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Updated: Jul 29, 2025
Establishment of a Primary Culture of Patient-derived Soft Tissue Sarcoma
Published on: April 11, 2018
Intra-abdominal desmoplastic small round cell tumor: The European pediatric Soft tissue sarcoma Study Group (EpSSG)
Pablo Berlanga1, Daniel Orbach2, Reineke A Schoot3
1Department of Pediatric and Adolescent Oncology, Gustave-Roussy, Université Paris-Saclay, Villejuif, France.
Insights
Despite intensive multimodal treatment, outcomes for pediatric desmoplastic small round cell tumors (DSRCT) remain poor. Loco-regional treatment significantly impacts event-free survival in these rare abdominal cancers.
Area of Science:
- Pediatric Oncology
- Sarcoma Research
- Clinical Trial Analysis
Background:
- Focuses on intra-abdominal desmoplastic small round cell tumor (DSRCT) in pediatric and adolescent patients.
- Utilizes data from European pediatric Soft tissue sarcoma Study Group (EpSSG) protocols (BERNIE, EpSSG MTS 2008, EpSSG NRSTS 2005).
Purpose of the Study:
- To describe clinical findings and outcomes for pediatric DSRCT patients.
- To evaluate the effectiveness of multimodal treatment approaches in DSRCT.
Main Methods:
- Prospective enrollment of patients under 21 with abdominal DSRCT.
- Multimodal treatment including chemotherapy, surgery, and/or radiotherapy.
- Analysis of 32 cases with a median follow-up of 76 months.
Main Results:
- 32 patients analyzed (median age 13.7 years); 22 had extraperitoneal metastases.
- 5-year event-free survival (EFS) and overall survival (OS) were 19.7% and 21.0%, respectively.
- EFS was significantly worse for patients not receiving loco-regional treatment (p=0.007).
Conclusions:
- Outcomes for DSRCT remain dismal and have not improved despite intensive multimodal treatment.
- Loco-regional treatment is crucial for improving event-free survival in DSRCT patients.
Background:
This study describes the clinical findings of a consecutive series of pediatric and adolescent patients with a diagnosis of intra-abdominal desmoplastic small round cell tumor (DSRCT) prospectively enrolled in European pediatric Soft tissue sarcoma Study Group (EpSSG) protocols: the BERNIE study, the EpSSG MTS 2008 study, and the EpSSG NRSTS 2005 study.
Methods:
Patients aged less than 21 years with a diagnosis of DSRCT arising in the abdomen were included. All trials recommended a multimodal approach including intensive multidrug chemotherapy and loco-regional treatment with surgery and/or radiotherapy whenever possible.
Results:
The analysis included 32 cases (median age 13.7 years, male:female ratio 1.5:1). Three patients had localized tumors, seven had regionally disseminated disease, and 22 extraperitoneal metastases. All but one patient received multidrug chemotherapy and 11 had maintenance chemotherapy. Loco-regional treatment consisted of surgery only in seven cases, surgery plus adjuvant radiotherapy in 10, and radiotherapy only in six. Among the 17 cases who had radiotherapy, six had irradiation of the primary site, 10 had whole abdominopelvic radiotherapy plus boost to macroscopic residual disease, and one had irradiation to lung metastases only. With a median follow-up of 76 months (range: 18-124 months), 5-year event-free and overall survivals were 19.7% and 21.0%, respectively. Event-free survival was significantly worse for patients who did not receive loco-regional treatment (p-value .007).
Conclusions:
The study confirmed that the outcome of patients with DSRCT remains dismal and did not improve over recent years despite an intensive multimodal treatment approach.
