase in size with pain in pregnancy should suggest malignant transformation
- Multiple small peritoneal nodules secondary to leiomyomas indicates malignant transformation
- They do not involve the uterine ligaments
- *A leiomyoma may spread unto uterine veins and even to lungs and still be considered benign
Concerning polycystic ovarian syndrome (PCOS), which of the following is most correct?
- It is associated with premature atherosclerosis
- It affects 0.5 -1 % of women of reproductive age
- Polycystic ovaries are seen in 50 - 70% of women and are not specific to PCOS
- It is associated with infertility but not proven to have associations with malignancy
- There is associated markedly elevated progesterone causing associated infertility
- *It is associated with premature atherosclerosis
Concerning ovarian neoplasms which of the following statements is LEAST correct?
- 80% of ovarian neoplasms are benign
- 10-15% of ovarian neoplasms are benign teratomas
- Serous surface epithelial tumors account for 30% of ovarian neoplasms (and ~50% of malignancies)
- Mucinous surface epithelial tumors account for only 2-5% of ovarian neoplasms
- Only 30% of serous surface epithelial tumors are malignant
- *Mucinous surface epithelial tumors account for only 2-5% of ovarian neoplasms
Concerning ovarian malignancy which of the following statements is LEAST correct?
- The presence and extent of papillary projections correlates with an increased risk of malignancy
- The presence and extent of solid components correlates with an increased risk of malignancy
- The presence of bilateral lesions strongly suggests malignancy (>60% risk of malignancy)
- The presence of fine calcifications / psammoma bodies is a feature of serous tumors but not necessarily carcinoma
- The presence of associated ascites suggests an increased risk of malignancy
- *The presence of bilateral lesions strongly suggests malignancy (>60% risk of malignancy)
Concerning endometroid carcinomas, which of the following is most correct?
- They account for less than 1% of ovarian carcinomas
- They have associations with both adenocarcinoma of the endometrium and endometriosis
- Peritoneal haemorrhagic nodules strongly suggest late stage disease
- Bilateral disease is rare (<1%)
- They are uniformly solid masses
- *They have associations with both adenocarcinoma of the endometrium and endometriosis
A PET study for carcinoid syndrome shows abnormal activity in an 8 cm heterogenous left ovarian mass but no other abnormal uptake. Which of the following is most correct?
- The appearances suggest a hypermetabolic focus, but likely incidental in the clinical setting
- The appearances may represent a carcinoid but carcinoid syndrome would not be expected given the apparent absence of metastasis
- Dysgerminomas are a common cause of 5 -HT production/ carcinoid syndrome
- The appearances suggest a special form of ovarian teratoma
- The appearances suggest choriocarcinoma
- *The appearances suggest a special form of ovarian teratoma
A 25 year old patient has PV bleeding and heterogenous non-cystic mass intrauterine mass expanding the uterus but states she has not had intercourse since her recent normal delivery 9 weeks ago. Beta hCG is positive. Which of the following is most correct?
- Choriocarcinoma or Placental Site trophoblastic tumor can present after a normal pregnancy
- An ovarian hormonally active neoplasm with endometrial hyperplasia is most likely
- A partial molar pregnancy was associated with the previous pregnancy
- A hydatiform mole associated with the previous pregnancy is most likely
- It may represent a retained infected cotyledon
- *Choriocarcinoma or Placental Site trophoblastic tumor can present after a normal pregnancy
The enlargement of the uterus in pregnancy is largely due to / best described as :
- Uterine hyperplasia
- Uterine hypertrophy
- Either uterine hypertrophy or hyperplasia are acceptable: the terms are interchangeable
- “Uterine enlargement” (the myometrium thins but overall cell numbers change by less than 10%)
- Endocrine-induced paraplasia
- *Uterine hypertrophy
Which is true? (March 2015)
a. Vulval melanoma is usually invasive at presentation
Vulval melanoma
o Second most common vulval cancer, typically affecting post-menopausal women
o Classified as mucosal melanomas
o Very rare – ten cases in Australia per year
o Usually diagnosed late and invasive or metastasized at presentation
ANSWER: Vulval melanoma is usually invasive at presentation
Which of these associations is true? (August 2014, March 2015)
a. Condylomata acuminatum is a precursor for SCC
Condylomata accuminatum (genital warts) are benign sexually transmitted warts
o Assoc w HPV 6 & 11; low malignant potential
o Occurs on moist mucocutaneous surfaces in either gender
o Tend to recur but only rarely progress to in situ or invasive SCC
• HPV 16 and 18 are considered to have high malignant potential and are assoc w increased risk of cervical cancer
ANSWER: Condylomata accuminatum is rarely a precursor lesion for SCC
Which of these is true? (March 2015)
a. Adenocarcinoma of the cervix has the same implicated HPV as SCC
Adenocarcinoma of the cervix arises at the squamocolumnar junction (as SCC)
• May arise from cervical adenoCa in situ
