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Intraabdominal desmoplastic small round cell tumour.

L Mingo1, F Seguel, V Rollán

  • 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
PubMed
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.

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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.

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  • 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.