ependymoma case

[Reaganite]

30 something with grade 2 ependymoma s/p resection several years ago, ? enhancement on postop MRI, but no XRT offered. (Major academic center). Patient now has 2.5 cm recurrence and said center has recommended SRS only. New paradigm I'm unaware of?

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[radoncgrad2019]

I would SRS it

 

[evilbooyaa]

Is it resectable? I'll be honest and say that I'm not up to date on complicated ependymoma cases.

 

[medgator]

I would SRS it

Why not surgery? Several years out, could be both diagnostic and therapeutic

 

[radoncgrad2019]

Why not surgery? Several years out, could be both diagnostic and therapeutic

Good point. Was Assuming surgery said no. At an academic center if they are recommending SRS I’m assuming it came from NSurg to rad onc with plan for SRS

 

[Reaganite]

Good point. Was Assuming surgery said no. At an academic center if they are recommending SRS I’m assuming it came from NSurg to rad onc with plan for SRS

Yeah, no surg....nsg wants srs

 

[Palex80]

Re-resect and irradiate. I would opt for protons, keeping in mind age. You can spare relevant OARs better.

SRS is not a good idea. There is limited data on its value and even less in the recurrence scenario.
Look at this data:

Stereotactic Radiosurgery for Intracranial Ependymomas: An International Multicenter Study - PubMed

SRS provides another management option for residual or recurrent progressive intracranial ependymoma patients who have failed initial surgery and RT.

www.ncbi.nlm.nih.gov

44% 5-years-OS??? That's BAD.

The goal should be to cure this patient!

I am not sure why RT was not suggested at first occurence since it was a grade 2. It should have at least been discussed.

 

[Neuronix]

Where is it located?

Management of ependymoma is surgery. Long term control is predicated on degree of resection. The standard here is resection followed by fractionated RT.

Why is neurosurgery saying no? I would ask their reasons and if not convinced I would get second opinion. SRS alone is unlikely to control this long term.

 

[gmsquid]

Yeah location is a critical question here. Supra or infratentorial

 

[radiaterMike]

I don’t think the results of that paper apply. All patients had prior resection and prior RT. So the ‘poor’ results are for that subgroup of patients.

Re-resect and irradiate. I would opt for protons, keeping in mind age. You can spare relevant OARs better.

SRS is not a good idea. There is limited data on its value and even less in the recurrence scenario.
Look at this data:

Stereotactic Radiosurgery for Intracranial Ependymomas: An International Multicenter Study - PubMed

SRS provides another management option for residual or recurrent progressive intracranial ependymoma patients who have failed initial surgery and RT.

www.ncbi.nlm.nih.gov

44% 5-years-OS??? That's BAD.

The goal should be to cure this patient!

I am not sure why RT was not suggested at first occurence since it was a grade 2. It should have at least been discussed.

 

[thecarbonionangle]

Im not aware of good data for dose escalation for ependymoma. I woukd re-resect, thats always key. No amount of xrt or potency can make up for a bad resection

 

[Reaganite]

On the same page as all of you! Found the recs dating back to no XRT after initial surgery very strange, but don't see too many of these and this rec is coming from nsg at a major academic center. Of note, patient is only seeing me because insurance refused to cover gamma knife at said center. I suspect I'm the first rad onc patient has actually seen.

 

[xrthopeful]

lot of details in the case missing including location of original disease and location of current disease as well as resectabiity status. I would guess the academic center thought of a lot of this stuff before making a recommendation, but if you have concerns, a second opinion from a high volume center makes sense IMO.

on the original case - per the COG, for intracranial ependymomas, you don't offer adjuvant RT for grade II, so that doesn't seem crazy that he didn't get RT up-front.

Now for a local recurrence, of course if you can resect it you do. If you can't, radiation makes sense, and for something small and focal, you could make an argument for either a larger volume and fractionating, or just going after the gross disease and treating a small volume with fractionated SRS. The academic center leaned towards the latter it sounds.

Key is confirming the unresectability with the referring surgeon and if you don't believe him/her, send them to someone else. Aren't you in California? There is surely someone else you can ask for their neurosurgical opinion for resection.

 

[Palex80]

I don’t think the results of that paper apply. All patients had prior resection and prior RT. So the ‘poor’ results are for that subgroup of patients.

True, you are correct. This patient only had resection without RT.

 

[Palex80]

on the original case - per the COG, for intracranial ependymomas, you don't offer adjuvant RT for grade II, so that doesn't seem crazy that he didn't get RT up-front.

This is not the case in the European protocols.

We give RT to all patients with ependymoma.

Many patients are currently treated in a clinical protocol that tests chemotherapy after complete resection & RT.

ClinicalTrials.gov

clinicaltrials.gov

We would have advised for adjuvant RT in this patient after the first resection.

Only very small kids dont get RT immediately (<12 months).

