Parameningeal Rhabdomyosarcoma—Clinical Profile, Outcomes, and Prognostic Factors in Children Treated at a Single Center Over a Decade
Bibliographic record
Abstract
BACKGROUND: Parameningeal rhabdomyosarcomas (PM-RMS) in children is challenging to treat. While a 10-year event-free survival (EFS) of 62% has been reported from high-middle income countries (HMICs) for localized disease, data is limited from low-middle income countries (LMICs). We studied the clinical profile, outcomes, and prognostic factors in PM-RMS. MATERIALS AND METHODS: Children aged 15 years or younger with PM-RMS treated on a uniform chemotherapy protocol from January 2013 to December 2021 were retrospectively analyzed. Local therapy at 10 to 12 weeks of induction was radiotherapy (RT)+/-surgery, where possible, with early RT for intracranial extension (ICE). RESULTS: Seventy-six patients with a median age of 6.7 years (range: 3.2 to 15 y), male to female ratio of 1.8:1 formed the study cohort. Eleven patients (14.5%) had metastasis (lungs: 8, bone: 2, and bone marrow: 1) and ICE was seen in 46.1% (n=35). Twenty-five patients (49.0%) had alveolar histology with PAX3/7 positive in 17/59 (28.8%). The median tumor size (t size ) at baseline was 5.2 cm (range: 1.2 to 12.8 cm). Seventy-one patients received RT and 5 also underwent surgery. At a median follow-up of 65 months (range: 53 to 76 mo) 4 year EFS, OS of the whole cohort were 47.3% (95% CI: 34.8%-58.8%), 51.7% (95% CI: 38.0%-64.0%), respectively. Four-year EFS, OS of the localized and metastatic cohort were 54.7% (95% CI: 41.3%-68.1%), 56.0% (95% CI: 42.0%-70.0%), and 9.1% (95% CI: 0%-26.5%), 18.2% (95% CI: 0%-47.8%), respectively. Metastases (HR: 3.38, 95% CI: 1.57-7.26, and P =0.002), t size (HR: 1.17, 95% CI: 1.02-1.34, and P =0.026) were prognostic for survival on multivariate analysis. CONCLUSIONS: Survival of children with localized PM-RMS in our study is relatively fair compared with the reported literature probably due to the application of RT in all despite higher proportion of larger tumors, unfavorable sites of primary, and intracranial extension. Identification of high-risk subsets and optimizing current treatment strategies, both systemic and local therapy may partly improve outcomes.
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 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 | 0.001 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.000 | 0.000 |
| Bibliometrics | 0.001 | 0.001 |
| Science and technology studies | 0.001 | 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) | 0.001 | 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 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".