RTHP-19. DIFFUSE INTRINSIC PONTINE GLIOMA AS A LATE COMPLICATION OF MEDULLOBLASTOMA THERAPY
Bibliographic record
Abstract
Radiation therapy has been associated with increased risk of secondary gliomas in survivors of childhood medulloblastoma. Despite this clear association, no studies have specifically addressed the risk of diffuse intrinsic pontine glioma (DIPG) after craniospinal irradiation in medulloblastoma survivors. We performed a systematic review of patients enrolled in the DIPG Registry and Repository as well as a literature review of studies reporting recent cooperative group medulloblastoma trials in order to identify cases of DIPG occurring after radiation therapy for pediatric medulloblastoma. Eleven cases of secondary DIPG in patients with primary medulloblastoma were identified from the DIPG Registry (n=5) and literature review (n=6). Patients were diagnosed with primary medulloblastoma between the ages of 2-9 years. All patients underwent surgical resection followed by craniospinal photon irradiation (range 18-36 Gy) and posterior fossa boost (range 19.8-36 Gy). For cases with dosimetric information available, mean brainstem exposure was 50.1-54.0 Gy. Median time to diagnosis of secondary DIPG was 7.4 years (range 2-11 years). Patients died of secondary DIPG a median of 8 months after diagnosis (range 4-17 months). We performed germline and tumor DNA and RNA sequencing of secondary DIPGs at autopsy, when available, which revealed no germline cancer predisposition syndromes and mutations associated with DIPG and non-brainstem high-grade glioma (TP53 loss, PTEN loss, and NRAS mutation). The incidence of DIPG as a secondary malignancy after medulloblastoma ranged from 0.3-2.6% between the involved institutions and reported studies. In conclusion, we report that survivors of pediatric medulloblastoma are at increased risk for the development of secondary DIPG. While treatment varied for the patients in our cohort, all cases resulted in death within 2 years of diagnosis. This risk highlights the importance of radiation field and modality in the treatment of children with medulloblastoma, and provides a compelling argument for efforts to reduce brainstem exposure.
Fetched live from OpenAlex and de-inverted. Abstracts are not stored in this database: the inverted indexes are 8.6 GB of the frame’s 9.3 GB of text, and the host has 13 GB free.
How this classification was reachedexpand
Full frame distilled prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. Learned from the 10,348 direct Codex labels and 10,348 direct Gemma labels. Candidate is the union of thresholded teacher heads; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels or direct frontier model labels.
Codex and Gemma teacher scores by category
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.000 | 0.000 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.001 | 0.000 |
| Bibliometrics | 0.000 | 0.000 |
| Science and technology studies | 0.000 | 0.000 |
| Scholarly communication | 0.000 | 0.000 |
| Open science | 0.000 | 0.000 |
| Research integrity | 0.000 | 0.000 |
| Insufficient payload (model declined to judge) | 0.000 | 0.000 |
Machine scores (provisional)
The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.
Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.
score_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from itClassification
machine, unvalidatedMachine predicted; a candidate call from one teacher head, not a consensus.
How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".