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
3252 - Genes involved in DNA replication, chromatin remodeling and cell cycle as potential biomarkers for therapy outcome to immune therapy in patients with metastatic cutaneous malignant melanoma
Presenter: Fernanda Costa Svedman
Session: Poster Display session 3
Resources:
Abstract
5545 - Phase Ib/II Study (SENSITIZE) assessing safety, pharmacokinetics (PK), pharmacodynamics (PD), and clinical outcome of domatinostat in combination with pembrolizumab in patients with advanced melanoma refractory/non-responding to prior checkpoint inhibitor therapy
Presenter: Jessica Hassel
Session: Poster Display session 3
Resources:
Abstract
5213 - Genomic landscape of primary malignant melanoma of esophagus
Presenter: Jie Dai
Session: Poster Display session 3
Resources:
Abstract
2716 - A phase III, randomised, double-blind study of adjuvant cemiplimab versus placebo post-surgery and radiation in patients with high-risk cutaneous squamous cell carcinoma (CSCC)
Presenter: Danny Rischin
Session: Poster Display session 3
Resources:
Abstract
3550 - ILLUMINATE 301: A randomized phase 3 study of tilsotolimod in combination with ipilimumab compared with ipilimumab alone in patients with advanced melanoma following progression on or after anti-PD-1 therapy
Presenter: Marcus Butler
Session: Poster Display session 3
Resources:
Abstract
1645 - PRIME002 - Early phase II study of Azacitidine and Carboplatin priming for Avelumab in patients with advanced melanoma who are resistant to immunotherapy
Presenter: Andre Van Der Westhuizen
Session: Poster Display session 3
Resources:
Abstract
4440 - Pembrolizumab (pembro) Plus Lenvatinib (len) for First-Line Treatment of patients (pts) With Advanced Melanoma: Phase 3 LEAP-003 Study
Presenter: Alexander Eggermont
Session: Poster Display session 3
Resources:
Abstract
3454 - Proof of concept study with the histone deacetylase inhibitor vorinostat in patients with resistant BRAFV600 mutated advanced melanoma
Presenter: Sanne Huijberts
Session: Poster Display session 3
Resources:
Abstract
1832 - A phase Ia/Ib clinical study to evaluate the safety, pharmacokinetics (PK) and preliminary anti-tumor activity of FCN-159 in patients with advanced melanoma harboring NRAS-aberrant (Ia) and NRAS-mutation (Ib).
Presenter: Lu Si
Session: Poster Display session 3
Resources:
Abstract
3996 - A Phase I Clinical Trial Investigating the Therapeutic Cancer Vaccine UV1 in Combination with Pembrolizumab as First-Line Treatment of Patients with Malignant Melanoma
Presenter: Sanjiv Agarwala
Session: Poster Display session 3
Resources:
Abstract