Note: Some citations in the text of this section are followed by a level of evidence. The PDQ editorial boards use a formal ranking system to help the reader judge the strength of evidence linked to the reported results of a therapeutic strategy. (Refer to the PDQ summary on Levels of Evidence for more information.)
When doctors announced that Sen. Edward Kennedy had a kind of brain cancer called malignant
glioma, many people hearing the news had probably never heard of the cancer.
For some, however, the diagnosis was painfully familiar. WebMD talked to
three survivors of brain cancer similar to that affecting the senator,
including two who have survived it for more than 10 years. Their advice to
Kennedy: Don't listen to statistics, and don't give up hope.
Here are their stories:
Conventional treatment for children with diffuse intrinsic pontine glioma (DIPG) is radiation therapy to involved areas. Such treatment will result in transient benefit for most patients, but over 90% of patients will die within 18 months of diagnosis. The conventional dose of radiation therapy ranges between 54 Gy and 60 Gy given locally to the primary tumor site in single daily fractions.
Hyperfractionated (twice daily) radiation therapy techniques have been used to deliver a higher dose, and studies using doses as high as 78 Gy have been completed. Evidence demonstrates that these increased radiation therapy doses do not improve the duration or rate of survival for patients with DIPG whether given alone,[1,2] or in combination with chemotherapy. Studies evaluating the efficacy of various radiosensitizers as a means for enhancing the therapeutic effect of this modality have been undertaken but to date have failed to show any significant improvement in outcome.[2,3,4,5,6] Radiation-induced changes may occur a few months after the completion of radiation therapy and may mimic tumor progression. When considering the efficacy of additional treatment, care needs to be taken to separate radiation-induced change from progressive disease.
The utility of chemotherapy in the treatment of patients with newly diagnosed DIPG is unproven.[2,3,5,6,8,9,10]; [Level of evidence: 2A] Currently, no chemotherapeutic strategy-including neoadjuvant, concurrent, post-radiation survival or immunotherapy-when added to radiation therapy has led to long-term survival for children with DIPG.[12,13,14] Similarly, studies utilizing high-dose, marrow-ablative chemotherapy with autologous hematopoietic stem cell rescue have been ineffective in extending survival. Consonant with other brain tumors, radiation therapy is often omitted for infants with DIPG and chemotherapy-only approaches are utilized.
Treatment options under clinical evaluation
Studies using new anticancer agents with alternative mechanisms of actions and brain stem radiation are ongoing.
PBTC-030: The Pediatric Brain Tumor Consortium (PBTC) is conducting a phase II study of capecitabine and concomitant radiation therapy.
Focal or Low-grade Brain Stem Gliomas
In general, maximal surgical resection should be attempted.[16,17] Patients with residual tumors may be candidates for additional therapy including 3-dimensional conformal radiation therapy approaches with or without adjuvant chemotherapy. Information about ongoing clinical trials is available from the NCI Web site.
Patients with small tectal lesions and hydrocephalus but no other neurological deficits may be treated with cerebrospinal fluid diversion alone and have follow-up with sequential neuroradiographic studies unless there is evidence of progressive disease.