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[Mid-long term follow-up reports on head and neck rhabdomyosarcoma in children]
1Department of Medical Oncology, Pediatric Oncology Center,Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, National Key Clinical Discipline of Pediatric Oncology, Laboratory for Clinical Medicine, Capital Medical University, Key Laboratory of Major Diseases in Children, Ministry of Education, Beijing100045, China.
Insights
The China Children's Cancer Group Rhabdomyosarcoma 2016 (CCCG-RMS-2016) regimen shows improved outcomes for pediatric head and neck rhabdomyosarcoma (RMS), particularly in parameningeal cases. However, high recurrence rates and poor re-treatment effectiveness persist for parameningeal RMS.
Area of Science:
- Pediatric Oncology
- Rhabdomyosarcoma Research
- Clinical Trial Analysis
Context:
- Head and neck rhabdomyosarcoma (RMS) is a rare pediatric malignancy.
- Treatment strategies have evolved, necessitating updated efficacy analyses.
- Comparing different chemotherapy regimens is crucial for optimizing patient outcomes.
Purpose:
- To evaluate the clinical characteristics of pediatric head and neck RMS.
- To compare the mid-to-long term efficacy of the Beijing Children's Hospital Rhabdomyosarcoma 2006 (BCH-RMS-2006) and China Children's Cancer Group Rhabdomyosarcoma 2016 (CCCG-RMS-2016) regimens.
- To analyze treatment outcomes and recurrence patterns in pediatric head and neck RMS.
Summary:
- A retrospective study analyzed 137 children with newly diagnosed head and neck RMS.
- The CCCG-RMS-2016 regimen demonstrated a higher 5-year overall survival (OS) rate compared to the BCH-RMS-2006 regimen, especially in parameningeal and meningeal-risk groups.
- Orbital and non-orbital non-parameningeal RMS showed good prognosis, while parameningeal RMS exhibited high recurrence rates and poor re-treatment outcomes.
Impact:
- The CCCG-RMS-2016 regimen appears more effective for RMS in the meningeal region.
- Findings highlight the need for improved therapeutic strategies for recurrent parameningeal RMS.
- This study provides valuable data for refining treatment protocols for pediatric head and neck RMS.
Abstract:
Objective: To analyze the clinical characteristics of children with head and neck rhabdomyosarcoma (RMS) and to summarize the mid-long term efficacy of Beijing Children's Hospital Rhabdomyosarcoma 2006 (BCH-RMS-2006) regimen and China Children's Cancer Group Rhabdomyosarcoma 2016 (CCCG-RMS-2016) regimen. Methods: A retrospective cohort study. Clinical data of 137 children with newly diagnosed head and neck RMS at Beijing Children's Hospital, Capital Medical University from March 2013 to December 2021 were collected. Clinical characteristic of patients at disease onset and the therapeutic effects of patients treated with the BCH-RMS-2006 and CCCG-RMS-2016 regimens were compared. The treatments and outcomes of patients with recurrence were also summarized. Survival analysis was performed by Kaplan-Meier method, and Log-Rank test was used for comparison of survival rates between groups. Results: Among 137 patients, there were 80 males (58.4%) and 57 females (41.6%), the age of disease onset was 59 (34, 97) months. The primary site in the orbital, non-orbital non-parameningeal, and parameningeal area were 10 (7.3%), 47 (34.3%), and 80 (58.4%), respectively. Of all patients, 32 cases (23.4%) were treated with the BCH-RMS-2006 regimen and 105 (76.6%) cases were treated with the CCCG-RMS-2016 regimen. The follow-up time for the whole patients was 46 (20, 72) months, and the 5-year progression free survival (PFS) and overall survival (OS) rates for the whole patients were (60.4±4.4)% and (69.3±4.0)%, respectively. The 5-year OS rate was higher in the CCCG-RMS-2016 group than in BCH-RMS-2006 group ((73.0±4.5)% vs. (56.6±4.4)%, χ2=4.57,P=0.029). For the parameningeal group, the 5-year OS rate was higher in the CCCG-RMS-2016 group (61 cases) than in BCH-RMS-2006 group (19 cases) ((57.3±7.6)% vs. (32.7±11.8)%, χ2=4.64,P=0.031). For the group with meningeal invasion risk factors, the 5-year OS rate was higher in the CCCG-RMS-2016 group (54 cases) than in BCH-RMS-2006 group (15 cases) ((57.7±7.7)% vs. (30.0±12.3)%, χ2=4.76, P=0.029). Among the 10 cases of orbital RMS, there was no recurrence. In the non-orbital non-parameningeal RMS group (47 cases), there were 13 (27.6%) recurrences, after re-treatment, 7 cases survived. In the parameningeal RMS group (80 cases), there were 40 (50.0%) recurrences, with only 7 cases surviving after re-treatment. Conclusions: The overall prognosis for patients with orbital and non-orbital non-parameningeal RMS is good. However, children with parameningeal RMS have a high recurrence rate, and the effectiveness of re-treatment after recurrence is poor. Compared with the BCH-RMS-2006 regimen, the CCCG-RMS-2016 regimen can improve the treatment efficacy of RMS in the meningeal region.
