Undifferentiated (embryonal) sarcoma of the liver complicated with rupture in a child.
ABSTRACT Undifferentiated (embryonal) sarcoma of the liver (UESL) is a rare hepatic tumor and mainly occurs in children. It usually has cystic changes on computed tomography scan, which represents areas of necrosis and hemorrhages. UESL is a rapidly growing tumor and tumor rupture does occur. We reported a girl with a large multicystic hepatic tumor who died of tumor rupture shortly after the diagnosis of UESL. It is mandatory to be aware of this potentially fatal complication especially in patients with UESL of large tumor volume.
SourceAvailable from: Cecilia Amelia Fazzio Escanhoela[Show abstract] [Hide abstract]
ABSTRACT: Embryonal (undifferentiated) sarcoma is a primitive and unusual malignant neoplasm of the liver that occurs mainly in children between 6 and 10 years of age. The aim of this case report is to describe one case of this neoplasm, emphasizing clinical and anatomopathological findings as well as review the literature about the theme.Jornal Brasileiro de Patologia e Medicina Laboratorial 12/2007; 43(6):455-457.
Jornal Brasileiro de Patologia e Medicina Laboratorial 01/2007; 43(6). DOI:10.1590/S1676-24442007000600011
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ABSTRACT: Background: Outcome for hepatic undifferentiated embryonal sarcoma (HUES) is historically limited by persistent unresectable disease and subsequent development of disease resistance and dissemination. We present our institutional experience with HUES assessing current treatment trends and outcomes in the era of liver transplantation. Procedure: Retrospective chart review of cases presenting with HUES at our institution over the past ten years. Data collected included age, gender, presenting symptoms, imaging and associated PRETEXT score, pathology, chemotherapy, surgical intervention, and outcome. Approval was obtained by the Cincinnati Children's Hospital Medical Center Institutional Review Board. Results: HUES was identified in six patients (4 male and 2 female) with a median age at diagnosis of 10.7 years (range 7 to 13 years). Initial imaging was available in all but one patient. The PRETEXT stage in these patients ranged from II to III. One patient was diagnosed with lung metastases. Two patients underwent upfront resection and one patient received neoadjuvant therapy followed by conventional resection. Three patients were treated with orthotopic liver transplantation (OLT) following neoadjuvant chemotherapy, two by primary intent and one as salvage for local recurrence. Two received post-transplant adjuvant chemotherapy. All six patients remain in clinical remission with a median follow up of 35 months (12 to 84 months). Conclusions: OLT has rarely been reported as a treatment option for HUES. The addition of liver transplantation as a surgical option to treat patients with HUES results in improved survival in patients who are initially unresectable or recur. Liver Transpl , 2013. © 2013 AASLD.Liver Transplantation 02/2014; 20(2). DOI:10.1002/lt.23773 · 3.79 Impact Factor