Ovarian tumor on a 7 year old

[burrango]

I am a father of a 7-year old girl that was diagnosed with an ovarian tumor. The tumor was resected and was mixed part dysgerminoma and part yolk sac tumor. The test on the liquid they got out of my daughter abdomen proved negative as did the epiplon sample they took. All good news but the problem is that they found no presence of any ovary tissues.

Two questions: 1) they are telling us that they need to perform a genetic test to see if the other ovary is ok because they might need to resect the other ovary. Is that correct? Should there be any ovary tissues in the tumor they resected?

2) The alfabetaproteins went down significantly after only 3 days from surgery (from 3950 to 1900). They want to start chemotherapy right away. Shouldn´t they wait longer given that the liquid and tissues tested negative to any presence of bad cells?

Thanks in advance,

Esteban

[Annekathryn Goodman, M.D.]

Dear Esteban,
thank you for your question
Your daughter has a rare tumor: mixed part dysgerminoma and part yolk sac tumor

Those cell types are part of a general group of ovarian tumors called "germ cell tumors'

Just as background, the ovary has three major tissue types:
-epithelial cells: these cover the ovary. Most tumors both benign and malignant come from this layer.
-sexcord stromal cells : these cells are the packing tissue of the ovary, they also produce hormones. malignant tumors from this layer also produce hormones  such as granulosa cell tumors, sertoli-leydig cell tumors.
they most commonly occur in women in their 50's

and finally germ cell tumors
these come from the egg cell of the ovary.
The most common germ cell tumor is the benign dermoid tumor or mature teratoma.
malignant germ cell tumors are extremely rare but are among the more common ovarian tumors seen in children and teenagers.

mixed part dysgerminoma and part yolk sac tumor:  these are both malignant tumor.Usually chemotherapy is given even if they are stage I.
I agree with Plonanon - you need to take your daughter to a major cancer center for this.

It maybe that no normal ovarian tissue was seen because the tumor competel replaced the ovary.

There is a rare condition where a little girl is actually genetically a boy but because of complex factors does not develop male secondary sexual characteristics.However, instead of ovaries, she has testicles that have not descended. When there is lack of descent of the testicle, there is a very high risk of malignant change.

The test I suspect her doctors are recommending is a genetic chromosome test to see if she has XX (female) chromosomes or XY (male) chromosomes.

I would just have the pathology of her tumor reviewed at a major center and have a consultation before doing any more intervention locally
please let us know what happens
best wishes

[Plonanon]

Sorry, I didn't realize this was the expert's forum. I thought it was the regular ovarian cancer forum. Perhaps you will get a reply from the doctor.

[Plonanon]

I am sorry about your daughter's medical problems. This must be really hard for you all.

My guess is that this is a very unusual case, and therefore, I would strongly recommend that you get a second opinion at a major cancer center such as Sloan Kettering in New York or MD Anderson in Texas. With an unusual case, most medical centers will not have the experience to know what the right course of treatment is.

A young doctor at Sloan Kettering whom I had occasion to speak to mentioned some rare cancer and told me he had already seen several cases of it because so many people with rare cancers go to Sloan Kettering.

I have read of a number of cases in which people were told they had to do extensive surgery and when they went to Sloan Kettering, were offered much less extensive surgery.

I would be very much surprised if there was anyone else on this forum who had knowledge of a similar situation, given how rare it is.

Good luck.