Abstract 1172
Background
Rhabdomyosarcoma (RMS) represents a malignant tumor of skeletal muscle origin, accounting for 5-10% of childhood cancers and more than 50% of pediatric soft tissue sarcoma. The head and neck occupied about 35% of the main sites of RMSs and most of these tumors are located in orbit, which contributes to 10% of all sites of RMSs. Due to the rarity of orbital RMS, the published studies of the clinical and pathological factors of orbital RMS could only provide limited information due to the small numbers of cohort or single institution. The aim of the current study was to evaluate the cumulative incidence of cancer-specific and competing risk death for patients with orbital RMS after surgery and build nomograms to predict survival based on a large population-based cohort.
Methods
Patients pathologically diagnosed with orbital RMS between 2004 and 2015 from the Surveillance, Epidemiology, and End Results (SEER) database were retrospectively collected. Nomograms for estimating overall survival (OS) and cancer-specific survival (CSS) were established based on Cox regression model and Fine and Grey’s model. The precision of the nomograms was evaluated and compared using concordance index (C-index) and the area under receiver operating characteristic (ROC) curve (AUC).
Results
A total of identified 217 patients with orbital RMS were retrospectively collected. The 10-, 20- and 40-year OS rates and cancer-specific mortality were 82.5%, 72.2% and 48.9%, respectively and 14.8%, 21.7% and 21.7%, respectively. The established nomograms were well calibrated and validated, with C-index of 0.901 and 0.944 for OS prediction, 0.923 and 0.904, for CSS prediction in the training and validation cohort, respectively. The values of AUC for 10-, 20-, and 40-year OS and CSS prediction were 0.908, 0.826 and 0.847, and 0.924, 0.863 and 0.863, respectively.
Conclusions
The survival rates were evaluated for patients with orbital RMS and the specialized nomograms were established for OS and CSS prediction for the first time, which showed relatively good performances and could be convenient individualized predictive tools for prognosis.
Clinical trial identification
Editorial acknowledgement
Legal entity responsible for the study
The authors.
Funding
This study was supported by the funding of Sun Yat-sen University Grant for Medical Humanities Practice and Teaching (No. 23000-18008023).
Disclosure
All authors have declared no conflicts of interest.
Resources from the same session
2600 - Atezolizumab (atezo) vs chemotherapy (chemo) in patients (pts) with platinum-treated locally advanced or metastatic urothelial carcinoma (mUC): a long-term overall survival (OS) and safety update from the Phase III IMvigor211 study
Presenter: Michiel Van der Heijden
Session: Poster Display session 3
Resources:
Abstract
3598 - Three-Year Follow-Up From the Phase 3 KEYNOTE-045 Trial: Pembrolizumab (Pembro) Versus Investigator’s Choice (Paclitaxel, Docetaxel, or Vinflunine) in Recurrent, Advanced Urothelial Cancer (UC)
Presenter: Andrea Necchi
Session: Poster Display session 3
Resources:
Abstract
2382 - First Report of Efficacy and Safety From a Phase 2 Trial of Tislelizumab, an Anti-PD-1 Antibody, for the Treatment of PD-L1+ Locally Advanced or Metastatic Urothelial Carcinoma (UC) in Asian Patients
Presenter: Dingwei Ye
Session: Poster Display session 3
Resources:
Abstract
2388 - Quality of Life of Metastatic Urothelial Cancer (mUC) Patients Treated with Enfortumab Vedotin (EV) Following Platinum-Containing Chemotherapy and a Checkpoint Inhibitor (CPI): Data from EV-201 Cohort 1
Presenter: Bradley McGregor
Session: Poster Display session 3
Resources:
Abstract
3748 - Safety and efficacy of atezolizumab (atezo) in patients (pts) with autoimmune disease (AID): subgroup analysis of the SAUL study in locally advanced/metastatic urinary tract carcinoma
Presenter: Yohann Loriot
Session: Poster Display session 3
Resources:
Abstract
1126 - Validation of the VIO prognostic index in patients with metastatic urothelial carcinoma treated with immune-checkpoint inhibitors
Presenter: Rafael Morales Barrera
Session: Poster Display session 3
Resources:
Abstract
3693 - Pathologic outcomes after neoadjuvant chemotherapy for high-risk muscle invasive bladder cancer
Presenter: Justin Matulay
Session: Poster Display session 3
Resources:
Abstract
4840 - Analysis of response to prior therapies and therapies after treatment with erdafitinib in fibroblast growth factor receptor (FGFR)-positive patients (pts) with metastatic urothelial carcinoma (mUC)
Presenter: Arlene Siefker-Radtke
Session: Poster Display session 3
Resources:
Abstract
1221 - Clinical outcomes by sex with atezolizumab (atezo) monotherapy in patients (pts) with locally advanced/metastatic urothelial carcinoma (mUC)
Presenter: Jean Hoffman-censits
Session: Poster Display session 3
Resources:
Abstract
1715 - National Small Cell Bladder Cancer Audit: Results from 26 UK institutions
Presenter: Caroline Chau
Session: Poster Display session 3
Resources:
Abstract