Thirty years of experience with pediatric primary malignant liver tumors

Giacomantonio, Michael; Ein, Sigmund H.; Mancer, Kent; Stephens, Clinton A.

doi:10.1016/s0022-3468(84)80095-0

Cited by 74 publications

(36 citation statements)

References 12 publications

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“…7 Various surgical techniques have been used to increase the percentage of resectable tumors: profound hypothermia and circulatory arrest, 8 hemodilutional anesthesia, 9 total hepatic vascular exclusion, 10 and concomitant liver and inferior vena cava resection. 11 However, none of these techniques have reliably increased the number of liver tumors amenable to partial hepatectomy.…”

Section: Discussionmentioning

confidence: 99%

Resection, including transplantation, for hepatoblastoma and hepatocellular carcinoma: Impact on survival

Tagge

Reyes

et al. 1992

Journal of Pediatric Surgery

118

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Long-term survival in children with primary hepatic malignancies can not be expected without complete tumor resection. In the last ten years we have treated 21 children with hepatocellular carcinoma (HCC) and 21 children with hepatoblastoma (HEP), with tumor extirpation our surgical goal. Operative treatment included partial hepatectomy ([PH] 20), either primary (10) or delayed (following chemotherapy) (10), total hepatectomy and orthotopic liver transplantation ([OLT] 13), or upper abdominal exenteration and multiple organ transplantation (2). Two patients had both PH and subsequent total hepatectomy and OLT. Overall survival was 48% (20/42), with 9 patients dying of progressive disease prior to removal of their tumor. HEP patient survival was 67% (14/21), including 2 of 6 who underwent primary PH, 7 of 8 who had delayed PH, and 5 of 6 who underwent OLT. Survival for the children with HCC was 29% (6/21), including 1 of 4 after primary PH, 1 of 2 following delayed PH, 3 of 7 following OLT, and 1 of 2 after exenteration and multiple organ transplantation. Preoperative chemotherapy facilitated removal of 10 initially unresectable tumors (8 HEP, 2 HCC) at a second-look procedure. Total hepatectomy and OLT markedly improved survival in patients with disease unresectable by standard methods. Partial hepatectomy, either primary or delayed, should be attempted in all children with hepatic malignancies. Total hepatectomy and OLT appears to be a viable adjunct in the treatment of childhood malignancies, and should be used for otherwise unresectable tumors as part of a carefully planned protocol. INDEX WORDSHepatoblastoma; hepatocellular carcinoma; liver transplantation Childhood liver tumors continue to have a poor prognosis despite increasingly sophisticated diagnostic methods, aggressive surgical techniques to achieve complete resection, and more effective systemic chemotherapy. Total surgical removal remains the mainstay of treatment, although there are rare reports of long-term survivors achieved with chemotherapy alone. Preoperative chemotherapy has increased the number of patients undergoing potentially curable resections. 1 However, a large proportion of children, especially with hepatocellular carcinoma (HCC), are not resectable by standard methods. Total hepatectomy and orthotopic liver transplantation (OLT), the only alternative for unresectable tumors, is still controversial due to the high incidence of tumor recurrence. 2Address reprint requests to Edward P. Tagge, MD,

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Section: Discussionmentioning

confidence: 99%

Resection, including transplantation, for hepatoblastoma and hepatocellular carcinoma: Impact on survival

Tagge

Reyes

et al. 1992

Journal of Pediatric Surgery

118

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“…The incidence of associated congenital and acquired disorders varies from 10-20% [4,7]. While the association of HBL with Beckwith-Wiedemann syndrome, and also early virilisation is well known [S-lo].…”