From what I understand the US protocol is also prescribing RT to all patients with an infratentorial tumors post gross-total resection:

ACNS0831: Phase III Randomized Trial of Post-Radiation Chemotherapy in Patients with Newly Diagnosed Ependymoma Ages 1 to 21 years

CHILDREN'S ONCOLOGY GROUP -- The world's childhood cancer experts.

www.childrensoncologygroup.org

That would be Arm 2

 

[Neuronix]

Control was only 61% for observation for grade 2 supratentorial in the cog protocol. Conformal Radiation Therapy for Pediatric Ependymoma, Chemotherapy for Incompletely Resected Ependymoma, and Observation for Completely Resected, Supratentorial Ependymoma.

They should be radiated.

For supratentorial, that's another cautionary tale not to treat according to cog experimental arms. For infratentorial, adjuvant RT has not been in doubt.

 

[radiaterMike]

Not sure you can extrapolate results from peds protocols to adults. I agree that observation for subtotally resected low grade gliomas is not standard after updated RTOG 9802 results (though Ependymomas weren’t included and the trial really didn’t address timing of radiation). ‘Several years‘ ago (however long ago that might be) observation probably was an accepted standard though.

 

[Neuronix]

I treat a lot of young adults with peds tumors and it really annoys me to hear people say things like peds trials don't apply to 20s/30s year old patients. It's usually used to justify not doing standard of care.

9802 has nothing to do with ependymoma. Totally different disease.

 

[medgator]

I treat a lot of young adults with peds tumors and it really annoys me to hear people say things like peds trials don't apply to 20s/30s year old patients. It's usually used to justify not doing standard of care.

Agree. Not sure what other evidence people really have in those situations??

 

[Reaganite]

Sorry want to be careful and not give too many specifics, but it's an infratentorial lesion. I'm still waiting for their tumor board discussion, but the gist of their discussion appeared to be area too risky for surgery, will try SRS first, and then surgery if it fails. I don't want to assume anything since this is coming from a reputable institution. Just wondered if others are approaching these cases like this since my training was always adjuvant XRT esp if grade 2+, infratentorial, and possible STR. Was thinking maybe there was some chemo protocol patient was being considered for. Still waiting on these details.

 

[radiaterMike]

I treat a lot of young adults with peds tumors and it really annoys me to hear people say things like peds trials don't apply to 20s/30s year old patients. It's usually used to justify not doing standard of care.

9802 has nothing to do with ependymoma. Totally different disease.

Peds tumors are different though.
Outcomes are different (usually more favorable). Anyone who treats kids will tell you they are not little adults. Their cancers are different and their developing bodies are different. I agree that age is not a finite cut off and many young adults developed their tumors as children. I never said 9802 applied to epndymoma. My point was that observation for subtotally resected glioma fell out of favor after 9802. But at one time observation for subtotally resected low grade gliomas (including ependymoma) was considered acceptable.

 

[Neuronix]

Infratentorial should have been radiated from the start with fractionated RT as grade 2 regardless of str/gtr. Also the plan of SRS followed by surgery in case of recurrence is not good because marginal failure risk at the brainstem is high and then no room for fractionated RT to a decent sized field as is standard of care once the SRS brainstem dose has been maxed out.

Further, this will very likely fail and each recurrence carries risk of spinal metastases. This reminds me, has patient had LP and total spine MRI for this recurrence?

I don't know what they're thinking at the academic center, but I'm extremely concerned.

There is no chemo that works for ependymoma. There are protocols for patients who failed standard of care, but this patient has not (and apparently will not) received standard of care.

 

[xrthopeful]

yes if infratentorial should have gotten RT after GTR up front for sure.

I know you can't give all details - but is this recurrence a local recurrence at prior site of resection? Or distant intracranial failure?

 

[Reaganite]

yes if infratentorial should have gotten RT after GTR up front for sure.

I know you can't give all details - but is this recurrence a local recurrence at prior site of resection? Or distant intracranial failure?

Original site

 

[thecarbonionangle]

The chemo data for ependymoma is TERRIBLE. peds tumors different than adults. For example the peds equivalent of the Stupp trial was actually NEGATIVE.

 

[xrthopeful]

Original site

Got it. Then yes I favor fractionated RT to the entire original resection cavity plus margin at this point. He should have gotten it up front. could SRS boost the gross 'unresectable' disease.


luckily he doesn't have distant intracranial or spinal failure (i assume full workup has been done). This may still be salvageable

 

[Neuronix]

The chemo data for ependymoma is TERRIBLE. peds tumors different than adults. For example the peds equivalent of the Stupp trial was actually NEGATIVE.

Guess what--peds gbm still gets RT + tmz.

Show me data that adult intracranial ependymoma should be treated differently than pediatric ependymoma and I'll consider it. Small retrospective series is all you'll find.

If you want to make the generic argument that kids are not just little adults, you'll have to do better than that.

I deal with these tumors and surgeons who don't know the management all the time. It doesn't mean I'm going to do the wrong thing.

 

[thecarbonionangle]

Guess what--peds gbm still gets RT + tmz.

Show me data that adult intracranial ependymoma should be treated differently than pediatric ependymoma and I'll consider it. Small retrospective series is all you'll find.

If you want to make the generic argument that kids are not just little adults, you'll have to do better than that.

I deal with these tumors and surgeons who don't know the management all the time. It doesn't mean I'm going to do the wrong thing.

i have seen two institutions with different practice for peds gbm. One gave TMZ regardless and one did not give it.

