小児横紋筋肉腫における診断遅延と生存率：時間は重要な要因か？

Timely diagnosis is considered critical in pediatric oncology to optimize treatment outcomes, as delays may impact tumor extension and prognosis. We aimed to assess whether the time to diagnosis and treatment initiation for pediatric patients with rhabdomyosarcoma (RMS) improved over time in Italy and whether longer delays were associated with tumor extension and prognosis.

We analyzed 749 pediatric patients diagnosed with RMS between 1996 and 2016. Diagnostic interval (DI) was defined as the number of days from symptom onset to diagnosis, while treatment interval (TI) was defined as the time from symptom onset to treatment initiation. DI was correlated with tumor characteristics at diagnosis, and TI was analyzed in relation to survival, using Kaplan-Meier analysis.

The median DI was 32 days, showing a decreasing trend from 39.5 days in 1996 to 2000 to 30 days in 2011 to 2016. A longer DI was associated with age, unfavorable histology, and metastatic disease in univariate analysis, but these were not confirmed in multivariate analysis. The median TI was 48 days. Five-year event-free survival (EFS) and overall survival (OS) were 59.7% and 69.3%. In multivariate analysis, prognosis was negatively associated with age at diagnosis, unfavorable site, nodal involvement, and metastatic disease. TI was not associated with survival.

In our national cohort, the time from symptom onset to diagnosis showed a trend toward shortening in recent years. While a timely diagnosis can provide clarity on the child's condition and potentially reduce parental anxiety, it does not substantially impact tumor characteristics or patient outcomes.