Related Experiment Videos
Intraabdominal desmoplastic small round cell tumour
1Service of Pediatric Surgery, Niño Jesús University Hospital, Avenida Menéndez Pelayo n 64, 28009 Madrid, Spain. ldemingo@wanadoo.es
Pediatric Surgery International
|March 12, 2005
Summary
Desmoplastic small round cell tumour (DSRCT) is a rare, aggressive cancer primarily affecting young males. This report details two pediatric cases, highlighting the tumor's high malignancy and poor prognosis despite aggressive treatment.
Area of Science:
- Oncology
- Pediatric Oncology
- Rare Cancers
Background:
- Desmoplastic small round cell tumour (DSRCT) is an exceptionally rare and aggressive neoplasm.
- It predominantly affects adolescent males and young adults, characterized by high malignancy and a poor 3-year survival rate of 29%.
Observation:
- This paper presents two pediatric cases of DSRCT, including a 4-year-old, the second youngest documented.
- Case 1: A 10-year-old boy with advanced DSRCT (Stage III) presented with abdominal pain and liver/retroperitoneal masses, succumbing to hepatic failure.
- Case 2: A 4-year-old boy with DSRCT experienced abdominal distension and an inguinal mass; despite chemotherapy and surgery, he died from peritoneal carcinomatosis.
Findings:
- Both pediatric cases demonstrated the aggressive nature and poor prognosis of DSRCT, with fatalities occurring due to disease progression and treatment complications.
- The youngest patient's presentation and outcome underscore the challenges of managing DSRCT in very young children.
Implications:
- The study emphasizes the critical need for more effective treatments for DSRCT, particularly in the pediatric population.
- Current recommendations include a multimodal approach combining chemotherapy, surgery, and radiotherapy, coupled with vigilant patient monitoring.