27yrs /M, Onset-06 months back ,swelling right neck,hoarseness voice-01 month,MRI-large parapharyngeal mass from base of skull ,destr of C2,encasement of vertebral art.Biopsy-MPNST(as per IHC).Inoperable.No other mets.
RT-how ,how much and with or without adjuvant/neoadjuvant ??
Intent of treatment: PALLIATIVE
(cannot cure this chap)
High dose Pall RT for symptom control is reasonable :- In view of young age, and if relatively good PS
Dose determined by what you think is safe :-
Organs at Risk: Brain Stem, Spinal Cord, Brain , Eyes, Pituitary etc
I hope you wont offer IMRT, but some simple 3DCRT.
(Understanding the limitation of newer technology and you ability to make a difference between radical doses and palliative doses)
PTV = 1.5 -2 cm margin
you may have to be tight at the spinal cord inferiorly
Max dose you can go for will be 48-50 Gy ( Spinal cord TD 5/5 ) - accepting a slightly higher risk.
We recently treated a Recurrent chordoma of the spine at D5-6 - we went upto 50 Gy, explaining the risk to the pt.
If you want to be more conservative and be safer settle for 46 Gy.
If you want chemo, in adjuvant setting —-
Single agent Doxorubicin gives response rates : 25-30%
Combination Chemo Doxorubicin + Ifosphamide : upto 40% response rate.
We usually go for the RT option first and then wait for him —-> to relapse locally or systemically
and then prefer to go for Palliative chemo option. (delayed chemo rather than offer them early).
Young pts go for combination
Older, less fit ones go for single agent.
Majority of pts fail / progress ( > 60%)
Second line chemo : Trabectidin (Yondelis) licensed for sarcomas
Cheers
Rohit
just to comment on the chemo regimes—- i agree with rohit abt not jumping in with chemo as there is no definite evidence for adjuvant upfront chemo.
first line - SA dox or Combination with Cis/Dox or Dox/Ifos.
second line - Gemcitabine/Docetaexel.
third line - Trabectadine - 10-15% RR but very poorly tolerated ( i have seen one pt being treated)
fourth line - can try temozolamide.
amar
As part of the workup,a DSA was done which has shown an aneurysm in the petrous region -contralateral to growth.i am planning 3-D CRT.What are your thoughts on embolisation or pre treatment debulking-R2 resection.
I ve got no idea on embolisation in sarcomas mate…… but must be good from a surgical aspect if this is a vascular tumour taking its feeds
from the vertebral artery which it encases.
R2 resection is as good as No surgery in terms of overall survival and DFS.
But the time interval from neurolgical free debility and /or symptom control and /or QOL may be better with debulking foll by Post op RT.
If your surgeon is offering it —-> i m sure he knows it why and for what :: seek his opinion as we do not have any surgeons in this isocentre.
Your surgeon would be able to quote the risks of this procedure.
As long as you are not desperate after second opinion from surgeons and pushing surgery for this patient yourslef, knowing what the
final outcome is and patient understands the risk and what R2 resection means.
Hi Hari,
Unfortunately Palliative situation for such a young man. I will give my best shot. RT alone and chemo (Adria) based. I will not offer surgery as nothing much to gain and many times incomplete resections may accelerate the growth of sarcoma.
Overall dismal outcome.
I agree.We have treated a similar patient last year,32 year old lady,with a large parapharyngeal sarcoma.We gave her radiation only.She discontinued treatment after 20 fractions.
The cytoreduction with radiation was remarkable.
With good cytireduction by radiation, adjuvant chemotherapy may be given.
Dr. Swarupa Mitra
Consultant Radiation Oncologist.
Artemis Hospital gurgaon
09812399708,9717041602