 

[taserlaser]

i have seen two institutions with different practice for peds gbm. One gave TMZ regardless and one did not give it.

Have a peds low grade but molecular profile and behavior more consistent with a DIPG on treatment right now. She’s getting RT only and will save chemo for progression.

 

[Palex80]

Have a peds low grade but molecular profile and behavior more consistent with a DIPG on treatment right now. She’s getting RT only and will save chemo for progression.

Which is obviously fine.

 

[radiation]

The fact that there appears to be controversy in the discussed cases reinforces the need for more standardized peds rad onc training. For the ependymoma case, there should be an attempt for gross total resection, followed by adjuvant fractionated RT to post op bed to 54-59.4. Every prospective paper has shown lack of gross total resection to be the worst prognostic factor that cannot be made up with radiation.

There are no COG protocols that recommend withholding adjuvant radiation for infratentorial ependymoma. Supratentorial ependymoma is more controversial, with Arm 1 of ACNS0831 showing 50% recurrence, but 100% survival at 5 years (indicating successful salvage options), although there were only 11 kids in this arm. The current St Jude trial for recurrent ependymoma recommends repeat resection and full dose re-irradiation
RERTEP: Phase II Ependymoma Clinical Trial

Even if a GTR is not possible, newly diagnosed pts with a near total resection followed by radiation (as defined as residual tumor evident on postoperative neuroimaging with thickness or nodularity measuring 0.5 cm or smaller in the greatest dimension) seemed to do as well as those with a GTR + radiation in ACNS0831

The chemo data for ependymoma is TERRIBLE. peds tumors different than adults. For example the peds equivalent of the Stupp trial was actually NEGATIVE.

This is misleading, as there was never a "peds equivalent of Stupp trial". ACNS0126 was a single arm trial using Temodar that demonstrated worse survival than historical controls. There are a number of interpretations of why this trial did worse than historical controls, but the leading thoughts have been that there were likely more "true" GBMs in this trial as central pathology review of the older studies demonstrated significant downstaging to LGG.

CCG-943 established chemoradiation as standard of care in peds HGG, as PCV in this setting extended OS. Since that trial there have been no COG trials (past or future) that do not incorporate chemotherapy as it is deemed unethical.

The effectiveness of chemotherapy for treatment of high grade astrocytoma in children: results of a randomized trial. A report from the Childrens Cancer Study Group - PubMed

Fifty-eight patients with high-grade astrocytoma were treated by members of the Childrens Cancer Study Group in a prospective randomized trial designed to study the effectiveness of chemotherapy as an adjuvant to standard surgical treatment and radiotherapy. Following surgical therapy, patients...

www.ncbi.nlm.nih.gov

The newest COG study ACNS1721 incorporates a concurrent PARP inhibitor during radiation with adjuvant Temodar, indicating there is still belief Temodar has activity in this setting

ClinicalTrials.gov

clinicaltrials.gov

 

[Neuronix]

I'm not really sure where the controversy is here regarding standard of care, but thanks for expanding on what we've discussed.

Do you have specific thoughts on what should be done with the case presented? Do you agree or disagree with SRS in this setting?

 

[medgator]

I'm not really sure where the controversy is here regarding standard of care, but thanks for expanding on what we've discussed.

Do you have specific thoughts on what should be done with the case presented? Do you agree or disagree with SRS in this setting?

From what I gathered from that post, I am guessing an attempted resection would be preferred over srs, even if that ends up being a STR, followed by adjuvant fractionated xrt.

That's pretty much the case in every CNS malignancy I've ever seen, adult or peds.... Resection preferable, even if subtotal

 

[radiation]

I'm not really sure where the controversy is here regarding standard of care, but thanks for expanding on what we've discussed.

Do you have specific thoughts on what should be done with the case presented? Do you agree or disagree with SRS in this setting?

Sorry, these were the controversial posts I was referring to

on the original case - per the COG, for intracranial ependymomas, you don't offer adjuvant RT for grade II, so that doesn't seem crazy that he didn't get RT up-front.

i have seen two institutions with different practice for peds gbm. One gave TMZ regardless and one did not give it.

The chemo data for ependymoma is TERRIBLE. peds tumors different than adults. For example the peds equivalent of the Stupp trial was actually NEGATIVE.

Would not recommend SRS. Pt should get second opinion from NSG and at least attempt resection followed by adjuvant fractionated radiation. If one wanted to really interpolate that findings of ACNS0831 to this case, bridging chemo could be considered, as 56% achieved GTR on second look surgery after chemotherapy. It is unclear if chemo in this study actually had any effect of downstaging the tumor so that a GTR was possible, but most likely bought some time to facilitate surgical planning or get a second opinion to get access to a surgeon willing to do the second look operation.

 

[xrthopeful]

It has already been stated that if infra-tentorial then he surely should have gotten adjuvant RT up-front

It has also been said that a second or third opinion should be sought to ensure the resectability (or rather the unresectability)

 

[seper]

I'd go on that dreadful path of a second surgical opinion. Sorry you have to deal with case like this.

 

[Neuronix]

What ended up happening with the case? I'm curious.