Treatment of undifferentiated embryonal sarcoma of the liver in children—single center experience

Ismail, Hor; Dembowska‐Bagińska, Bożenna; Broniszczak, Dorota; Kaliciński, Piotr; Maruszewski, Przemysław; Kluge, Przemysław; Swieszkowska, Ewa; Kościesza, Andrzej; Lembas, Agnieszka; Perek, Danuta

doi:10.1016/j.jpedsurg.2013.05.020

Cited by 53 publications

(53 citation statements)

References 15 publications

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“…However, these data were from an era that preceded the widespread use of combined therapy as the preferred treatment. Since then, the long‐term survival rate of UESL patients has steadily improved (Table ) and is currently reported to be >70% . Some of the first cases of long‐term survivors treated with combination chemotherapy and surgery were described in the late 1980s .…”

Section: Discussionmentioning

confidence: 99%

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Characteristics and outcomes in children with undifferentiated embryonal sarcoma of the liver: A report from the National Cancer Database

Shi

Rojas

Zhang

et al. 2016

Pediatr Blood Cancer

112

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ObjectiveTo examine patient characteristics and outcomes in children with undifferentiated embryonal sarcoma of the liver (UESL) using a multi‐institutional database.Summary Background DataUESL is a rare disease (incidence is one per million). Therefore, the current literature is mostly limited to small case series.MethodsThe National Cancer Database was queried for primary UESL diagnosed between 1998 and 2012.ResultsA total of 103 patients (<18 years) were identified. The 5‐year overall survival of the entire group was 86%. The best outcomes were seen in children who had tumors smaller than 15 cm and were able to undergo surgical resection with or without chemotherapy. Margin status did not appear to significantly affect survival. The most common type of resection was hemihepatectomy (37%), followed by sectionectomy (10%) and trisectionectomy (10%). Orthotopic liver transplant was performed in 10 children, all of whom survived to 5 years.ConclusionSurgical resection with or without chemotherapy should be the mainstay of treatment in children with UESL, and is associated with very favorable outcomes. Negative surgical margins were not associated with improved survival. Orthotopic liver transplantation may be a viable method of attaining local control in tumors, which would otherwise be unresectable.

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Section: Discussionmentioning

confidence: 99%

“…Since then, the longterm survival rate of UESL patients has steadily improved ( Table 4) and is currently reported to be >70%. 5,14,[20][21][22][23][24][25] Some of the first cases of long-term survivors treated with combination chemotherapy and surgery were described in the late 1980s. 26 survivors for periods ranging from 14 to 240 months.…”

Section: Discussionmentioning

confidence: 99%

Characteristics and outcomes in children with undifferentiated embryonal sarcoma of the liver: A report from the National Cancer Database

Shi

Rojas

Zhang

et al. 2016

Pediatr Blood Cancer

112

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“…As more institutions share their experiences, it is clear that a radical resection combined with multiagent adjuvant chemotherapy is necessary to improve survival. Reported regimens favor anthracycline and alkylator backbones . There have also been reports of success with a vincristine, actinomycin‐D, and cyclophosphamide regimen .…”

Section: Discussionmentioning

confidence: 99%

“…UESL is an aggressive pediatric tumor classically diagnosed between the ages 6 and 10 without gender predominance . It accounts for between 5% and 15% of pediatric liver tumors . Patients commonly present with abdominal mass, pain, and systemic symptoms such as nausea, anorexia, and weight loss.…”

Section: Introductionmentioning

confidence: 99%

A single‐center experience with undifferentiated embryonal sarcoma of the liver

Mathias

Ambati

Chou

et al. 2016

Pediatric Blood & Cancer

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“…Undifferentiated embryonal sarcoma of the liver (UESL) is an uncommon malignant hepatic neoplasm that occurs more frequently in older children but has also been described in infancy [66,67] . Malignant transformation from mesenchymal hamartoma or a solitary liver cyst to UESL has been reported.…”

Section: Undifferentiated Embryonal Sarcomamentioning

confidence: 99%

Differential diagnosis and management of liver tumors in infants

Fernández-Pineda

Cabello-Laureano

2014

WJH

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During the first year of life, most of the liver neoplasms are benign in origin, but some of these histologically benign lesions may be challenging in their management. Although most hepatic hemangiomas can be safely observed until involution is documented, some patients will need treatment due to progressive hepatomegaly, hypothyroidism and/or cardiac failure. Large mesenchymal hamartomas may require extensive hepatic resection and an appropriate surgical plan is critical to obtain good results. For malignant neoplasms such as hepatoblastoma, complete surgical resection is the mainstay of curative therapy. The decision about whether to perform an upfront or delayed resection of a primary liver malignant tumor is based on many considerations, including the ease of resection, surgical expertise, tumor histology and stage, and the likely chemosensitivity of the tumor. This article reviews the initial management of the more common hepatic tumors of infancy, focusing on the differential diagnosis and treatment options.

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Treatment of undifferentiated embryonal sarcoma of the liver in children—single center experience

Cited by 53 publications

References 15 publications

Characteristics and outcomes in children with undifferentiated embryonal sarcoma of the liver: A report from the National Cancer Database

Characteristics and outcomes in children with undifferentiated embryonal sarcoma of the liver: A report from the National Cancer Database

A single‐center experience with undifferentiated embryonal sarcoma of the liver

Differential diagnosis and management of liver tumors in infants

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