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Gyn Onc > Ovary - Non epithelial > Flashcards

Ovary - Non epithelial Flashcards

1
Q

Name 10 Ovarian stromal tumours?

A
  • Fibroma
  • Cellular fibroma
    Thecoma
  • Luteinized thecoma associated with sclerosing peritonitis
  • Fibrosarcoma
  • Sclerosing stromal tumor
  • Signet-ring stromal tumor
  • Microcystic stromal tumor
  • Leydig cell tumor
  • Steroid cell tumor
  • Steroid cell tumor, malignant
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2
Q

Name 4 pure sex cord tumours?

A
  1. Adult granulosa cell tumor
  2. Juvenile granulosa cell tumor
  3. Sertoli cell tumor
  4. Sex cord tumor with annular tubules
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3
Q

Name 8 germ cell tumours and the Mnemonic

A 
TIDY Semen (CMEM)
T - teratocarcinomna
I - Immature teratoma
D - Dysgerminaoma
Y - Yolk Sac

C - Choriocarcinoma
M - Mixed germ cell tumour
E - Endodermal sinus tumour
M - Mature teratoma with malignant transformation

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4
Q

Name 7 - Monodermal teratoma and somatic-type tumors arising from a dermoid cyst

A
  1. Struma ovarii, benign
  2. Struma ovarii, malignant
  3. Carcinoid
    - Strumal carcinoid
    - Mucinous carcinoid
  4. Neuroectodermal-type tumors
  5. Sebaceous tumors
    - Sebaceous adenoma
    - Sebaceous carcinoma
  6. Other rare monodermal teratomas
  7. Carcinomas
    - Squamous cell carcinoma
    - Others
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5
Q

What % of benign ovarian neoplasms are Benign ovarian sex cord stromal tumours?

A

SCSTs account for <4 percent of ovarian benign neoplasms

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6
Q

What % of malignant ovarian neoplasms

are sex cord stroma tumours?

A

malignant ovarian SCSTs account for <8 % of ovarian malignant neoplasm

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7
Q

What is the incidence of sex cord stromal tumours?

A

0.2 : 100 000 (US)

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8
Q

What is the avg age at diagnosis of ovarian sex cord stromal tumours?

A

50

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9
Q

What % of germ cell tumours are in women less than 30?

A

12%

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10
Q

In women with granulosa cell tumours what % will have endometrial hyperplasia and what % will have carcinoma?

A

25 - 50% will have endometrial hyperplasia/intraepithelial neoplasia
5 to 10 % will have carcinoma

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11
Q

In patients with thecoma what % will have endometrial hyperplasia and what % will have carcinoma?

A

Endometrial hyperplasia in 15% and Carcinoma present in 20 - 25%

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12
Q

What % of sex cord stromal tumoru present with disease 1. confined to the ovary, 2. spread to surrounding organs or tissues, 3. distant metastases/

A
  • confined to the ovary 57 %
    spread to surrounding organs or tissues - 15 %
    and distant metastases 22%
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13
Q

Fibromas are the most common of the benign SCSTs and account for what % of all ovarian Neoplasms?

A

4%

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14
Q

Describe the histological features of fibromas

A

Fibromas are made almost entirely of fibroblasts.
Cellular fibromas are a type of fibroma characterized by:
- mildly increased cellular density,
- mild nuclear atypia, and an
- average of three or fewer mitotic figures per 10 high power fields (HPF)

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15
Q

What is Gorlin syndrome?

A

Gorlin Syndrome, also known as nevoid basal cell carcinoma cyndrome.
Affects 1: 31,000. Most commonly presents with BCC’s but increased risk of Ovarian fibromas.
Mutations in the PTCH1 gene are the main cause Gorlin syndrome.

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16
Q

Describe the microscopic appearance of thecomas

A
  • composed of theca cells.
  • may contain granulosa cell components; called granulosa-theca cell tumors
  • If granulosa cells predominate, then they are considered granulosa cell tumors.
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17
Q

Granulosa cell tumours are the most common type of potentially malignant SCST and compromise what % of malignant ovarian neoplasms?

A

2 - 5%

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18
Q

What % of malignant SCST are granulosa cell tumours?

A

90%

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19
Q

Describe the histological appearance of adult granulosa cell tumours?

A

Contain small, bland, cuboidal (to polygonal) cells with scant cytoplasm and pale, uniform and usually grooved nuclei (coffee bean). Mitotic activity is usually not brisk ( < 3 / 10 HPF)

  • Hypervascular stroma with vairable amounts of fibroblasts and theca cells
  • cells may arrange themselves in small clusters or rosette around a central cavity - termed call exner bodies.
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20
Q

When does a thecoma or fibroma with sex cord elements become categorised as a granulosa cell tumour?

A
  • need 10% granulosa cells to be classified as adult granulosa cell tumor, otherwise best classified as thecoma or fibroma with minor sex cord element
21
Q

What is a call-exner body?

A

Are small eosinophilic fluid filled punched out spaced between granulosa cells. The granulosa cells are usually arranged haphazardly around the space. They are pathognomonic for granulosa cell tumours.

22
Q

What % of granulosa cell tumours are adult and juvenile?

