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Diffuse intraabdominal desmoplastic small round cell tumor: a ten-year experience.
E Livaditi1, G Mavridis, M Soutis
12nd Department of Pediatric Surgery, Aghia Sophia Children's Hospital, Athens, Greece.
Summary
Intraabdominal desmoplastic small round cell tumors (IDSRCT) are aggressive pediatric malignancies. Despite aggressive multimodal therapy including chemotherapy, surgery, and stem cell transplant, prognosis remains poor with frequent recurrence.
Area of Science:
- Pediatric Oncology
- Surgical Oncology
- Medical Oncology
Background:
- Intraabdominal desmoplastic small round cell tumors (IDSRCT) are rare pediatric malignancies, primarily affecting adolescent males.
- This study details the management of five pediatric cases with diffuse IDSRCT.
Observation:
- Patients presented with abdominal distention, pain, and vomiting.
- Treatment involved aggressive chemotherapy, surgery (biopsy, debulking, or radical excision), radiotherapy, and stem cell transplantation.
- Diagnosis was confirmed via immunohistochemistry and molecular analysis.
Findings:
- Tumor recurrence was common, occurring within months of initial treatment across all management strategies.
- Aggressive treatments including radical surgical excision, radiotherapy, and stem cell transplantation did not significantly improve long-term survival.
- The majority of patients (all but one) died within three years of diagnosis.
Implications:
- IDSRCT is a highly aggressive cancer with a dismal prognosis in children.
- Current therapeutic modalities offer limited long-term benefit, highlighting the need for novel treatment strategies.
- Surgical interventions are largely palliative in the context of this aggressive disease.