MPNST of the brachial plexus

Summary:

The role of adjuvant RT

MPNST of the brachial plexus

Indranil Mallick 15 Dec 2010 18:19

A 57 yr old lady presents with a supraclavicular swelling and some tingling near her left elbow.
MRI reveals a mass that is indistinguishable from the brachial plexus about 5x 5 cm in size
A neurosurgeon operates - with piecemeal excision. HPE reveals MPNST - high grade.
Post op MRI at 4 wks reveals a residual signal abnormality - about 2.5 x 3 cm. An independent surgical consult is taken - surgeons opine that this is likely some residual disease and some post-op change but repeat surgery is unlikely to clear the residual.
Patient is referred for post-op RT.
Question: What dose - given that there is R2 resection but this is on the brachial plexus. And is there any role of chemotherapy?

Re: MPNST of the brachial plexus

Nikhilesh Patil 15 Dec 2010 22:12

Dear Indranil,
Very interesting case. MPNST are bad sarcomas, they tend to come back with mets. Does she have NF-1 ?
As you are aware few HN and Breast protocols mention about Brachial Plexus Tolerance and some new protocols do define it as an avoidance structure, however I have not seen very concrete data, if there is then please update me.
This is one interesting article that you may want to read http://www.ncbi.nlm.nih.gov/pubmed/20580590.
For your patient I would give post-op rads max dose 60 Gy , conventional fractionation. If someone else chooses 50Gy, thats fine too. I will do 60 Gy becoz it is R2 resection and high grade histology.
Re:Chemotherapy: Doxorubicin and Ifos should be offered/discussed. Again we dont have great or Level-I evidence to do that. http://www.ncbi.nlm.nih.gov/pubmed/20838322
I know you are beyond case reports, your junior colleague may want to report this.

Re: MPNST of the brachial plexus

suman mallik 16 Dec 2010 05:40

Brachyal Plexus is one of the common sites of MPNST. Anyway the outcome is poor. Is there any neurodeficit? 50 to 60 Gy dose is reccommended. Two studies of large series of peripheral MPNSTs failed to show any benefit of chemotherapy. So chemotherapy may be added or may be considered at the time of recurrence.
[Gachiani J, Kim D, Nelson A, Kline D : Surgical management of malignant peripheral nerve sheath
tumors. Neurosurg Focus 2007,22(6):E13.
Chen L, Mao Y, Chen H, Zhou LF: Diagnosis and management of intracranial malignant peripheral nerve sheath tumors. Neurosurg 2008,62(4):825-832
Carli M, Ferrari A, Mattke A, Zanetti I, Casanova M, Bisogno G, Cecchetto G, Alaggio R, De Sio L,
Koscielniak E, Sotti G, Treuner J, Carli M, Ferrari A, Mattke A :Pediatric malignant peripheral nerve sheath
tumor: The Italian and German Soft Tissue Sarcoma Cooperative Group. J Clin Oncol 2005,23:8422-
8430.].

Re: MPNST of the brachial plexus

Nikhilesh Patil 16 Dec 2010 20:40

Thanks for enlightening me,I did not know brachial plexus is commonest sites. I havn't seen any. The series that you mentioned (Gachiani etal) had n=34 of which 19(56%) cases were brachial plexus and the Peads JCO paper had 60(40%) Extremities and 38(23%) Trunk wall but that is all Pediatric population. Chen et al is Intracranial MPNST.
I think the largest series that we are looking at has only n=34. I agree with Suman we dont know if the data is enough to recommend chemo or not. It should be discussed with the patient though. Will be happy to learn from our members if they have any experience with this tumor.
Indranil do let us know what you end up doing for your patient.

Last edited on 16 Dec 2010 20:42 by Nikhilesh Patil Show more