Risk factors: o Almost always associated w HPV 16 (80%) (less commonly HPV 18 - 10%) - Subtypes clear cell carcinoma and mesonephric carcinoma are not associated o Sexual history: Multiple previous or current partners; Young age at first intercourse o High parity o Immunosuppression o Oral contraceptives o HLA subtypes o Not assoc w smoking (although SCC is)
ANSWER: Adenocarcinoma of the cervix has the same implicated HPV as SCC (HPV 16>18)
Which is true of cervical adenocarcinoma? (March 2015)
a. More likely to be detected by pap smear than SCC
b. Spreads to the endometrium preferentially
c. Adenocarcinoma has the same HPV risk factors as SCC
AdenoCa of the cervix
o Less common histological subtype, accounting for 12.5% of cervical cancers
- (Proportion increasing, as it is less likely to be detected by pap smear)
Pathology:
- Arise from the squamocolumnar junction - Thought to arise from cervical adenoCa in situ, which is almost always assoc w HPV 16
Risk factors:
- HPV 16>18 - Multiple sexual partners, early age of first intercourse - High parity - Immunosuppression - HLA subtypes - Oral contraceptives - Cigarette smoking is not a risk factor (but is for SCC)
Pattern of spread:
Local: vagina, laterally to the bladder
Metastases: more likely to spread to the lung & adrenal glands than other cervical cancers
Subtypes:
- Clear cell – associated w DES, not assoc w HPV - Endometroid - Mucinous - Serous - Mesonephric
ANSWER: Cervical adenocarcinoma has the same HPV risk factors as SCC
Which is false regarding cervical carcinoma? (March 2017)
a. Adenocarcinoma has a worse prognosis than SCC
b. Neuroendocrine tumour has a poor prognosis
c. Upper vaginal involvement has a poor prognosis
d. Rectal involvement poor prognosis
e. Ureter involvement has a poor prognosis
ANSWER: Upper vaginal involvement does not have a poor prognosis – there is 29% mortality at 5 years (stage IIA disease)
Which are associated? (August 2014)
a. Adenocarcinoma of the cervix and HPV
b. SCC of the cervix and HIV
ANSWER: Adenocarcinoma of the cervix is associated with HPV
Regarding adenomyosis, which of the following is true? (September 2013)
a. Adenomyosis tends to cause more diffuse uterine enlargement than leiomyomas
b. Found in 1% of resected hysterectomy sections
c. Early loss of response to the cyclical hormone influence
d. Each rest of cells represents a polyclonal population
e. Rare but characteristic venous/villous infiltration
Adenomyosis:
o Benign lesion of the uterus
- Considered on the spectrum of endometriosis
- Ectopic endometrial tissue in the myometrium
- Smooth muscle hyperplasia
- Dysfunctional myometrium does not contract properly and leads to menorrhagia
o Clinical:
- Menorrhagic
- Dysmenorrhoea
- Chronic pelvic pain
o Epidemiology:
- Multiparous women
- Women with a history of instrumentation
- 20% of women affected
o Four types:
- Diffuse adenomyosis (most common)
- Focal adenomyosis
- +/- Adenomyoma (controversial as a separate entity)
- Cystic adenomyosis and adenomyotic cyst (rare)
o Globular enlargement of the uterus - Contour usually preserved
ANSWER: Adenomyosis tends to cause more diffuse uterine enlargement than leiomyomas
Association of cervical cancer with smoking (March 2014)
Risk factors for cervical cancer:
o HPV 16 and 18 - Except for clear cell carcinoma of the cervix & mesonephric carcinoma of the cervix
o Multiple sexual partners or a male partner w multiple previous partners
o Young age at first intercourse
o High parity
o Immunosuppression
o Certain HLA subtypes
o Oral contraceptives
o Smoking - Except for cervical adenocarcinoma
Different types of cervical carcinoma: o Squamous cell carcinoma: - Large cell keratinizing squamous cell carcinoma - Large cell nonkeratinizing SCC - Small cell nonkeratinizing (poorly differentiated) - Morphologic variants: • Spindled • Lymphoepithelial-like carcinoma • Varrucous carcinoma • Condylomatous (warty) carcinoma • Papillary squamous and squamotransitional carcinoma • Basaloid squamous carcinoma
o Adenocarcinoma
o Villoglandular adenocarcinoma
o Endometroid adenocarcinoma
o Clear cell adenocarcinoma
o Adenoid basal carcinoma
o Adenoid cystic carcinoma
o Neuroendocrine tumours:
- Carcinoid - Atypical carcinoma - Small cell neuroendocrine neoplasia - Large cell neuroendocrine carcinoma
Which is false regarding adenomyosis? (March 2017)
a. Involved uteruses have a coarsely nodular external contour
b. Uterine enlargement/wall thickening is predominantly due to muscle hyperplasia/hypertrophy
ANSWER: Involved uteruses typically have an enlarged, smooth globular contour (not coarse or nodular)
Regarding endometrial hypertrophy:
a. If complex with atypia, 1/3 progress to carcinoma
b. If simple with atypia, 1/3 progress to carcinoma
As per statdx
o Complex with atypia – 25% progress to carcinoma
o Simple with atypia – 2% progress to carcinoma
ANSWER: If complex with atypia, 1/3 (25%) progress to endometrial carcinoma
Regarding endometrial cancer: (March 2015)
a. Tamoxifen is associated with type 2
b. Type 1 is associated with atrophy
c. Type 2 is associated with oestrogen secretion
Type 1 endometrial cancer (80%): o Arises in the setting of unopposed hyper-oestrogenism and endometrial hyperplasia o Epidemiology: women 55-65 years o Well differentiated, slow progression, good prognosis o PTEN gene mutation in 30-80% o Risk factors: Oestrogen replacement therapy PCOS and anovulatory cycles Tamoxifen Obesity Early menarche and late menopause Nulliparity Oestrogen producing ovarian tumours e.g. granulosa cell cancer Diabetes
