pediatric ependymoma

Summary:

1.5 year with ependymoma

pediatric ependymoma

HM 22 Feb 2010 15:07

one and a half year old girl,presented with left hemiparesis.MRI showed brainstem/CP angle ICSOL.Surgical decompression done on 12.2.10.Histopathology Anaplastic ependymoma.Now referred for RT.Awaiting postop MRI(not done in immediate postop period).Am planning CSF and only local RT if CSF negative upto 54 Gy (to be started in 2 nd week of March).Opinions/differences invited.

Re: pediatric ependymoma

kundan_25 22 Feb 2010 16:41

Girl less than 3 year —— Historically radiotherapy is to be avoided I presume and chemotherapy can be considered. But with innvations in the field of radiation (eg SRS, Cyberknife) I dont know what the current scenario is.

Chemo :-cisplatin, etoposide (VP-16), carboplatin, vincristine, and mechlorethamine, or ifosfamide, carboplatin, and etoposide (ICE), have been administered with variable success.

She is 1.5 year old girl and chances of neurocognitive impairment is maximum and also conventional chemotherapy has limited success. So currently there is lot of emphasis in subclassification of these tumors on the basis of molecular biology. hTERT negative tumors have 5 year survival of 84% verses positive tumors 41%. Thus indvidualized therapy can be considered for these patients.

Ref:
Tabori U, Ma J, Carter M, Zielenska M, Rutka J, Bouffet E, et al. Human telomere reverse transcriptase expression predicts progression and survival in pediatric intracranial ependymoma. J Clin Oncol. Apr 1 2006;24(10):1522-8. [Medline].

Sowar K, Straessle J, Donson AM, Handler M, Foreman NK. Predicting which children are at risk for ependymoma relapse. J Neurooncol. May 2006;78(1):41-6. [Medline].

Modena P, Lualdi E, Facchinetti F, Veltman J, Reid JF, Minardi S, et al. Identification of tumor-specific molecular signatures in intracranial ependymoma and association with clinical characteristics. J Clin Oncol. Nov 20 2006;24(33):5223-33. [Medline].

Re: pediatric ependymoma

abhinavahluwalia 23 Feb 2010 13:14

infratentorial Ependymoma surely need radiation no matter whats the grade or status of resection. dose is correct. only worrying factor is the age. I think Chemo will not be able to sustain relapse free interval for 1 and 1/2 yrs (if you think for postponing radiotherapy till 3 years age)… hence if general condition is good and CSF is clear…. consider conformal RT to the tumor bed with margin.

Re: pediatric ependymoma

HM 22 Feb 2010 18:00

If not RT then what?

1.Eric Bouffet , Uri Tabori , Ute Bartels .Paediatric ependymomas: should we avoid radiotherapy?The Lancet Oncology.August 2007; 8(8):665 - 666
2.Merchant TE, Li C, Xiong X, Kun LE, Boop FA, Sanford RA.Conformal radiotherapy after surgery for paediatric ependymoma: a prospective study.Lancet Oncol. 2009 Mar;10(3):258-66.
3.Shu HK, Sall WF, Maity A, et al.Childhood intracranial ependymoma: twenty-year experience from a single institution.Cancer. 2007 Jul 15;110(2):432-41.

Re: pediatric ependymoma

Srinivas Chilukuri 22 Feb 2010 19:17

There are two schools of thought here. The former more conventional say that to delay radiotherapy till 3 years, till then ICE, Mod.PCV regimens can be used. the other school say by delaying radiotherapy, we are actually putting children at risk of progression.

the compromise approach is to start chemotherapy and delay radiotherapy till atleast 2 years instead of 3 years. German HIT skk trials showed excess deaths in patients not recieving rasdiotherapy. I spoke to Deickman from Austria who is a co-investigator on this study couple of months back, and he was strongly biased towards early radotherapy instead of delaying or avoiding it. The chemo protocols they have used is a useful guide although.

Kundan can you suggest me any hospital in the world where hTERT gene studies can be performed commerically? I think there is a lot of gap between these studies and their use clinically. even established markers like MGMT are not commercially available in the country. The places where 1p19q can be studied, there is a long way to go in standardizing these procedures to bring in uniformity in reporting.

Re: pediatric ependymoma

kundan_25 23 Feb 2010 03:00

U r right Srinivas I am not suggesting u do these test. I was just sharing the information what the future is. As far as this case is concerned if we go for upfront RT we must use best possible RT resources to avoid doses to normal brain structure. And if we OPT for delay till 2 year or 3 year I dont know which is the best chemotherapy regimen to use.