Germ Cell/ scst

Question 1

What are germ cell ca?

Answer 1

Dysgerminoma, immature teratoma, yolks sack, embryonal

Question 2

And who can you observe and not get chemotherapy in germ cell?

Answer 2

Stage IA dysgerminoma and stage I, grade I immature teratoma

Question 2

What is growing teratoma syndrome?

Answer 2

Growing mets during chemo and normal tumor markers.

Question 3

Can you substitute carbo for Cisplatinum in peb?

Answer 3

Yes for stage II b - III dysgerminoma

Carbo auc 5 and etop paise 120 mg/m2 on days 1-3

Question 4

In whom do you offer fertility sparing for scst?

Answer 4

Stage 1a or 1c but you should surgically stage but lymphadenectomy can be omitted

Question 5

What is considered high risk features for scst ?

Answer 5

Rupture or stage IC, poorly differentiated, tumor size greater than 10-15 cm, consider chemo

Question 6

In whom do you offer fertility sparing for scst?

Answer 6

Star 1a or 1c but you should surgically stage but lymphadenectomy can be omitted, endometrial sampling for granulosa cell

Question 7

How to treat stage II - IV granulosa cell ?

Answer 7

Chemo with BEP or carb taxol or consider RT for limited disease, vac, bvp also

Question 8

What is the chance that granulosa cell tumors have an endometrial abnormality?

Answer 8

40% atypical hyperplasia and 5% with adenocarcinoma

Question 9

How do you treat recurrent granulosa cell?

Answer 9

Recurrences can occur even up to 30 years.

Surgery, if they haven’t seen carbotaxol or BEP then those, lupron,aromatase inhibitors, tamoxifen, avastin, megace

Question 10

Is bilateral disease common on granulosa cell?

Answer 10

No, 0-8%

Question 11

What is the relapse rate and avg time to recurrence? Survival rate?what is survival after relapse? What about stage III?

Answer 11

Relapse rate is 10-30% and the avg time to recurrence is 5-10 years. Survival is good 90% for stage I and 75-90% for all stages. The median survival after recurrence is 5.6 years. Stage III has a poor prognosis with survival of only 0-22%

Question 12

What is different about juvenile granulosa cell tumors?

Answer 12

They are less well differentiated and also have a high cure rate. BUT it is aggressive in advanced stage and you are quick to relapse and die within 3 years

Question 13

Sertoli Leydig: when do they present? symptoms? lab test?

Answer 13

2nd and 3rd decade. virilization: clitoromegaly, oligomenorrhea, deepening of the voice, acne, breast atrophy, hirsuitism. Labs: increase testosterone, androstenedione, and normal DHEA.

Question 14

how do juvenile granulosa cell present?

Answer 14

precocious puberty: breast enlargement, pubic hair, vaginal bleeding, advanced skeletal development.

Question 15

Are there syndromes a/w Juvenile GCT?

Answer 15

Ollier’s disease (enchondromatosis or with Maffucci’s syndrome - hemangioma). also been reported with leprechaunism. (characterized by rare insulin resistance)

Question 16

Sex cord tumor with annular tubules

Answer 16

in btw granulosa cell and sertoli leydig. Present with VB, If a/w with Peutz Jaeger Syndrome may present with intussusception secondary to colonic polyps. 15 % with adnoma malignum of cervix: which has high mortality

Question 17

Which germ cells patients would you recommend BEP?

Answer 17

Embryonal/endodermal sinus tumors(any stage)

stages II - IV dysgerminoma,

stage I, grade 2 or 3 or stage II to IV immature teratoma

Question 18

If a frozen section of germ cell is diagnosed, but there is obvious metastatic disease and the patient desires fertility, do you remove uterus and ovary?

Answer 18

No- Even those with bulky metastatic disease, outcomes are not compromised by fertility preserving surgery. 80% w adv dz will be long term survivors

Question 19

To perform the lymphadenectomy for germ cells?

Answer 19

Not necessary

Question 20

Do late relapses occur in germ cell?

Answer 20

The risk is greatest for dysgerminoma follow for at least 10 years

Question 21

What is the evidence for Nodal metastasis in granulosa cell tumors

Answer 21

MD Anderson 2008 showed that 50% of sex cord stromal tumors of the ovary had very low incidence of lymph node metastasis. Although only 52% had full staging, none had positive nodes. However in recurrent disease 5% had lymph node metastasis