Type 2 endometrial cancer (20%): o Arises in the setting of endometrial atrophy o Epidemiology: women 65-75 years o P53 mutation in up to 50% o Less well differentiated - Lymphatic spread early - Peritoneal seeding via fallopian tubes - Poorer prognosis
Associated cancer syndromes:
o HNPCC – 30-50x increased risk
o Precursor lesions of complex hyperplasia with atypia are associated in ~40%
ANSWER: All options are incorrect
Which is true? (September 2013)
a. Imaging can differentiate between endometrial hyperplasia and carcinoma
b. Tamoxifen causes endometrial thickening
c. Polyps develop malignancy in 75%
d. Endometrial atrophy is not a cause of post-menopausal bleeding
ANSWER: Tamoxifen causes endometrial thickening
Which is true? (March 2015)
a. Pregnancy is associated with disseminated peritoneal leiomyomatosis
b. Leiomyoma is a precursor lesion for leiomyosarcoma
c. Leiomycosarcoma typically presents with metastases
d. Leiomyosarcoma typically spreads to the brain
Leiomyosarcoma accounts for 1/3 of uterine sarcomas and 8% of uterine tumours
o Arise de novo or from a prexisting leiomyoma
(Sarcomatous transformation of leiomyoma occurs in 0.1-0.8%)
Pattern of tumour spread:
- Myometrium, pelvic blood vessels and lymphatics, adjacent pelvic structures, abdominal cavity
- Metastases later, most commonly to lungs (liver and brain)
Presentation:
- Abnormal PV bleeding
- Pelvic mass (massive uterine enlargement)
- Pelvic pain
- Symptoms from metastases is an uncommon presentation
ANSWER: Leiomyoma is a precursor lesion for leiomyosarcoma (0.1-0.8% sarcomatous degeneration)
Regarding leiomyosarcoma, which is true? (March 2017)
a. Haematogenous spread commonly occurs
b. Development from a leiomyoma is rare
c. Distinguishing benign from malignant is most difficult in young patients
ANSWER: Development from leiomyoma is rare (0.1-0.5%)
Regarding leiomyoma, which is false? (March 2017)
a. Most commonly presents in women in their 20s and 30s
b. Vascular invasion is possible
c. Enlarge with pregnancy and infarction
ANSWER: Leiomyomas more commonly present in women in their 30s and 40s (not 20s)
Which is associated with diffuse uterine enlargement with an irregular contour? (March 2016)
a. Adenomyosis
b. Leiomyomas
c. Endometrial carcinoma
ANSWER: Leiomyomas
Which association is true? (August 2014)
a. Leiomyosarcoma and DES
b. Rhabdomyosarcoma and retinoblastoma
DES associated abnormalities:
- synthetic oestrogen prescribed to women in 1948-1971
o First generation:
- Increased risk of breast cancer and breast cancer mortality
o Second generation (daughters):
- Vaginal clear cell adenocarcinoma
- Cervical squamous cell dysplasia
- Breast cancer
- T shaped uterus (Increased risk of poor pregnancy outcomes)
- Vaginal adenosis
o Second generation (sons):
- Cryptorchidism
- Hypospadias
- Hypogonadism
Retinoblastoma germline mutations: o Retinoblastoma o Retinocytoma o Pineoblastoma o Osteosarcoma
ANSWER: DES is not associated with leiomyosarcoma, but it is strongly associated with vaginal clear cell adenocarcinoma. Retinoblastoma is associated with osteosarcoma, not rhabdomyosarcoma.
Regarding leiomyoma, which is true? (September 2013)
a. The presence of PV bleeding is assoc with an increased risk of malignant transformation
b. There is not even moderate mitotic activity
c. Size greater than 10cm is associated with increased risk of malignancy
d. Cords of cells in the venous system is consistent with malignancy
e. Benign leiomyomas are polyclonal
• 30% of patients with benign leiomyoma have abnormal PV bleeding
• Leiomyomas have a very low mitotic rate (<5 figures/10 high power fields)
• Atypical growth patterns for leiomyomas (not necessarily malignant):
o Disseminated intraperitoneal leiomyomatosis
o Benign metastasizing leiomyoma (to lungs)
o Intravenous leiomyomatosis
o Lymphangioleiomyomatisis
ANSWER: There is not even moderate mitotic activity associated with leiomyoma
Which of these is true? (March 2015)
a. Carcinosarcoma of the cervix
b. Adenosarcoma of the endometrium
- Adenosarcoma of the uterus is an uncommon cancer of the mesenchymal tissues of the uterus
- Carcinosarcoma of the cervix is extremely rare
Classification of uterine sarcomas:
o Mixed:
- Malignant mixed mullerian tumour of the uterus (~50-70%)
- Adenosarcoma of the uterus
- Mixed uterine leiomyosarcoma and endometrial stromal sarcoma
o Pure:
- Uterine leiomyosarcoma (35-50%) - Endometrial stromal sarcoma (10%) - Other rare sarcomas of the uterus (Fibrosarcoma, Rhabdosarcoma, Liposarcoma, Angiosarcoma)
Main histological types of cervical cancer:
o Squamous cell carcinoma (80-90%)
o Adenocarcinoma of the cervix (5-20%)
- Clear cell carcinoma of the cervix
- Endometroid carcinoma of the cervix (7%)
- Mucinous carcinoma of the cervix - Adenoma malignum (3%)
- Serous carcinoma of the cervix
- Mesonephric carcinoma of the cervix (3%)
o Neuroendocrine tumours of the cervix - Small cell carcinoma (0.5-6%)
o Adenosquamous carcinoma of the cervix (rare)
ANSWER: Adenosarcomas of the uterus are a subtype of mixed uterine sarcomas. Carcinosarcoma of the cervix is extremely rare.
