Angiosarcoma of the ovary

Question:
CASE:
A 23-year-old female presented to the emergency room for evaluation of severe abdominal pain lasting 1 week. On physical examination a large abdominal mass was palpated. CT Scan performed revealed a16X11cm mass with a cystic component, suspected arising from adnexa. There was no associated enlarged lymphanodes or ascitis. Laboratory tests showed only one abnormal result: Hb=9.4gr/dl. Tumor markers were as follows: CA125=25;CA19.9=4.9;AFP=0.8.

On May 25th  2009, the patient underwent exploratory laparotomy. The abdominal cavity showed a 14 x 10 cm left hemorrhagic adnexal mass which was involving sigma-rectum. A preliminary biopsy showed mesenchymal tumor so standard debulking surgery was performed including left bowel resection. Surgery was optimal without macroscopic residual disease.

Pathology examination revealed a   Grade 3 Angiosarcoma involving: left ovary (14x10cm); capsule, omentum ( 7cm nodule) , rectum, pelvic peritoneum.
Pelvic and Para Aortic Lymphanodes were negative: (0/9) and (0/17) respectively.
Microscopically : left ovary was replaced by hemorrhagic and necrotic tumor. The mitotitc rate was >30 mitotic figures per 10 high-power fields. Inmunohistochemical analysis revealed tumor cells strongly positive for Vimentin, CD31 and CD34.
Final Diagnosis: Angiosarcoma of the ovary, FIGO Stage IIIC; TNM: pT3C, pN0, M0

I saw the patient in my office on July 1st. She is doing extremely well. CT Scan post Op does not show any suspicious lesion and Blood test are OK.

I would very grateful if you can help me with the management of this young lady:
1.    Adjuvant chemotherapy?
2.    Which regimen?
3.    Role of PET/CT on follow-up?

Many thanks for your help in advanced
Best Regards,
Ana Oaknin


 
Response # 1:
I doubt if there is any published  evidence  based  literature on this, so you  have to use educated  guess work, and  look at anecdotal reports
 
I guess the choices are:
 
carboplatin and  paclitaxel
as above  plus  epirubicin (TEC)
TIP
 
personally i would  plump for the TEC as  it  is easy to use
 
And  i would  plan to give six cycles q 3 weeks
 
PET CT?? If you  have easy access do it, if  not  just stick to simple CT or MRI
 
Nick Reed

 

Response # 2:
Adjuvant chemotherapy based on ifosfamide, 10 g/m2 as a continuous infusion over 5 days, plus doxorubicin intravenously, 25 mg/m2/day for 3 days with Mesna and granulocyte-colony-stimulating factor every 21 days.

4 cycles

We used PET/on follow up only in case of suspicious CT imaging.

Best regards
Mauro Signorelli
 

Response # 3:
Largest ovary sarcoma series of which I am aware is an N of 31 there may have been no angiosarcoma in the specific series.

lst author TL Rutledge
Gyn Onc 2006; 100:128
suggested cis ifos had best results

If performance status, renal function, albumin  allow:
cis ifos

If not an ifos candidate
soft tissue sarcoma regimen of choice

All the best
Kathy Look