Carcinosarcoma of the Ovary

 
Question:
I need some advice from the GCIG community about management of this case of aggressive recurrence of Carcinosarcoma with predominant Rhabdomyosarcoma features post secondary debulking surgery.

37 year old woman who has recently been diagnosed with carcinosarcoma of the ovary who has been referred to PMH for assessment and treatment planning.

She presented to community  hospital with an acute abdomen in June 2010. US revealed a large pelvic mass (17cm by 15cm) and an emergency laparotomy was undertaken. Owing to extensive macroscopic disease the patient was referred to Gyne Oncologist for definitive surgery.

She underwent second surgery in 82010 and TAH, BSO, ileal disection, LAR and diverting loop ileostomy was performed. Disease involved the bowel serosa although surgical margins were negative.

Pathology revealed high grade ovarian carinosarcoma with  predominantly rhabdomyosarcomatous elements. This pathology was reviewed by gyne pathologist and  confirmed the diagnosis.

Post operative CT scans were done in 92010 and did not show evidence of disease recurrence.

Referred to PMH in November for opinion on systemic therapy.  Clinical assessment and  re-staging scans ( 19th Nov) however reveal a pelvic mass (10cm by 8cm) with necrotic elements which is most likely representative of recurrent disease in the opinion of the radiologist.  Clinically the patient is fully active and gaining in weight since her surgeries. Her only symptom is ongoing vaginal bleeding which is mild to moderate.

Questions:

In terms of systemic therapy, interested in what regimen should be considered for tumour is a carcinosarcoma but elements are predominatly rhabdomyosarcoma – would the recommendation be chemo regimen geared towards rhabdomyosarcoma or epithelial carcinosarcoma? Experience with use of radiation in this setting?

Dr. Amit M. Oza


 
 
Response # 1:  
Dear Amit:

I would favor surgical resection followed by radiation and chemotherapy.  Intraoperative RT may be a good option depending on the findings at the time of surgery.  Radiation therapy is a common modality for the treatment of GU rhabdomyosarcomas.  In the randomized trial by Nick Reed et al evaluating the use of post operative RT, the carcinosarcoma group appeared to derive a benefit on subset analysis.  I don’t know the optimal type of chemo to use in this setting and I don’t know of any data indicating a specific direction on how to sequence chemo and RT.

 
Kind Regards,

David K Gaffney, MD, PhD
 

 
Response # 2:  
I suggest our current regimen in advance stage carcinosarcoma of uterus or ovary. TIP (placlitaxel .ifosfamide, cisplatin) 6  cycles every 3 weeks

this regimen has been used before for locally advanced cervical cancer 8 neoadjuvant chemo) for details see buda a et al,  the SNAP Italian trial , on JCO.

quite toxic but the girl has a good PS and only 37 years.

our previous experience with PAC regimen (Signorelli et al,  international journal of gyn cancer 2008) was quite ineffective

what about a cytoreductive surgery before chemo (large mass!!!)

Mauro Signorelli, MD
 

 
Response # 3:  
Hi Amit
Would probably still offer carbo / taxol as epithelial tumours with heterologous elements. True sarcomas we would treat with Dox/Ifos.
Assuming there was disease outside the pelvis at diagnosis then I do not see a role for RT. Would consider RT if resistant to chemo and  surgically resectable and disease confined to pelvis at presentation.

Mary
 
Dr Mary McCormack PhD FRCR
 

 
Response # 4:  
I treat them with TEC, currently analysing  our results but some  long term survivors
 
No place for radiation in my  view
nick
 
Dr Nicholas REED
 
 
Response # 5:  
This is an evidence free zone, but in a fit 37 year old I would use a rhabdomyosarcoma regimen, based on what you have described.This is not a typical carcinosarcoma - do you know what sort of rhabdomyosarcoam it is- embryonal/aleveolar- was tissue sent for cytogenetics?. The high risk regimens include VAC /IA but in the past they included cisplatin as well but this was dropped as didn’t impact-however in a case like this I would consider trying to find that regimen- I would also consider post chem RT depending on response and whether disease localised. I would also discuss in the sarcoma MDT 

Regards Michael Friedlander
 

 
Response # 6:  
Confirm the progressive disease with eventual per cutaneous biopsy
And we have some long survivors with CAP (using anthracyclines with platinum) due to the rhabdomyosarcoma dedifferentiation, then surgical resection of residual disease then chemotherapy again…
Surprisingly we diagnosed 2 carcinosarcoma (with rhabdomyosarcoma dedifferentiation) from ovarian origin this month!
Kind regards
Isabelle
 
Isabelle Ray-Coquard, MD, PhD
 
 
Response # 7:  

This is a very interesting and challenging case, because of the nature of the unusual tumor and the treatment options as well.

I may suggest new surgery for debulking plus chemotherapy based on ifosfamide and cisplatinum, this approach based on the results on uterine carcinosarcomas.

For initial treatment I may not recommend radiotherapy because of the widespread nature of the disease.

Patient desire is the most important issue in a case like this, no more treatment might be a good option specially if the patient does not want more.

I would like to know what the final decision is, if possible.

Sincerely,

David Cantú MD MSc PhD