Which is true of endometrial cancer? (August 2016)
a. Type 1 is usually low grade
b. In endometrial sarcoma, the most common epithelial component is clear cell
c. Malignant mixed mullerian tumour often has the morphology of a polyp
d. Type 1 is associated with atrophy
e. Type 2 is associated with ovarian endometroid cancer
Type 1 endometrial cancer:
o 80%; Arises in the setting of unopposed hyperoestrogenism and endometrial hyperplasia
o Women 55-65 years
Pathology:
- Well differentiated tumours, favourable outcomes - PTEN gene mutation in 30-80% - Histological subtypes: Endometroid carcinoma of the endometrium (85%)
Risk factors:
- Oestrogen replacement therapy - PCOS and anovulatory cycles - Tamoxifen - Obesity - Early menarche or late menopause - Nulliparity - Oestrogen producing ovarian tumours e.g. granulosa cell cancer - Diabetes mellitus
Type 2 endometrial cancer:
o 20%; Arises in the setting of endometrial atrophy
o Women 65-75 years
Pathology:
- p53 mutation in 50%
- Poorly differentiated, early spread via lymphatics and fallopian tubes into the peritoneum
- Poorer prognosis
- Histological subtypes:
• Papillary serous carcinoma of the endometrium
• Clear cell carcinoma of the endometrium
• Adenosquamous carcinoma of the endometrium
• Adenocarcinoma of the endometrium with squamous differentiation
• Undifferentiated of the endometrium e.g. small cell undifferentiated carcinoma of the endometrium
Associations: Hereditary non-polyposis colon cancer: 30-50x increased lifetime risk
• Precurser lesions (complete hyperplasia with atypia) associated in >40% of cases
Endometrial sarcoma (endometrial stromal sarcoma):
o Malignant subtype of endometrial stroma tumours
- Low grade endometrial stromal sarcoma
- Undifferentiated uterine sarcoma
o <2% of uterine malignancies; 10% of uterine sarcomas
o Pre-menopausal women – 5th decade
Malignant mixed mullerian tumour of the uterus (uterine carcinosarcoma):
o 50% of uterine sarcomas
o Epithelial and mesodermal components
Epithelial component subtypes: • Endometroid adenocarcinoma • Clear cell carcinoma • Mucinous carcinoma • Papillary-serous carcinoma
Sarcomatoid component subtypes:
• Undifferentiated sarcoma
• Rhabdomyosarcoma
o Present as an intracavity mass with dilatation of the endometrial canal
ANSWER: Endometrial carcinoma is usually low grade (80% - type I)
What is the most common cause of diffuse uterine enlargement with an irregular contour? (August 2016)
a. Multiple leiomyomas
b. Adenomyosis
ANSWER: Multiple leiomyomas
Regarding PCOS: (March 2015)
a. It is a risk factor for ovarian cancer
b. Unilateral ovarian enlargement is seen in stromal hyperthecosis
• PCOS is a risk factor for endometrial cancer
Ovarian hyperthecosis:
o Presence of luteinised thecal cells w/in a hyperplastic ovarian stroma
Clinical manifestations:
- Hyperandrogenism - Obesity - Hypertension - Impaired glucose tolerance - Virilisation more common in pre-menopausal women and hyper-oestrogenism in post-menopausal women
Pathology:
- Moderately hyperplastic ovarian stroma with luteinised thecal cells (single cells, nests or nodules)
- Luteinised cells located centrally in ovarian hyperthecosis, and located peripherally in PCOS
Associations:
- Endometrial hyperplasia - Endometrial carcinoma - Ovarian fibrothecoma
Features:
- Increased ovarian size bilaterally - May appear normal or nodular
ANSWER: Both options are incorrect, PCOS is a risk factor for endometrial cancer and ovarian hyperthecosis typically causes bilateral ovarian enlargement
Which association is false? (March 2016)
a. Turner syndrome and gonadoblastoma
b. Fibroma and Meig syndrome
c. Thecoma and endometrial thickening
d. Sertoli-Leydig cell and hyperandrogenism
Gonadoblastoma:
o Associated w disorders of sexual development:
- Newborn with ambiguous genitalia
- Precocious puberty or virulisation
Epidemiology:
- Usually discovered before age 30, most commonly in the neonatal period
- May occur in phenotypic males or females
Pathology:
- Benign tumour, can develop into a germ cell tumour if not resected
- If the patient has a contralateral undescended testis, this is often removed as well due to the risk of bilateral gonadoblastoma
- Bilateral in 50% of cases
- Assoc w chromosomal abnormalities & gonadal dysgenesis
- Only assoc w Turner syndrome if there is XY mosaicism
ANSWER: Turner syndrome is not typically associated with gonadoblastoma unless there is XY mosaicism
Which ovarian tumour is most likely to occur in post-menopausal women? (March 2015)
a. Mucinous cystadenoma
b. Granulosa
c. Serous cystadenoma
d. Serous cystadenocarcinoma
e. MMMT
Serous cystadenoma:
o Peak incidence in 4th and 5th decades
o 25% of all benign ovarian neoplasms (50-70% of serous tumours are benign)
o 10-20% bilateral
o 84% of simple cysts in post menopausal women are serous cystadenomas at surgery
ANSWER: Serous cystadenoma
Which is true regarding BRCA2 mutation? (March 2017)
a. Associated with serous ovarian carcinoma
b. Associated with mucinous ovarian carcinoma
ANSWER: BRCA2 is associated with a 15-25% lifetime risk of ovarian serous carcinoma
Regarding ovarian cystadenocarcinoma, what is most true? (August 2016)
a. Ovarian serous adenocarcinoma arises from the fallopian fimbria
Ovarian cystadenocarcinoma
o Malignant ovarian epithelial tumour (serous tumour)
o Largest proportion of malignant epithelial tumours (50-80%) - 25% of serous tumours
o 6th – 7th decades
Pathology:
- Multilocular cystic ovarian tumour with papillary projections
- Psammomatous bodies in ~30%
- Elevated CA-125 in >90%
- Arise from the epithelium at the fimbriated end of the fallopian tube
ANSWER: Ovarian cystadenocarcinoma arises from the fallopian fimbria
