Ovary - Non epithelial Flashcards
Name 10 Ovarian stromal tumours?
- 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
Name 4 pure sex cord tumours?
- Adult granulosa cell tumor
- Juvenile granulosa cell tumor
- Sertoli cell tumor
- Sex cord tumor with annular tubules
Name 8 germ cell tumours and the Mnemonic
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
Name 7 - Monodermal teratoma and somatic-type tumors arising from a dermoid cyst
- Struma ovarii, benign
- Struma ovarii, malignant
- Carcinoid
- Strumal carcinoid
- Mucinous carcinoid - Neuroectodermal-type tumors
- Sebaceous tumors
- Sebaceous adenoma
- Sebaceous carcinoma - Other rare monodermal teratomas
- Carcinomas
- Squamous cell carcinoma
- Others
What % of benign ovarian neoplasms are Benign ovarian sex cord stromal tumours?
SCSTs account for <4 percent of ovarian benign neoplasms
What % of malignant ovarian neoplasms
are sex cord stroma tumours?
malignant ovarian SCSTs account for <8 % of ovarian malignant neoplasm
What is the incidence of sex cord stromal tumours?
0.2 : 100 000 (US)
What is the avg age at diagnosis of ovarian sex cord stromal tumours?
50
What % of germ cell tumours are in women less than 30?
12%
In women with granulosa cell tumours what % will have endometrial hyperplasia and what % will have carcinoma?
25 - 50% will have endometrial hyperplasia/intraepithelial neoplasia
5 to 10 % will have carcinoma
In patients with thecoma what % will have endometrial hyperplasia and what % will have carcinoma?
Endometrial hyperplasia in 15% and Carcinoma present in 20 - 25%
What % of sex cord stromal tumoru present with disease 1. confined to the ovary, 2. spread to surrounding organs or tissues, 3. distant metastases/
- confined to the ovary 57 %
spread to surrounding organs or tissues - 15 %
and distant metastases 22%
Fibromas are the most common of the benign SCSTs and account for what % of all ovarian Neoplasms?
4%
Describe the histological features of fibromas
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)
What is Gorlin syndrome?
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.
Describe the microscopic appearance of thecomas
- 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.
Granulosa cell tumours are the most common type of potentially malignant SCST and compromise what % of malignant ovarian neoplasms?
2 - 5%
What % of malignant SCST are granulosa cell tumours?
90%
Describe the histological appearance of adult granulosa cell tumours?
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.
When does a thecoma or fibroma with sex cord elements become categorised as a granulosa cell tumour?
- need 10% granulosa cells to be classified as adult granulosa cell tumor, otherwise best classified as thecoma or fibroma with minor sex cord element
What is a call-exner body?
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.
What % of granulosa cell tumours are adult and juvenile?
95% adult and 5 % Juvenile.
? age range for germ cell tumours
10 - 30
What % of Ov Ca in women 10 - 30 are germ cell
70%
Name 8 types of germ cell malignancy
Tidy Semen TIDY CMEM Teratocarcinoma - Immature Teratoma - Dysgerminoma Yolk sac / endodermal sinus Choriocarcinoma Mixed germ cell Embryonal Mature teratoma with malignant degeneration
Rank from most to least common 8 germ cell tumours
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%
In advanced Ca which has a poorer prognosis and what is the HR - Serous or clear cell
Clear cell - HR 2.3 for survival
After recurrence what is the 5yr OS for clear cell vs serous ca
Serous 18.2% vs clear cell 13.2 %
What are the two most common mutations in clear cell carcinoma?
ARID1A = present in ~50% and PIK3CA also present in about 50%
What % of clear cell carcinoma have a mutation in ARID1A?
~50%
What % of clear cell carcinoma have a mutation in PIK3CA?
~50%
What is the most common germ cell tumour
Mature teratoma ~ 25 % of all benign ovarian tumours
What is the likelihood that a dermoid will develop malignant transformation
0.2 - 2 %
What are the RF for malignant transformation of a mature teratoma
Age over 45
Greater than 10cm
rapid growth
Low resistance intratumor flow on Doppler.
Which is the most common Ca that develops within a mature teratoma
SCC most common. also AdenoCa, Melanoma and sarcoma, thyroid carcinoma, basal cell carcinoma.
Symptoms of Carcinoid syndrome - name 5
Flushing - normally of head and upper thorax
Diarrhoea
Abdominal pain
Bronchoconstriction
Carcinoid heard disease - Serotonin induced fibrosis in R heart valves.
Fried egg cells on histo commonly refer to
Dysgerminoma - large vesicular cells with clear cytoplasm, well defined cell boundaries and centrally placed regular nuclei.
What is a gonadoblastoma?
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.
What is first and second line chemotherapy for Dysgerminoma
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.
Does adjuvant CT effect survival for Stage IC Germ cell tumours of the Ovary
MITO 9 included ~40 women with Stage IC - chemotherapy made no difference to OS
Recurrences were salvageable with OT/CT
Is BSO and hysterectomy associated with improved survival in SCST?
In retrospective analysis fertility sparing was not associated with worse outcomes in women with stage I and II disease.
What is the median time to recurrence of Granulosa cell tumours? What is the range.
Median time 4-6yrs. Can recur up to 30 years post diagnosis.
What are RF for recurrence of Granulosa cell tumours
Older age at disease onset stage at diagnosis Sub-optimal debulking surgery tumour size greater than 5cm Diffuse histologic pattern
Which Sex cord stromal tumours produce hormones?
SCST an steroid cell tumours are a heterogenous group of tumours and vary in their capacity to produce clinically significant amounts of steroid hormones.
Sertoli Leydig tumours typically secrete?
Androgen and there is clinical virilisaiton in 70-85% of women.
In morphological ambiguous sex cord stromal tumors a panel of inhibin alpha, calretinin and FOXL2 can confirm the diagnosis of:
Adult granulosa cell tumour.
What is the recommended treatment of Stage IA dysgerminoma? What is the rate of recurrence?
Surveillance is recommended.
The recurrence rate is between 15 - 25%.
What are 3 histological differences between JGCT and AGCT
Juvenile has more mitosis, no coffee beans and larger more irregular follicles
How are immature teratoma graded?
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