Section: Discussionmentioning

confidence: 99%

Primary hepatic tumours in children: A 26—year review

Gururangan

O’Meara

Macmahon

et al. 1992

Journal of Surgical Oncology

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Twenty-one children were admitted to a single paediatric institution between 1964-1990 with histologically proven primary liver tumours. The diagnosis was hepatoblastoma (HBL) in 15 patients, hepatocellular carcinoma (HCA) in 2, rhabdomyosarcoma (RMS) in 2, non-Hodgkin's lymphoma (NHL) in 1, and haemangioendothelioma (HE) in 1. The common presenting clinical features were anaemia, abdominal mass, and abdominal pain. Serum alpha-foetoprotein was useful in establishing a diagnosis in HBL and in monitoring disease activity. Computed tomographic (CT) scan, ultrasound, and angiography were useful preoperative investigations for assessing site and resectability of tumour. There were no survivors in patients with malignant hepatic tumours (n = 10) who had surgery alone prior to 1981. Of 7 patients with HBL diagnosed after 1981 who had adequate surgical resection and chemotherapy, 5(72%) are currently alive and disease free between 15 months and 8 years from diagnosis. We conclude that adequate surgical resection and adjuvant chemotherapy can improve disease free survival for children with HBL. Optimal treatment has yet to be devised for other malignant hepatic tumours.

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“…Traditionally, hepatoblastomas were considered to be tumours of poor prognosis with a survival rate of only 25% until the late 1970s [1,2]. With the introduction of multidisciplinary approaches and combination chemotherapy with cisplatin and doxorubicin as both pre-and postoperative therapy, outcomes have improved significantly [5,6,[11][12][13][14][15][16][17][18].…”

Section: Discussionmentioning

confidence: 99%

“…Hepatoblastoma, the most common primary malignant liver tumour in infants and children, has been traditionally associated with poor prognosis [1]. Unresectability is largely due to the size of the tumour with attendant invasion of hepatic vessels and the inferior vena cava [2,3].…”

Section: Introductionmentioning

confidence: 99%

Midterm results with hepatectomy after preoperative chemotherapy in hepatoblastoma

et al. 2005

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We evaluated the results of surgical treatment for hepatoblastoma in infants and children after intensive preoperative chemotherapy, with special reference to histology and extent of liver involvement. The clinical features of 10 children with hepatoblastoma were reviewed regarding response to neoadjuvant chemotherapy, histological subtypes, extent of hepatectomy, operative complications, and prognosis. Response to chemotherapy was measured by volumetric assessment of tumour size by computed tomography scan. Cisplatin and Adriamycin (PLADO regime) up to three cycles markedly reduced the tumour volume on computed tomography (mean regression rate 65.9%); alpha-foetoprotein (AFP) levels also decreased from an initial mean of 16,116.4 ng/ml to 2,050.9 ng/ml. Five patients underwent right hepatectomy, two had right trisegmentectomy, two had left hepatectomy, and one had left trisegmentectomy. Histopathology of resected specimens revealed foetal histology in four patients, poorly differentiated (anaplastic) subtype in three, and mixed histology with mesenchymal components and osteoid formation in three. There was 100% resectability including six unresectable tumours (prechemotherapy). Moreover, hepatic resection tended to be less invasive in patients whose tumours had been much reduced after preoperative chemotherapy. Preoperative administration of cisplatin and Adriamycin reduces the tumour size significantly so that a safe radical hepatectomy can be performed. It also allows early administration of postoperative chemotherapy. Although overall good results were obtained with the current protocol, we also document our experience of unfavourable outcomes in patients with bilobar tumours (despite trisegmentectomy), patients with tumours showing poor response to neoadjuvant chemotherapy, and patients with anaplastic histology. Overall, at a 60-month follow-up we report an 80% survival rate by a combined approach.

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Thirty years of experience with pediatric primary malignant liver tumors

Cited by 74 publications

References 12 publications

Resection, including transplantation, for hepatoblastoma and hepatocellular carcinoma: Impact on survival

Resection, including transplantation, for hepatoblastoma and hepatocellular carcinoma: Impact on survival

Primary hepatic tumours in children: A 26—year review

Midterm results with hepatectomy after preoperative chemotherapy in hepatoblastoma

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