Regarding mucinous cystadenocarcinoma, which is true? (August 2016)
a. Usually unilateral
b. Usually bilateral
c. Is the most common cause of peritoneal carcinomatosis
Bilaterality of surface epithelial stromal tumours o Serous - Serous cystadenoma (benign): 20% - Serous cystadenoma (borderline): 30% - Serous cystadenocarcinoma: 66% o Mucinous cystadenoma and carcinoma: 5% o Endometroid carcinoma: 40% o Brenner (transitional cell): 10%
Mucinous cystadenoma:
o 30-50 years
o 20-25% of benign ovarian tumours, 80% of mucinous tumours
Pathology: lined by columnar epithelium, multiloculated filled with thick, gelatinous mucin
- Mural calcification more common than in serous tumours
Mucinous cystadenocarcinoma
o 5-10% of ovarian mucinous tumours
o Very rarely arises from degeneration of an ovarian teratoma
ANSWER: Mucinous cystadenocarcinoma is usually unilateral
Which of the following ovarian lesions is most commonly bilateral? (September 2013)
a. Endometroid
b. Mucinous
c. Fibroma
d. Brenner
ANSWER: Endometriod tumours are the most likely to be bilateral out of these options
Serous (65%)> Metastatic (>50%) > Endometrioid and clear cell (40%) > Teratoma (15%) > Mucinous (5-10%) > Granulosa cell tumour (5%)
A patient has a lesion in the right ovary. Which of the following would most favour serous cystadenocarcinoma? (September 2013)
a. A similar lesion on the left
b. Extensive calcification
c. Solid enhancing components
d. Increased AFP
- 65% of malignant ovarian cystadenocarcinomas are bilateral, whereas 25% of benign ovarian cystadenomas are unilateral
- Solid enhancing components and papillary projections are markers of malignancy (But not specific for cystadenocarcinoma)
- Cystadenocarcinoma is associated w elevated serum Ca-125
ANSWER: Bilaterality would favour serous cystadenocarcinoma over other diagnoses
The first lymph nodes involved in ovarian cancer are: (August 2016)
a. Retroperitoneal
b. Inguinal
ANSWER: Retroperitoneal (pelvic also common)
What is the most likely non-cystic ovarian tumour? (August 2016, March 2017)
a. Brenner tumour
Predominantly solid ovarian neoplasms: o Brenner tumour o Thecoma o Fibroma o Endometroid granulosa cell tumours o Dysgerminoma o Endodermal sinus tumour (yolk sac tumour) o Metastatic
ANSWER: Brenner tumour
Which association is true? (March 2014)
a. Carcinosarcoma and post-menopausal females
b. Vulval sarcoma and …
Carcinosarcoma of the ovary
o Rare type of malignant mixed mullerian tumour (MMMT) of the ovary
o Less than 1% of ovarian cancers
Epidemiology: Post menopausal females; 6th – 8th decades
Pathology:
- Biphasic carcinomas w epithelial & stromal elements
- High incidence of haemorrhagic ascites
o Aggressive neoplasms with a poor prognosis
ANSWER: Carcinosarcoma occurs in post-menopausal females
Regarding ovarian tumours (?cancer): (September 2013)
a. Struma ovarii is a recognized pattern
Struva ovarii
o Subtype of ovarian teratoma composed entirely (or predominantly) of thyroid tissue & containing variable-sized follicles w colloid material
- >50% of the tumour should be thyroid tissue for diagnosis
o 5-8% of patients show evidence of clinical thyrotoxicosis
o 90-95% of struma ovarii are benign
ANSWER: Unclear – struva ovarii is a recognized pattern in ovarian teratoma
Which is true regarding ovarian teratomas? (March 2016)
a. Ovarian teratomas are associated with limbic encephalitis
b. Immature teratomas are associated with carcinoid syndrome
Causes of limbic encephalitis: o Small cell lung cancer o Testicular germ cell tumours o Thymic neoplasms o Breast cancer o Ovarian tumours e.g. ovarian carcinoma or teratoma o Haematological malignancies e.g. Hodgkin lymphoma o Gastrointestinal malignancy o Neuroblastoma
Syndromes rarely associated with mature teratomas:
o Hyperthyroidism/thyrotoxicosis – struma ovarii
o Carcinoid syndrome
ANSWER: Ovarian teratomas are associated with limbic encephalitis
Which is false of ovarian cancer? (September 2013)
a. Gestation and non-gestational choriocarcinoma have the same prognosis
b. Prognosis of fibroma is not affected by ascites
c. Brenner tumours are usually solid
ANSWER: Non-gestational choriocarcinomas have a much worse prognosis than gestational choriocarcinoma
Which is false regarding choriocarcinoma? (March 2017)
a. Gestational and non-gestational choriocarcinoma have the same prognosis
b. Choriocarcinomas metastasise early and often have metastases at diagnosis
ANSWER: Non-gestational choriocarcinoma has a much worse prognosis than gestational choriocarcinoma
Regarding Meigs syndrome, what is most correct? (March 2015)
a. Right sided chylothorax
b. Right sided hydrothorax
c. Left sided haemothorax
d. Left sided chylothorax
e. Bilateral haemorrhagic pleural effusions
Features of Meigs syndrome:
o Ascites and pleural effusion assoc w a benign, usually solid ovarian tumour
- Usually a fibroma (80-90%), but less commonly fibrothecoma, thecoma, granulosa cell tumour or Brenner tumour
o Pleural effusion is right sided in 60-70%
ANSWER: Right sided hydrothorax
Which neoplasm does not cause Meigs syndrome? (August 2014)
a. Brenner
b. Dysgerminoma
c. Granulosa cell tumour
d. Fibroma
e. Thecoma
Meigs syndrome
Clinical:
- ascites & pleural effusion assoc w a benign (usually solid) ovarian tumour
- Pleural effusion and ascites usually resolve post resection of the tumour
Associated ovarian tumours: Fibroma (90%) Fibrothecoma Thecoma Granulosa cell tumour Brenner tumour (rare)
ANSWER: Meigs syndrome is not associated with dysgerminoma
What is most likely to be hormonally active in a young patient? (March 2015)
a. Juvenile granulosa cell
b. Choriocarcinoma
c. Yolk sac tumour
