Ewing Sarcoma of the ovary

Question:

25 year old woman, 0 para, median laparotomy with salpingoophorectomy on the right side, omentectomy and peritoneal biopsies (during surgery fresh frozen section: granulosa cell tumor).

Pathology report (including external reference pathology): Ewing sarcoma of the ovary.

Other biopsies were negative. She had complete staging including CT/MRI pelvis/abdomen/thorax/brain. Coloscopy and gastroscopy. Everything was negative.

Would you recommend further surgery? If yes, fertility sparing surgery including lymphadenectomy or radical staging surgery?

Systemic treatment?

Best regards,

Philipp Harter

 
Response # 1:  

Dear Philipp

I hope you are well.

If this is a true Ewing’s sarcoma, then systemic treatment is the most important next step and she should be referred immediately to a specialist sarcoma oncology team who will undertake this and do the necessary bone marrow staging etc.

Best wishes

Dearbhaile

 
Response # 2:  

Why isn't it a small cell carcinoma  of  ovary, much more likely

How can your pathologist be confident  it is Ewing's and  not a SCCOHT which would  be  more likely

Re further management, almost certainly needs  chemotherapy but if  operated  on by you can avoid further surgery.,radiation of  uncertain value

Need to see tumour markers  in serum and  full path report with icc

Interested to hear other views

Nick Reed

 
Response # 3:  

Major question :specific EWS translocation ? is a possible dedifferentiation from immature teratoma?

For treatment, if Ewing confirmed treatment recommended as EuroEwing trial (inclusion is recommended) is 6 cycles of VIDE local treatment (if residual disease surgery) and so 6 cycles of VAC as maintenance treatment

Nodes surgery is not recommended (for other localisation) and radical surgery probably not benefit (but no evidence data

PS  In our Center, those patient benefited of cryo conservative surgery of a part of the controlat ovary (due to the risk of sterility after 6 VIDE)

Kind regards

Isabelle Ray-Coquard

 
Response # 4:  

Dear Philip,

only case reports to help. I presume it was a intracapsular tumor, maybe arising in an immature teratoma (any sign of that). I would prefer to axamine the lymphnodes (so fertility sparing lymphadenectomy), and would only give adjuvant treatment if any metastasis is found., evidence is lacking completely, so of course this is an advise on gut feeling.

kind regards

Ruud Bekkers

 
Response # 5:  

Dear Philipp,

Further surgery is not required, though she should be treated according to EORTCs Ewing Sarcoma protocol, which means quite heavy adjuvant chemotherapy

Best wishes

Mansoor

Check under their sarcoma group

 
Response # 6:  

Dear Philipp,

I discussed this case with our sarcoma team and their recommendation was that she should not have further surgery but would require intensive chemotherapy on a Ewings sarcoma protocol.

Best wishes

Ros

 
Response # 7:  

Reply from the head of sarcoma clinic at The Norwegian Radium Hospital:

It is of utmost importance to give this patient systemic treatment with cytostatic drugs according to a total Ewing treatment protocol. In my opinion lymphadectomy should not be done.  The histological margins and the quality of the operation should be thoroughly discussed by the sarcoma multidisplinary team.  If the surgery has been radical with wide margins - no further local treatment.

 

With kind regards,

Kirsten Sundby Hall