Resection of a large, ruptured, undifferentiated (embryonal) sarcoma of the liver in a child: a case report and review of the literature.

David C. Yu, Ravi Tandon, Angela K. Bohlke, Rodney B. Steiner, Salima Haque, Sander S. Florman

Research output: Contribution to journalArticlepeer-review

3 Scopus citations

Abstract

BACKGROUND: Undifferentiated (embryonal) sarcoma (UES) of the liver is a malignant hepatic neoplasm accounting for 7% of pediatric hepatic tumors. Current use of multimodal therapy, including chemotherapy and surgery, has greatly improved survival. Tumor rupture is uncommon and, prior to the adjuvant use of sarcoma based chemotherapy regimens, was thought to be poor prognostic sign. CASE PRESENTATION AND DISCUSSION: A 10-year-old girl presented with acute worsening of abdominal pain while being worked up for liver mass. At surgery, she was found to have a ruptured, 20 x 15 x 5 centimeter (cm) UES, the largest reported ruptured UES to be resected primarily. The size and position of her tumor required very aggressive surgery for complete resection. She subsequently received adjuvant chemotherapy and radiation. Twenty-four months following surgery she is doing well with no evidence of disease. CONCLUSION: Despite tumor rupture, complete tumor resection gives the patient the best chance of long-term survival. Aggressive surgery is warranted if the tumor can be completely resected.

Original languageEnglish
Pages (from-to)41-44
Number of pages4
JournalThe Journal of the Louisiana State Medical Society : official organ of the Louisiana State Medical Society
Volume161
Issue number1
StatePublished - 2009
Externally publishedYes

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