d. Serous cystadenoma
e. Immature teratoma
Juvenile granulosa cell tumour:
- Ovarian sex cord/stromal tumour
- accounts for 5% of granulosa cell tumours
Epidemiology:
- Premenarche girls and young women - Mean age at presentation 13
Endocrine:
- Precocious puberty as a result of oestrogen secretion (May cause resultant uterine enlargement and endometrial thickening) - Rarely produce androgens
Associations:
- Mafucci syndrome - Ollier disease
o 90% low grade – surgery is curative in these patients. Higher grade may require chemoTx
Summary of other options:
o Choriocarcinoma: secretes bHCG
o Yolk sac tumour: secretes AFP
o Serous cystadenoma: non-secretory
o Immature teratoma: elevated AFP in 50%, usually does not secrete bHCG
- 3% of mature cystic teratomas will contain struma ovarii (mature thyroid tissue) and secrete thyroid hormone (8% present with thyrotoxicosis)
ANSWER: Juvenile granulosa cell tumour
What neoplasm is associated with pseudohermaphrodism? (March 2014)
a. Leydig cell tumour
b. Sertoli-Leydig cell tumour
c. Graulosa cell tumour
d. Serous malignancy
e. Mucinous tumour
Sertoli-Leydig tumour
- Rare tumours of the ovary
- Assoc w mutations of the DICER1 gene
- 25% malignant
Pathology:
- Variable proportions of Sertoli and Leydig cells - Tubules lined with Sertoli cells and intervening clusters of Leydig cells in well differentiated tumours
Clinical:
- Excess testosterone excreted by the tumour
• High serum testosterone in 2/3
• No specific tumour markers
- 1/3 of female patients present with progressive masculinization, Anovulation, Oligomenorrhoea/amenorrhoea, Defeminisation: acne, hirsuitism, clitoromegaly, temporal hair recession, increased musculature
ANSWER: Sertoli-Leydig cell tumours are associated with pseudohermaphrodism
Which tumour is most likely to result in hyperandrogenism? (March 2014)
a. Granulosa cell tumour
b. Leydig cell tumour
c. Sertoli-leydig cell tumour
d. Serous cystadenoma
- Granulosa cell: oestrogen
- Leydig cell: virulisation +/- hyperoestrogenism
- Sertoli-leydig cell: virulisation +/- masculinisation. Can be a cause of pseudohermaphrodism in women
ANSWER: Sertoli-leydig cell tumours are most associated
What is least likely to cause foetal hydrops? (March 2015)
a. Paroxysmal SVT
b. Parvovirus
c. Thoracic mass
Hydrops fetalis is excessive loss of fluid into the 3rd space in a foetus (at least two fetal compartments)
Causes of foetal hydrops:
o IMMUNE (10%)
- Fetomaternal blood group incompatibility – erythroblastosis foetalis (Less common due to management of Rhesus incompatibility)
o NON-IMMUNE
- Chromosomal abnormalies: Turner’s syndrome; Trisomies: T13, T18, 21
- Cardiac causes: Foetal tachyarrhythmias; Congenital cardiac anomalies; Foetal cardiac tumours e.g. cardiac rhabdomyoma
- Twin related complications: Twin-twin transfusion (recipient twin); Twin reversed arterial perfusion sequence (pump twin)
- In utero infections: TORCH group; Parvovirus B19 (most common infectious cause of hydrops - causes foetal anaemia); Coxsackie virus
- Fetal tumours: Sacrococcygeal teratoma; Hepatic haemangioendothelioma; Placenta choriocarcinoma
- Inborn errors of metabolism: Gaucher’s disease; Niemann-Pick disease
- Congenital foetal anaemias: Alpha thalassaemia / Haemoglobin Bart’s
- Thoracic/pulmonary anomalies (thought to be due to venous return obstruction) • Primary foetal hydrothorax • CPAM • Congenital diaphragmatic hernia • Pulmonary sequestration
Other: • Hypoproteinaemic states • Skeletal dysplasias • Lymphovascular anomalies • High output flow states
ANSWER: All options are potential causes of foetal hydrops
Twin-twin transfusion can occur in:
a. Monochorionic diamniotic
b. Other diamniotic options
Twin-twin transfusion is a complication of monochorionic twin pregnancies (MCDA)
o 10% of monochorionic pregnancies
Pathology:
o Unbalanced arterio-venous communication in the placenta - Asymmetric anastomotic patterns
o Donor twin (stuck twin): pump twin, oligohydramnios, smaller
o Recipient twin: polyhydramnios, larger
o >20% growth discordance bw the twins
o Amniotic fluid discrepancy
Staging:
o Stage I: visible bladder in donor twin with normal Dopplers
o Stage II: empty bladder in donor twin with normal Dopplers
o Stage III: empty bladder in the donor twin with abnormal Dopplers
o Stage IV: Hydrops fetalis in recipient twin
o Stage V: Demise of any twin
TAPS (twin anaemia-polycythaemia sequence)
o One twin develops anaemia and the other develops polycythaemia
o No amniotic fluid discordance
ANSWER: TTTS is seen in diamniotic monochorionic pregnancies
Which is correct regarding twin pregnancies? (March 2016)
a. Twin-twin transfusion can occur in dizygotic twins
b. Fused twins which are dichorionic diamniotic indicate a monozygotic pregnancy
c. Dichorionic diamniotic pregnancy indicates a monozygotic gestation
d. Monochorionic pregnancy indicates a monozygotic gestation
ANSWER: Monochorionic pregnancies arise from a monozygotic gestation
Most likely cause of placenta praevia? (March 2015)
a. Succenturiate lobe
b. Bilobed placenta
c. Velamentous cord insertion
d. Placenta membranacea
e. Circumvellate
Risk factors for placenta praevia: o Previous placenta praevia o Previous caesarean section o Increased maternal age o Increased parity o Large placentas: Multiple gestations; Erythroblastosis o Maternal history of smoking
o Succenturiate lobe:
- Smaller accessory placental lobe which is separate to the main disc of the placenta
- May be multiple
- Increased risk of vasa praevia
o Bilobed placenta:
- Variant placental development where the placenta consists of two discs of comparable size
- Assoc w velamentous cord insertion
