Primary hepatic sarcomas in children—a single-center experience over 19 years

Upadhyaya, Manasvi; McKiernan, Patrick; Hobin, David; Kelly, Déirdre; Brown, Rachel M.; Lloyd, Carla; Buckels, J. A. C.; Millar, Alastair J. W.; DeGoyet, Jean deVille; Mirza, Darius F.; Sharif, Khalid

doi:10.1016/j.jpedsurg.2010.07.016

Cited by 19 publications

(24 citation statements)

References 9 publications

(18 reference statements)

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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%

“…Currently, there are no standard chemotherapy regimens designed specifically for UESL. Accordingly, regimens reported in the literature are varied, and protocols originally designed for the treatment of pediatric rhabdomyosarcoma or other soft tissue sarcomas are often employed . The effectiveness of radiation therapy to prevent recurrence and improve survival in UESL is unknown.…”

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

130

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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%

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

130

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“…Subsequent articles suggest that the use of multimodal therapy has significantly improved prognosis. [6][7][8] This improvement in outcome may be related to a modern multidisciplinary approach, including potent combinations of neo-adjuvant and adjuvant chemotherapy, radical surgical resection and radiotherapy, and improved supportive care.…”

Section: Discussionmentioning

confidence: 99%

“…Although there are diagnostic criteria on clinical, radiological and histological grounds, [3][4][5] there is no treatment consensus. [6] Early literature reports a poor prognosis with limited survival, and long-term survival rates are difficult to assess due to relatively short follow-up. Subsequent reports suggest significantly improved prognosis with the use of multimodal therapy, [6][7][8] which may be secondary to one of many factors.…”

mentioning

confidence: 99%

“…[6] Early literature reports a poor prognosis with limited survival, and long-term survival rates are difficult to assess due to relatively short follow-up. Subsequent reports suggest significantly improved prognosis with the use of multimodal therapy, [6][7][8] which may be secondary to one of many factors. Some units favour a sequence of neo-adjuvant chemotherapy, followed by surgery and adjuvant chemotherapy.…”

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confidence: 99%

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Management of undifferentiated embryonal sarcoma of the liver in children: A case series and management review

et al. 2013

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Background. Undifferentiated embryonal sarcoma of the liver (UESL) is a rare neoplasm, and the third-most common paediatric hepatic malignancy. However, no treatment guidelines exist. No randomised, controlled trials support specific combinations of therapy. Objective. To compare presentation and management of UESL with other series, review the literature, and formulate treatment guidelines. Methods. A retrospective chart review of all hepatic malignancies was conducted from 1996 to 2007 and 5 children with UESL were identified. Management and outcomes were documented. The literature regarding treatment modalities up to September 2012 was reviewed. Results. Over a period of 11 years, 5 patients presented. All underwent surgery and 4 received chemotherapy. One received radiotherapy at relapse. Three are disease-free with follow-up of 58 -184 months. One died after relapse, as did the patient whose family declined chemotherapy. Conclusion. The improved outcomes are consistent with the international experience and are probably related to combined treatment modalities and advances in supportive care. Pre-operative percutaneous biopsy provides no benefit if the lesion is resectable because it may not prove to be diagnostic, and may cause recurrence in the biopsy tract. If resectable, the recommended treatment is primary excision and adjuvant chemotherapy, with radiotherapy in selected cases. If unresectable, open biopsy is necessary to document histology, and neoadjuvant chemotherapy is given prior to resection. If deemed unresectable, liver transplantation is considered.

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Embryonal sarcoma of the liver in pediatric and young adult patients: A report from Children’s Oncology Group study ARST0332

Spunt,

Xue,

Gao

et al. 2024

Cancer

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BackgroundEmbryonal sarcoma of the liver (ESL) is a rare mesenchymal tumor most common in childhood; the optimal treatment approach is uncertain. The clinical features and outcomes of patients with ESL enrolled in a Children’s Oncology Group (COG) clinical trial that evaluated a risk‐based strategy for treating soft tissue sarcomas in patients aged <30 years were evaluated.MethodsThis subset analysis included patients with ESL enrolled in COG study ARST0332. Central review of records, pathology, and imaging confirmed the diagnosis, presenting features, and surgery extent and complications. All patients received dose‐intensive ifosfamide/doxorubicin chemotherapy, with cycle timing dependent on surgery and radiotherapy. Tumor resection occurred before study entry or after four cycles of chemotherapy; radiotherapy for residual tumor was optional.ResultsThirty‐nine eligible/evaluable patients with ESL were analyzed. All tumors were >10 cm in diameter; four were metastatic. Tumor resection was performed upfront in 23 and delayed in 16. Positive surgical margins (n = 6) and intraoperative tumor rupture (n = 6) occurred only in upfront resections. Eight patients received radiotherapy. Estimated 5‐year event‐free and overall survival were 79% (95% confidence interval [CI], 65%–93%) and 95% (95% CI, 87%–100%), respectively. Positive margins increased the local recurrence risk. One of 13 patients with documented hemorrhagic ascites and/or tumor rupture developed extrahepatic intra‐abdominal tumor recurrence.ConclusionsThe treatment strategy used in ARST0332 achieved favorable outcomes for patients with ESL despite a substantial proportion having high‐risk disease features. Deferring tumor resection until after neoadjuvant chemotherapy may decrease the risk of intraoperative tumor rupture and improve the likelihood of adequate surgical margins.

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Primary hepatic sarcomas in children—a single-center experience over 19 years

Cited by 19 publications

References 9 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

Management of undifferentiated embryonal sarcoma of the liver in children: A case series and management review

Embryonal sarcoma of the liver in pediatric and young adult patients: A report from Children’s Oncology Group study ARST0332

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