P.037 Disseminated leptomeningeal hemangioblastoma in a case of Von Hippel Lindau
Bibliographic record
Abstract
Background: Leptomeningeal dissemination of hemangioblastomas (HB), whether sporadic or associated with Von Hippel Lindau (VHL), are extremely rare. Very scanty literature is available. Methods: A 36 year old female with VHL and stable pancreatic, adrenal and renal lesions was operated upon 4 years ago for a large symptomatic cervicomedullary cystic and solid tumor. 2 years after surgery the tumour recurred and further removal was unsuccessful due to medullary adhesions. Radiation was given to the posterior fossa area and to several small nodules over the cauda equina resulting in severe pain. Serial follow up imaging revealed diffuse leptomeningeal dissemination increasing in size of the suprasellar region, ambient cistern and Sylvian fissures. Clinically, she has been stable with small dose of steroids and VP shunt insertion for papilledema. Results: Review of the literature consists of 2 series of 7 and 21 patients each with leptomeningeal dissemination involving sporadic HB and VHL associated HB. Leptomeningeal dissemination is estimated at about 4.3%. It is postulated that the tumour starts in the Pia and spreads in an extra medullary fashion throughout the subarachnoid spaces. Conclusions: Long term recurrence has been noted raising the question of aggressive treatment with some drug therapy related to angiogenesis is postulated.
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How this classification was reachedexpand
Full frame machine prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.
Distilled classifier scores by category (both heads)
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.000 | 0.002 |
| Meta-epidemiology (narrow) | 0.001 | 0.000 |
| Meta-epidemiology (broad) | 0.000 | 0.000 |
| Bibliometrics | 0.002 | 0.001 |
| Science and technology studies | 0.001 | 0.001 |
| Scholarly communication | 0.001 | 0.001 |
| Open science | 0.001 | 0.001 |
| Research integrity | 0.002 | 0.001 |
| Insufficient payload (model declined to judge) | 0.003 | 0.001 |
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 source (direct Gemma or distilled Codex), 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".