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Undifferentiated embryonal sarcoma in children: beware of the solitary liver cyst
S K Chowdhary1, A Trehan, A Das
1Department of Pediatric Surgery, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
Insights
Undifferentiated embryonal sarcoma (UES) of the liver is a rare pediatric tumor. Prompt diagnosis and chemotherapy followed by surgery can lead to successful outcomes and long-term remission in children.
Area of Science:
- Pediatric Oncology
- Hepatobiliary Malignancies
- Pediatric Surgical Oncology
Background:
- Undifferentiated embryonal sarcoma (UES) of the liver is an exceptionally rare liver tumor primarily affecting children.
- Its varied clinical presentation can complicate and delay diagnosis, posing a significant challenge in pediatric oncology.
Observation:
- This report details three consecutive pediatric cases of liver UES, all presenting with diagnostic challenges.
- Clinical presentations included a solitary cyst, severe abdominal pain, and intermittent fever, highlighting the atypical nature of UES.
Findings:
- Two of the three children received preoperative chemotherapy, which effectively reduced tumor size.
- This allowed for successful anatomic liver resection with clear margins, achieving tumor-free status.
Implications:
- The findings suggest that a multimodal approach involving chemotherapy and surgical resection is effective for liver UES.
- Long-term follow-up confirmed no recurrence in the treated patients 2 years post-therapy, indicating a favorable prognosis with timely intervention.
Abstract:
Undifferentiated embryonal sarcoma (UES) of liver is a rare form of liver tumor in children. It may have an atypical presentation leading to delay in diagnosis. The authors report on 3 consecutive children with this condition, each of them presenting in older children who presented with this condition and an initial diagnostic dilemma. One of them presented initially with a solitary cyst, the second with severe abdominal pain, and the third with intermittent fever. Preoperative chemotherapy was successful in shrinking the tumors in 2 children to allow anatomic liver resection with tumor-free margin. Both remain free of recurrence 2 years after completing treatment.