Rationale and results of the international society of pediatric oncology (SIOP) Italian pilot study on childhood hepatoma: Surgical resectionD'Emblée or after primary chemotherapy?

Guglielmi, M; Perilongo, Giorgio; Cecchetto, Giovanni; Rondelli, Roberto; Lanino, E.; Siracusa, Fortunato

doi:10.1002/jso.2930530533

Cited by 5 publications

(1 citation statement)

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“…This first trial was called SIOPEL 1 and collected data about 194 patients during four years, investigating the role of the cisplatin/ doxorubicin combination chemotherapy [17]. The PRE-TEXT is based on pretreatment imaging with ultrasound and CT scans and/or magnetic resonance imaging, and describes the site and size of the tumor, invasion of the vessels and distant spread; the system identifies four PRETEXT stages (I-IV), which reflect the number of liver sections that are affected by the tumor [13,18].…”

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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Hepatocellular carcinoma in children

et al. 1997

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Hepatocellular carcinoma (HCC) occurs more frequently in subsaharan Africa and the Orient than in other geographical regions, but remains an uncommon tumour of childhood. We review six children with HCC (mean age 13 years) treated by the paediatric oncology unit at Tygerberg Hospital in Cape Town over an 8-year period (1983 - 1990). Patients presented with epigastric and right upper quadrant discomfort and hepatomegaly. The hepatitis B serum antigen (HbsAg) was positive in three patients; serum alpha-fetoprotein (AFP) levels were markedly elevated in three (range 100 - 453,000 microg/l). Age and sex did not differ significantly and all patients initially had irresectable advanced-stage tumours. Morphologically, three were highly malignant adult-type pleomorphic HCCs, two were differentiated tumours, and one a fibrolamellar subtype. The mean 2-year survival was 33% and the 5-year survival 16.6%. The biological behaviour and response to treatment of the tumours varied. Whereas three patients had a poor response to therapy, two with poorly-differentiated tumours, negative HbsAg, and normal serum AFP levels responded to doxorubicin/cisplatinum chemotherapy. This facilitated radical surgical excision. One patient of this group has survived for more than 75 months following surgical resection and remains well. HCC remains an uncommon tumour of childhood with a high mortality. Aggressive chemotherapeutic regimes in combination with surgical resection may lead to improved survival in some cases. Prevention of hepatitis B remains a priority.

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