Management of Malignant ovarian germ cell tumours

Question 1

What is the most common type of ovarian tumour in children and adolescents?

Answer 1

Germ cell

Question 2

What age due borderline ovarian tumours typically occur?

Answer 2

30-40 years old

Question 3

What age due epithelial ovarian tumours typically occur?

Answer 3

Over the age of 50

Question 4

Which type of germ cell ovarian tumour arises from undifferentiated cells?

Answer 4

Dysgerminoma (malignant), 45% of germ cell tumours

Question 5

Which type of germ cell ovarian tumours arise from the whole blastocyst?

Answer 5

Embryonal carcinoma (malignant), <5% of germ cell tumours

Question 6

Which type of germ cell ovarian tumours arise from embryologic tissue?

Answer 6

Teratomas – mature teratoma (dermoid) = benign, immature teratoma = malignant

Question 7

Which type of germ cell ovarian tumours arise from yolk sac tissue?

Answer 7

Yolk sac tumours = malignant, 20% of germ cell tumours

Question 8

Which type of germ cell ovarian tumour arise from trophoblastic tissue?

Answer 8

Choriocarcinoma = malignant, <1% of germ cell tumours

Question 9

From which cell lines do the following  malignant germ cell tumours arise? 
A) dysgerminoma 
B) embryonal carcinoma 
C) immature teratoma 
D) choriocarcinoma 
E) yolk sac tumour

Answer 9

A- undifferentiated germ cells (45%) 
B- Whole blastocyst cells (<5%) 
C- embryologic tissue (20% but includes mature teratoma) 
D- trophoplastic tissue (<1%) 
E- yolk sac tissue (20%)

Question 10

What is another name for a yolk sac germ cell tumour?

Answer 10

Endodermal sinus tumour

Question 11

Which tumour markers are expressed by choriocarcinoma of the ovary?

Answer 11

HCG

Question 12

Which tumour markers are expressed by yolk sac (endodermal sinus tumour) of the ovary?

Answer 12

AFP (sometimes LDH)

Question 13

Which tumour markers are expressed by dysgerminoma?

Answer 13

Sometimes HCG, sometimes LDH, never AFP

Question 14

Which tumour markers are expressed by immature teratomas?

Answer 14

Sometimes AFP, sometimes LDH, never HCG

Question 15

What is the half life of HCG?

Answer 15

1-2 days

Question 16

What is the half life of AFP?

Answer 16

5-7 days

Question 17

How do malignant ovarian germ cell tumours typically present?

Answer 17

Child/adolescent/young woman
Rapidly enlarging, unilateral ovarian mass
Acute pain due to rupture/torsion
May be menstrual/urinary or rectal symptoms
Typically stage 1 disease at presentation

Question 18

How common are bilateral dysgerminomas?

Answer 18

10-20% (malignant germ cell tumours typically present as unilateral)

Question 19

What is the overall survival rate for germ cell tumours of the ovary?

Answer 19

90%

Question 20

What is the overall survival rate for epithelial cell tumours of the ovary?

Answer 20

30%

Question 21

If a 15 year presents acutely with large ovarian mass suspicious for germ cell malignancy, which pre-operative tests are recommended?

Answer 21

AFP, LDH, HCG, Ca125
Group and save
Chest x-ray (choriocarcinoma is associated with lung mets)
+/- staging CT or MRI to assess if unclear that it is malignant

Question 22

How suspected stage 1 malignant germ cell tumours managed?

Answer 22

Surgery: Midline laparotomy, Oophorectomy (biopsy of contralateral ovary not recommended), Peritoneal washings, biopsies of omentum, peritoneum and suspicious lymph nodes for staging
Surveillance for 1A and 1B
Chemotherapy for 1C (sometimes also 1B)

Question 23

How are advanced staged malignant germ cell tumours?

Answer 23

Chemotherapy first then surgery

Question 24

How is tumour recurrence managed for patients with malignant germ cell tumours?

Answer 24

Surgical treatment is recommended followed by chemotherapy if histology confirms recurrence

Question 25

What is the follow up regimen for patients with Stage 1A germ cell ovarian cancer?

Answer 25

Do not require chemo
Require surveillance for 10 years (blood tests every 2 weeks for first 6 months, then monthly for next 6 months, then 2 monthly for 1 year, then 3 monthly for 1 year, then 4 monthly for 1 year, then 6 monthly)
MRI and chest xrays will also be performed intermittently
Patients advised not to fall pregnant for 2 years

Question 26

How are 1B and 1C stage malignant germ cell ovarian tumours managed?

Answer 26

Some centres will carry out surveillance on 1B after surgery
1C = Surgery then chemotherapy (BEP or POMB/ACE in high risk e.g. stage 3 or 4 disease)

Question 27

Which chemotherapy causes ototoxicity?

Answer 27

Cisplatin

Question 28

Which chemotherapy causes pneumotoxicity/lung fibrosis?

Answer 28

Bleomycin (this is dose related)

Question 29

hich chemotherapy causes a long term risk of leukaemia?

Answer 29

Etoposide

Question 30

After surgery for a malignant ovarian germ cell tumour, how long should patients wait until falling pregnant?

Answer 30

2 years as pregnancy makes surveillance results difficult to interpret

Question 31

What is the role of radiotherapy in the management of malignant ovarian germ cell tumours?

Answer 31

They are highly chemosensitive and radiotherapy can cause ovarian failure, risk of future malignancy and infertility, therefore it is reserved for palliative use

Question 32

What is the fertility rate in patients who have received chemotherapy for MOGCTs?

Answer 32

69-75% and there are no reported congenital abnormalities after treatment with chemotherapy