The Pediatric Blood & Cancer journal has just released an interesting report about the use of a metronomic maintenance in children with pediatric diffuse intrinsic pontine gliomas (DIPGs). In the article entitled Radiation therapy and concurrent topotecan followed by maintenance triple anti-angiogenic therapy with thalidomide, etoposide, and celecoxib for pediatric diffuse intrinsic pontine glioma by Porkholm and al. the NOPHO group reports on 17% long term survivors (>24 months) and 4 CR among the 41 patients recruited in this study. Of note, metronomic topotecan was given during the radiotherapy as a radiosensitizer and was then followed by a metronomic maintenance with thalidomide, etoposide, and celecoxib. Globally the treatment was well tolerated with acceptable adverse effects and allowed a good quality of life. While no biopsies were performed to confirm diagnosis and to try to indentify patients who may benefit fomr this approach, this intersting report paves the way for introducing metronomic maintenance in DPIG.

You can read the abstract bellow and acces the full text article on the PBC website here.

Abstract

Background Despite major treatment attempts, the prognosis for pediatric diffuse intrinsic pontine gliomas (DIPGs) remains dismal. Gliomas are highly vascularized tumors, suggesting that the prevention of vessel formation by anti-angiogenic treatment might be effective.

Procedure Forty-one pediatric patients with DIPG were treated according to the Angiocomb protocol, starting with radiotherapy combined with topotecan and followed by anti-angiogenic triple medication consisting of thalidomide, etoposide, and celecoxib. Overall survival, radiological response, quality of life, requirement of corticosteroids, and adverse effects were monitored. Eight patients treated with only radiotherapy were used as controls.

Results For study patients, the 12 and 24 months overall survival was 61% and 17%, respectively. The median overall survival was 12 months (range 4–60 months). Four radiological complete responses were seen, of which two were transient. Radiologically, 56% of the tumors reduced in size and 78% in signal intensity. Study patients were able to visit school or daycare and walk for a significantly longer time compared to controls (Log Rank 0.036 and 0.008, respectively). Adverse effects were generally minor.

Conclusions The Angiocomb protocol created a noticeable share of long-term survivors and was well tolerated, suggesting that anti-angiogenic