Clinical features and surgical challenges of advanced retroperitoneal rhabdomyosarcoma in children: a single-center 17-year experience
Related Experiment Video
Updated: May 21, 2026
Establishment of Cancer Stem Cell Cultures from Human Conventional Osteosarcoma
Published on: October 14, 2016
Yu Lin1, Fenghao Xu1, Shen Yang1
1Department of Oncology Surgery, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China.
Background:
Advanced retroperitoneal rhabdomyosarcoma (RRMS) is a rare pediatric tumor characterized by insidious onset and a predilection for specific sites, with an extremely poor prognosis for affected children. This study aimed to investigate the clinical features, surgical challenges, and prognostic factors of advanced RRMS in children.
Methods:
A retrospective analysis of 17 children with advanced RRMS (2007-2024) was conducted. Tumor-node-metastasis (TNM) and Intergroup Rhabdomyosarcoma Study Group (IRSG) systems were used for staging; image‑defined risk factors (IDRFs) were used to evaluate invasion. Survival was analyzed via Kaplan-Meier and log-rank tests.
Results:
The median age at diagnosis was 54.0 months, with 14 males (82.4%). The most common presenting symptoms were abdominal pain and abdominal mass, and hydronephrosis was present in 37.5% of patients. A preoperative misdiagnosis rate of 41.2% was identified. According to staging systems, 7 patients were classified as stage III/Group III, and 10 as stage IV/Group IV, with lung and lymph node metastases being the most frequent sites of distant spread. Imaging studies revealed 16 tumors with no internal calcifications, and hydronephrosis was present in 35.3% of patients. Of 14 patients who received neoadjuvant chemotherapy, 8 achieved a partial response (PR). The median number of IDRFs was significantly higher in patients who died (n=12) than in survivors (n=5) (P=0.02). With a median follow-up duration of 117.0 months, the 3-year event-free survival (EFS) and overall survival (OS) rates were 27.5% and 32.1%, respectively. Tumor recurrence occurred in 6 patients, all of whom subsequently died of disease.
Conclusions:
Advanced RRMS has nonspecific symptoms, high misdiagnosis rate, and aggressive vascular invasion. Local surgical procedures present significant challenges, and IDRFs correlate with prognosis. Neoadjuvant chemotherapy is effective; multidisciplinary collaboration is critical for surgical management.
