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A Mouse Model of Incompletely Resected Soft Tissue Sarcoma for Testing Neoadjuvant Therapies
Published on: July 28, 2020
Clinical features and outcomes of infantile soft-tissue sarcoma: A multicenter retrospective study in Beijing
1Department of Medical Oncology, Pediatric Oncology Center, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing Key Laboratory of Pediatric Hematology Oncology, Key Laboratory of Major Diseases in Children, Ministry of Education, Beijing, China.
Insights
Infant soft-tissue sarcomas are rare and diverse. This study analyzed 51 cases, finding that while multimodal therapy can control disease, standardized treatment protocols are crucial for improving outcomes in these challenging pediatric cancers.
Area of Science:
- Pediatric Oncology
- Cancer Research
- Rare Diseases
Background:
- Infant-onset soft-tissue sarcomas are rare and lack extensive study.
- This retrospective study addresses this gap by analyzing a cohort of infant sarcomas.
- Data was collected from a multi-institutional cohort in Beijing, China, over a decade.
Purpose of the Study:
- To review the clinical characteristics of infant-onset soft-tissue sarcomas.
- To analyze the treatments administered to these patients.
- To evaluate the outcomes of infant soft-tissue sarcomas.
Main Methods:
- Retrospective analysis of 51 patients diagnosed with soft-tissue sarcoma between 0-12 months.
- Data collected from four major children's hospitals in Beijing (2010-2019).
- Histological subtypes included rhabdomyosarcoma (RMS), extraosseous Ewing sarcoma (EES), and non-RMS soft-tissue sarcoma (NRSTS).
Main Results:
- Rhabdomyosarcoma (n=25), extraosseous Ewing sarcoma (n=6), and non-RMS soft-tissue sarcoma (n=20) were identified.
- Five-year event-free survival/overall survival (EFS/OS) rates varied by subtype: RMS (26.4%/56.2%), EES (50%), NRSTS (85.2%/100%).
- Abdominal/pelvic primary tumors in RMS and EWS indicated later-stage diagnosis and poorer prognosis.
Conclusions:
- Infant soft-tissue sarcoma is a heterogeneous group of cancers.
- Multimodal therapy demonstrated success in disease control for most patients.
- Standardized treatment protocols are recommended to improve care for these challenging pediatric conditions.
Background:
Soft-tissue sarcomas during infancy are rare and understudied. With no data on this specific condition, we performed a retrospective study of infant-onset sarcomas based on a multi-institutional cohort in Beijing, China, collected over the past decade. We reviewed infantile soft-tissue sarcomas' clinical characteristics, treatments, and outcomes.
Materials And Methods:
The patients with soft-tissue sarcoma diagnosed from 0 to 12 months in four primary children's hospitals in Beijing from January 2010 to December 2019 were evaluated.
Results:
Fifty-one patients were enrolled, including 31 males and 20 females. The median age at the diagnosis was five months (range, 0-12), and seven (13.7%) patients were diagnosed in the first month of their life. Histologically, twenty-five patients were diagnosed with rhabdomyosarcoma (RMS), six were diagnosed with extraosseous Ewing sarcoma (EES), and twenty were diagnosed with nonrhabdomyosarcoma soft-tissue sarcoma (NRSTS). The treatment principles and details of RMS focused on reference to the Intergroup Rhabdomyosarcoma Study Group (IRSG) protocols. For EES and NRSTS, chemotherapy was prescribed according to children's oncology group protocols. The five-year EFS/OS rates of RMS were 26.4% ± 19.5%/56.2 ± 17.8%, the five-year EFS/OS rate of EES was 50% ± 20.4%, and the five-year EFS/OS of NRSTS was 85.2% ± 9.8%/100%.
Conclusions:
Infant-onset soft-tissue sarcoma is heterogeneous. The primary location of the abdominal or pelvic cavity of RMS and EWS was at a later stage and had a poorer prognosis. Multimodal therapy resulted in successful disease control for the majority of patients. Standardization of treatment protocols will facilitate care for such challenging conditions.
