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


METHODS
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.


CONCLUSIONS
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.
Yu
David C
DC
Tulane University, New Orleans, Louisiana, USA.
Tandon
Ravi
R
Bohlke
Angela K
AK
Steiner
Rodney B
RB
Haque
Salima
S
Florman
Sander S
SS
D002454:Cell Differentiation; D002648:Child; D005260:Female; D006801:Humans; D009373:Neoplasms, Germ Cell and Embryonal / Q000000981:diagnostic imaging / Q000473:pathology / Q000601:surgery*; D011859:Radiography; D012196:Review Literature as Topic; D012509:Sarcoma / Q000473:pathology / Q000601:surgery*