A

95% adult and 5 % Juvenile.

23
Q

? age range for germ cell tumours

A

10 - 30

24
Q

What % of Ov Ca in women 10 - 30 are germ cell

A

70%

25
Q

Name 8 types of germ cell malignancy

A 
Tidy Semen
TIDY CMEM
Teratocarcinoma - 
Immature Teratoma - 
Dysgerminoma
Yolk sac / endodermal sinus
Choriocarcinoma
Mixed germ cell
Embryonal
Mature teratoma with malignant degeneration
26
Q

Rank from most to least common 8 germ cell tumours

A 
Immature Teratoma - 35.5%
Dysgerminoma - 32%
Yolk sac / endodermal sinus 14.5%
Mixed germ cell - 5.3%
Embryonal  4.1%
Mature teratoma with malignant degeneration 2.9%
Teratocarcinoma 2.6 %
Choriocarcinoma 2.1%
27
Q

In advanced Ca which has a poorer prognosis and what is the HR - Serous or clear cell

A

Clear cell - HR 2.3 for survival

28
Q

After recurrence what is the 5yr OS for clear cell vs serous ca

A

Serous 18.2% vs clear cell 13.2 %

29
Q

What are the two most common mutations in clear cell carcinoma?

A

ARID1A = present in ~50% and PIK3CA also present in about 50%

30
Q

What % of clear cell carcinoma have a mutation in ARID1A?

A

~50%

31
Q

What % of clear cell carcinoma have a mutation in PIK3CA?

A

~50%

32
Q

What is the most common germ cell tumour

A

Mature teratoma ~ 25 % of all benign ovarian tumours

33
Q

What is the likelihood that a dermoid will develop malignant transformation

A

0.2 - 2 %

34
Q

What are the RF for malignant transformation of a mature teratoma

A

Age over 45
Greater than 10cm
rapid growth
Low resistance intratumor flow on Doppler.

35
Q

Which is the most common Ca that develops within a mature teratoma

A

SCC most common. also AdenoCa, Melanoma and sarcoma, thyroid carcinoma, basal cell carcinoma.

36
Q

Symptoms of Carcinoid syndrome - name 5

A

Flushing - normally of head and upper thorax
Diarrhoea
Abdominal pain
Bronchoconstriction
Carcinoid heard disease - Serotonin induced fibrosis in R heart valves.

37
Q

Fried egg cells on histo commonly refer to

A

Dysgerminoma - large vesicular cells with clear cytoplasm, well defined cell boundaries and centrally placed regular nuclei.

38
Q

What is a gonadoblastoma?

A

A benign or in-situ germ cell ovarian neoplasm composed of germ cells and sex cord stroma in phenotyptic females who have a Y chromosome. e.g. Purge gonadal dysgenesis 46XY, Mixed gonadal dystenedis, or CAI. Also turner syndrome.
Karyotype when found. 50% of gonaadoblastoma –> Ca.

39
Q

What is first and second line chemotherapy for Dysgerminoma

A 
1st like BEP
Bleomycin - 30,000 IU IV on Days 1,8* 15
Etoposide 100mg/m2 IV daily on days 1-5
Cisplatin 20mg /m2 IV daily on days 1-5
for 3 or 4 cycles depending on prognosis. with bone marrow support.
2nd Line TIP
Taxol, Ifosphamide, Cisplatin.
40
Q

Does adjuvant CT effect survival for Stage IC Germ cell tumours of the Ovary

A

MITO 9 included ~40 women with Stage IC - chemotherapy made no difference to OS
Recurrences were salvageable with OT/CT

41
Q

Is BSO and hysterectomy associated with improved survival in SCST?

A

In retrospective analysis fertility sparing was not associated with worse outcomes in women with stage I and II disease.

42
Q

What is the median time to recurrence of Granulosa cell tumours? What is the range.

A

Median time 4-6yrs. Can recur up to 30 years post diagnosis.

43
Q

What are RF for recurrence of Granulosa cell tumours

A 
Older age at disease onset
stage at diagnosis
Sub-optimal debulking surgery
tumour size greater than 5cm
Diffuse histologic pattern
44
Q

Which Sex cord stromal tumours produce hormones?

A

SCST an steroid cell tumours are a heterogenous group of tumours and vary in their capacity to produce clinically significant amounts of steroid hormones.

45
Q

Sertoli Leydig tumours typically secrete?

A

Androgen and there is clinical virilisaiton in 70-85% of women.

46
Q

In morphological ambiguous sex cord stromal tumors a panel of inhibin alpha, calretinin and FOXL2 can confirm the diagnosis of:

A

Adult granulosa cell tumour.

47
Q

What is the recommended treatment of Stage IA dysgerminoma? What is the rate of recurrence?

A

Surveillance is recommended.

The recurrence rate is between 15 - 25%.

48
Q

What are 3 histological differences between JGCT and AGCT

A

Juvenile has more mitosis, no coffee beans and larger more irregular follicles

49
Q

How are immature teratoma graded?

A

Based on the number of low-power microscopic fields containing immature neuroepithelium in any one slide. 1 LPF = Grade 1, 2 = Grade 2. 3 = Grade 3

Gyn Onc (23 decks)

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