During time period between June 2008 and June 2009, Children’s
Healthcare of Atlanta treated three children diagnosed with DIPG with radiation followed by temozolomide and avastin. Two
children were still alive at 37 and 47 months from diagnosis. The other child had progression free survival for 12 months followed by rapid deterioration with death at 14 months from diagnosis.
Here is the recent case report of these two children who have surpassed the 3-year mark since diagnosis of a DIPG and were still
going strong at the time of publication.
Patient 1- was described as an 11-year-old male with a 2
month history of weakness on the left side as well as walking and swallowing
difficulties. The initial MRI showed a
4cmx3.5cmx3cm non-enhancing tumor of the pons and medulla.
Patient 2-was described as an asymptomatic 7-year-old female
who was found to incidentally have a 3cmx2.3cmx2.6cm pontine mass after head
trauma. She was observed closely with
serial MRIs. Two MRIs showed increased
tumor size and at 8 months from initial presentation she deteriorated developing a facial palsy,
right sided weakness and hydrocephalus.
The tumor was 4.2cmx4.3x3.8cm with new focal areas of enhancement.
The children were treated with temozolomide 200mg/2d/d for 5
days ever 28 days and avastin 10mg.kg.dose every 14 days. In both patients a 65% decrease in tumor
size was seen. No steroids were needed
after 10 weeks from radiation. The
therapy was tolerated well. At
publication both children were doing well and continuing on treatment. The paper notes that the boy was going to
school and playing soccer at 37 months.
The authors speculate that the combination of temozolomide
and avastin might deliver more temozolamide to the tumor because avastin might
normalize the tumor vasculature. As
support for this theory the authors point to cilengtide altering perfusion
which allowed for increased temozolamide delivery for gliomas. Perhaps avastin has a similar effect.
An additional advantage of this combination might be the
decreased need for steroids.
The authors recommend consideration of an expanded DIPG
clinical trial with this combination.
By the way, the female child appears to be atypical both in
presentation and focality in her tumor.
On the other hand, the male child seems to have a very typical presentation
for DIPG.
Reference:
Prolonged survival after treatment of diffuse intrinsic pontine glioma with radiation, temozolamide, and bevacizumab: report of 2 cases.
J Pediatr Hematol Oncol. 2013 Jan;35(1):e42-6.