- Increased risk of vasa praevia and post-partum haemorrhage secondary to retained products
- Incidence up to 4% of pregnancies
o Velamentous cord insertion: - Umbilical cord inserts into the foetal membranes outside the placental margin - Thought to result from processes leading to remodelling of the placenta as a response to abnormal blood flow Associations • Bilobed placenta • Twin pregnancy • Uterine anomalies • Presence of an IUD • Single umbilical artery • Placenta praevia Complications: • Vasa praevia • Increased risk of IUGR • Increased risk of complications of twin pregnancies: Discordant growth; TTTS
o Placenta membranacea:
- Extremely uncommon variant (1 in 20000-40000)
- Placenta develops as a thin membranous structure occupying the entire periphery of the chorion
- Placental mass can be 1-2cm thin & deficient in some areas
Associations: abnormal placental adherence in 30%
Complications:
• Placenta praevia
• IUGR
• Recurrent antepartum haemorrhage
• Second trimester miscarriages
• Foetal demise
• Post-partum complications: PPH; RPOC
o Circumvellate placenta:
- Due to a small chorionic plate, the amnion and the membranes double back around the edge of the placenta
Pathology:
• Excessive implantation occurs, covering more than half of the foetal sac
• Placenta reduces this excessive implantation by detaching at the sides
• Membranes cover the detached placenta giving a rolled edge
• Gives the appearance of a ‘placental shelf’ on ultrasound
Associations:
• Higher incidence of placental abruption
• Increased risk of IUGR
ANSWER: Placenta membranacea is the only CAUSE of placenta previa. Velamentous cord insertion would be the most common association.
What has the highest associated risk of uterine rupture? (March 2015)
a. Placenta praevia
b. Placenta increta
c. Placenta accreta
d. Placenta percreta
e. Abruption
Uterine rupture:
o >90% caused by an old Caesarian scar
- “Classic” scars tend to rupture before labour
- Lower uterine segment scars tend to rupture after labour
o Uterine dehiscence may be limited by an intact serosal layer
o Full thickness rupture is assoc w massive haemoperitoneum & high rate of mortality for both mother & foetus
o Among the spectrum of abnormal villous adherence, placenta percreta has the highest risk of uterine rupture
Spectrum of abnormal villous adherence:
o Risk factors:
Prior Caesarian section
Placenta praevia
Advanced maternal age
Uterine anomalies
Intrauterine adhesion bands
Previous surgery
o Placenta accreta:
Mildest and most common (75%)
Villi are attached to the myometrium but do not invade the muscle
o Placenta increta:
Intermediate form (20%)
Villi partially invade the myometrium
o Placenta percreta:
Most severe, but least common (5%) - Incidence increasing due to the increased rate of Caesarian delivery
Transmural extension of placental tissue w serosal breech
May involve adjacent organs such as bladder or bowel
May be complicated by uterine rupture or peripartum haemorrhage
ANSWER: Placental percreta
Regarding molar pregnancy: (March 2015)
a. Partial mole is paternally derived
b. Partial mole is associated with choriocarcinoma
c. Invasive mole is only from a complete mole
d. Complete mole has foetal parts
Complete hydatiform mole:
o Most common manifestation of gestational trophoblastic disease
o Characterised by the absence of foetal parts - Non invasive, diffuse swelling of the chorionic villi
o 90% have 46XX; 10% are 46XY - All chromosomes are paternally derived
Complications:
- Degeneration into invasive or malignant types of gestational trophoblastic disease occurs in ~10-20%
Partial hydatiform mole:
o Contains an embryo or foetus
o Greatly enlarged placenta relative to the uterus, commonly containing cystic spaces
o Triploid karyotype, with the extra set of chromosomes usually paternal
o Increased risk of invasive mole, no increased risk of choriocarcinoma
ANSWER: All are incorrect
Regarding hydatiform mole, which is least likely? (March 2014)
a. Partial mole is less associated with choriocarcinoma
b. Complete mole is frequently associated with foetal parts
c. Could be associated with abortion
ANSWER: Complete mole is not associated with foetal parts, however partial mole is
Regarding choriocarcinoma, which is most correct? (August 2016)
a. Severely secretes bHCG
b. 25% arise post abortion
Choriocarcinoma of the uterus
o One of the most common choriocarcinomas, assoc w gestational trophoblastic disease
o Usually occurs w/in one year of pregnancy
- Non-gestational choriocarcinoma of the uterus is rare
o bHCG is typically elevated above levels expected for molar pregnancies
- Can have lower bHCG, especially if there is a large necrotic component to the tumour
Pathology:
- Highly vascular neoplasm - Tumour of trophoblastic cells - Distinguished from other gestational trophoblastic disease by an absence of chorionic villi - 5% of cases of complete hydatiform mole are followed by choriocarcinoma (accounts for half of the cases)
- 25% arise after normal pregnancies
- 25% follow spontaneous abortion (20-25%) or ectopic pregnancy (2%) - 1% of gestational trophoblastic disease
Prognosis:
- Cases arising from complete hydatiform mole are usually completely cured by chemotherapy
- Cure rate 90-95% due to the presence of paternal DNA
- Non-gestational choriocarcinoma has a worse prognosis
ANSWER: Both responses are correct – choriocarcinoma secretes bHCG and 20-25% arise following abortion
Which is most correct regarding choriocarcinoma? (August 2014)
a. Unlikely to recur
b. Associated with ectopic pregnancy
ANSWER: Gestational choriocarcinoma is unlikely to recur as it is exquisitely chemosensitive. 2% are associated with ectopic pregnancy.
