717 Risk adapted treatment for childhood hepatoblastoma (HB): final report of the second study of the International Society of Paediatric Oncology SIOPEL' 2

Perilongo, Giorgio; Shafford, E.; Brugières, L; Brook, Pamela; Maibach, R; Czauderna, Piotr; MacKinlay, Gordon A.; Aroson, D.; Childs, Margaret; Plaschkes, J.

doi:10.1016/s1359-6349(03)90748-8

Cited by 105 publications

(188 citation statements)

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“…SIOPEL 1 data also showed that two risk groups could be distinguished: the standard risk patients, with resectable tumors and no metastases, and the high-risk patients, with either unresectable tumors and/or metastasis, or low AFP. Following, SIOPEL 2 (1994-1998) introduced a patient stratification for treatment based on the two risk categories; standard risk patients were treated with cisplatin alone to avoid the cardiotoxicity associated with doxorubicin, whereas high-risk patients were treated with the combination of cisplatin/doxorubin/carboplatin [19]. Other SIOPEL trials have occurred [20,21], and current trial is SIOPEL 6, with standard risk patients [22].…”

Section: Prevalence and Clinical Features Of Hepatoblastomasmentioning

confidence: 99%

The genetic and epigenetic landscapes of hepatoblastomas

et al. 2017

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Primary liver cancers are rare in children, and the most common type is hepatoblastoma (HB), an embryonal tumor with histological features that resemble different stages of liver cell differentiation. However, mainly because of its rarity, molecular data on HB tumorigenesis remain scarce. This article reviews the current knowledge regarding genetic and epigenetic alterations reported in HB cases, with emphasis on the recent findings of next-generation sequencing studies.

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Section: Prevalence and Clinical Features Of Hepatoblastomasmentioning

confidence: 99%

The genetic and epigenetic landscapes of hepatoblastomas

et al. 2017

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“…The combination of cisplatin-based chemotherapy and surgery has improved survival in patients with unresectable HB by increasing the number of patients whose tumors can be resected. Patients whose tumor may not be resectable even after neoadjuvant chemotherapy should be referred to a liver transplant center [44][45][46][47][48] .…”

Section: Fernandez-pineda I Et Al Liver Tumors In Infantsmentioning

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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“…Subsequent clinical trials have shown that cisplatin-based chemotherapy improves survival by increasing tumor resectability, treating metastatic disease, and reducing the incidence of local and distant relapse. [8][9][10][11][12] In the prechemotherapy era, Kasai and Watanabe 13 reported that among children with resectable tumors, there was a higher rate of survival for children with fetal histology than for those with anaplastic or poorly differentiated small-cell histology. Weinberg and Finegold 14 similarly reported long-term survival for six children who had completely resected tumors of pure fetal histology (PFH), whereas only two of 10 patients with other histologic cell types survived.…”

Section: Introductionmentioning

confidence: 99%

Complete Surgical Resection Is Curative for Children With Hepatoblastoma With Pure Fetal Histology: A Report From the Children's Oncology Group

Malogolowkin

Katzenstein

Meyers

et al. 2011

JCO

165

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Purpose Children with pure fetal histology (PFH) hepatoblastoma treated with complete surgical resection and minimal adjuvant therapy have been shown to have excellent outcomes when compared with other patients with hepatoblastoma. We prospectively studied the safety and efficacy of reducing therapy in all children with stage I PFH enrolled onto two consecutive studies. Patients and Methods From August 1989 to December 1992, 9 children with stage I PFH were treated on the Intergroup Hepatoblastoma study INT-0098 and were nonrandomly assigned to receive chemotherapy after surgical resection with single-agent bolus doxorubicin for 3 consecutive days. From March 1999 to November 2006, 16 children with stage I PFH enrolled onto Children's Oncology Group Study P9645 were treated with observation after resection. Central confirmation of the histologic diagnosis by a study group pathologist was mandated. The extent of liver disease was assigned retrospectively according to the pretreatment extent of disease (PRETEXT) system and is designated “retro-PRETEXT” to clarify the retrospective group assignment. Results Five-year event-free and overall survival for the 9 patients treated on INT-0098 were 100%. All 16 patients enrolled onto the P9645 study were alive and free of disease at the time of last contact, with a median follow-up of 4.9 years. Retro-PRETEXT for the 21 patients with available data revealed seven patients with stage I disease, 10 patients with stage II disease, and four patients with stage III disease. Conclusion Children with completely resected PFH hepatoblastoma can achieve long-term survival without additional chemotherapy. When feasible, surgical resection of hepatoblastoma at diagnosis, without chemotherapy, can identify children for whom no additional therapy is necessary.

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717 Risk adapted treatment for childhood hepatoblastoma (HB): final report of the second study of the International Society of Paediatric Oncology SIOPEL' 2

Cited by 105 publications

References 0 publications

The genetic and epigenetic landscapes of hepatoblastomas

The genetic and epigenetic landscapes of hepatoblastomas

Differential diagnosis and management of liver tumors in infants

Complete Surgical Resection Is Curative for Children With Hepatoblastoma With Pure Fetal Histology: A Report From the Children's Oncology Group

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