Diagnostics and treatment of diffuse intrinsic pontine glioma: where do we stand?

INTRODUCTION: Diffuse intrinsic pontine glioma (DIPG) is a rare clinically, neuro-radiologically, and molecularly defined malignancy of the brainstem with a median overall survival of approximately 11 months. Our aim is to evaluate the current tendency for its treatment in Europe in order to develop (inter)national consensus guidelines. METHODS: Healthcare professionals specialized in DIPG were asked to fill in an online survey with questions regarding usual treatment strategies at diagnosis and at disease progression in their countries and/or their centers, respectively. RESULTS: Seventy-four healthcare professionals responded to the survey, of which 87.8% were pediatric oncologists. Only 13.5% of the respondents biopsy all of their patients, 41.9% biopsy their patients infrequently. More than half of the respondents (54.1%) treated their patients with radiotherapy only at diagnosis, whereas 44.6% preferred radiotherapy combined with chemotherapy. When the disease progresses, treatment strategies became even more diverse, and the tendency for no treatment increased from 1.4% at diagnosis to 77.0% after second progression. 36.5% of the healthcare professionals treat children younger than 3 years differently than older children at diagnosis. This percentage decreased, when the disease progresses. Most of the participants (51.4%) included less than 25% of their patients in clinical trials. CONCLUSION: This survey demonstrates a large heterogeneity of treatment regimens, especially at disease progression. We emphasize the need for international consensus guidelines for the treatment of DIPG, possible by more collaborative clinical trials.

Subependymal giant cell astrocytoma with atypical clinical and pathological features: a diagnostic pitfall.

Subependymal giant cell astrocytoma (SEGA) is benign, slowly growing tumor linked to the tuberous sclerosis complex. It almost always occurs near the foramen of Monro. Parenchymal extension and worrisome histological features, such as necrosis, mitoses, microvascular proliferation and pleomorphism are unusual in these tumors, but can occur rarely. A case of SEGA is presented, in a patient with no signs of tuberous sclerosis so far, with atypical imaging findings and areas of necrosis found microscopically. These worrisome features initially led to the false diagnosis of glioblastoma. The differential diagnosis of SEGA is discussed.