Regarding placental site trophoblastic tumour, which is true? (March 2017)
a. The tumour severely secretes bHCG
b. Can occur years after a normal pregnancy
Placental site trophoblastic tumour (PSTT):
o <2% of gestational trophoblastic neoplasms
o Neoplastic proliferation of extravillous trophoblasts (Intermediate trophoblasts)
Clinical:
- Uterine mass - Either abnormal uterine bleeding or ammenorrheoa - Moderately elevated bHCG - May occur 2 weeks to 14 years following the gestation
Pathology:
- Malignant trophoblastic cells diffusely infiltrating the myometrium - May follow a normal pregnancy (50%), spontaneous abortion or molar pregnancy
Prognosis:
- Good for localized disease
- 10-15% die of disseminated disease
ANSWER: Placental site trophoblastic tumour can occur greater than two years following a normal pregnancy
Which is not associated with pre-eclampsia? (March 2017)
a. Hydatiform mole
b. Placental infarcts
c. Retroplacental haemorrhage
d. Maternal glomerulonephritis
e. HELLP syndrome
Pre-eclampsia:
- Widespread endothelial dysfunction which presents w HTN, oedema & proteinuria
- Eclampsia: severe illness with CNS involvement
Epidemiology: - 3-5% of pregnancies - More common in primigravid women - Increased risk with women carrying molar pregnancies - Usually begins in the 3rd trimester (34 weeks) - Earlier onset in women with: • Pre-existing renal disease • HTN • Coagulopathies
Maternal complications from systemic endothelial dysfunction: Hypercoagulability Acute renal failure Pulmonary oedema HELLP syndrome (10%) Eclampsia – convulsions
Pathophysiology:
- VEGF; PgI2
- Abnormal placental vasculature: failure of remodeling of the spiral arteries
- Endothelial dysfunction & imbalance in the circulating angiogenic & anti-angiogenic factors
- Inappropriate release of factors in response to placental hypoxia - Coagulation abnormalities: thrombi develop in capillaries and arterioles
- Organs most commonly affected: liver, kidney, brain, pituitary
Placental pathology:
- Infarcts: larger & more numerous than normal term placentas
- Exaggerated ischaemic changes (increased syncytial knots)
- Frequent retroplacental haematomas
• Reflect the instability of the uteroplacental vessels
- Abnormal decidual vessels
• Thrombi
• Lack of physiological conversion
• Fibrinoid necrosis
• Intraintimal lipid deposition
Management:
- Delivery – symptoms usually resolve in 1-2 weeks
- Anti-hypertensive medications do not alter the disease outcomes
o 20% of women develop non-pregnancy related HTN w/in 7 years
ANSWER: Maternal glomerulonephritis is not assoc w pre-eclampsia. Hydatiform mole increases the risk. Placental infarcts & retroplacental haemorrhage are pathological hallmarks. HELLP syndrome is a complication.
Complications of pre-eclampsia (March 2015)
a. Thrombocytosis
b. Pulmonary haemorrhage
c. Hypofibrinogen
d. Renal papillary necrosis
- Eclampsia: hyperreflexia and convulsions
- HELLP syndrome:
• Haemolysis, elevated liver enzymes, low platelets
• Where subclinical hepatic disease is the primary manifestation
Complications: o DIC o Hepatic infarction o Hepatic haematoma o Hepatic rupture o Placental abruption - Haemorrhagic or ischaemic stroke - Liver injury - Acute kidney injury – renal cortical necrosis
- Pregnancy related complications including placental abruption, chorioamnionitis (in the setting of foetal death) and severe eclampsia account for 50% of cases of renal cortical necrosis
- ARDS - Lung complications related to pulmonary oedema (not typically pulmonary haemorrhage)
Foetal complications:
• Growth restriction
• Foetal or perinatal death
ANSWER: None are correct – there is thymbocytopaenia in HELLP syndrome, pulmonary oedema – not pulmonary haemorrhage, higher levels of fibrinogen in pre-eclampsia patients and renal cortical necrosis rather than papillary necrosis
