Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Malignant primary and metastatic lesions of the liver have a myriad of imaging appearances. Discriminating between the various lesions requires an understanding of the underlying pathophysiology and imaging characteristics that lead to their malignant appearances. A pattern approach of recognition by imaging with understanding of why particular lesions behave the way they do on sonography, CT, MRI, and nuclear scintigraphy can be developed. A broad selection of malignant cystic liver lesions will be displayed in a multimodality pictorial style including hepatocellular carcinoma, cholangiocarcinoma, cystic and necrotic hepatic metastases, biliary cystadenoma/cystadenocarcinoma, epitheloid hemangioendothelioma, hepatoblastoma, and cystic sarcomas. Characteristic imaging features, clinical symptoms, and prognosis will be discussed. Emphasis will be placed on consistent features of these malignant lesions that help to discriminate them from other hepatic disorders.
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PMID:Malignant cystic and necrotic liver lesions: a pattern approach to discrimination. 1554 51

After an uneventful pregnancy, a boy was born by vacuum extraction at 40.6 weeks' gestation. Physical examination revealed several malformations due to a partial trisomy 9p [karyotype: 46,XY, dup(9)(p13p24)]. Three months after birth, the boy presented with a hepatoblastoma without distant metastases which was treated with chemotherapy combined with surgery. At the last follow-up, 15 years after the resection of the hepatoblastoma, he was still in complete remission. To our knowledge this is the first case report of a patient with a constitutional partial trisomy 9p associated with hepatoblastoma.
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PMID:Hepatoblastoma in a patient with a partial trisomy 9p syndrome: a case report. 1558 61

Early detection of recurrent hepatoblastoma is not always possible with conventional imaging methods such as computed tomography (CT) and magnetic resonance imaging (MRI). This article describes three cases of recurrent hepatoblastoma in which positron emission tomography (PET) using F(18)-fluoro-deoxy-glucose (FDG-PET) was used to locate the site of recurrence. In the first two patients, FDG-PET accurately located recurrent disease where it was not detected by conventional imaging modalities, including CT and MRI. In the third patient, FDG-PET imaging also located the recurrent disease in an MRI-identified adrenal metastasis. The technique of "coregistration" using PET with CT and MRI scans was used in the cases described. This improves the anatomical localization of metabolically active sites and was particularly useful for determining the surgical approach. The difficulties of conventional imaging in locating early tumor recurrence or metastatic disease in hepatoblastoma make FDG-PET imaging an important investigation that may impact patient management.
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PMID:Positron emission tomography in recurrent hepatoblastoma. 1583 79

Cisplatin-containing chemotherapy and complete surgical resection are both crucial in the cure of hepatoblastoma. Radical resection can be obtained either conventionally by partial hepatectomy or with orthotopic liver transplant, but the surgical approach to hepatoblastoma differs considerably across the world. Our main aim in this paper is to present the surgical recommendations of the Childhood Liver Tumour Strategy Group of the International Society of Paediatric Oncology (SIOPEL), as well as to stimulate international debate on this issue. We discuss biopsy, verification of resectability, resection principles, indications and potential contraindications for orthotopic liver transplant, as well as thoracic surgery for pulmonary metastases. We suggest that heroic liver resections with a high probability of leaving residual tumour should be avoided whenever possible. In such cases primary orthotopic liver transplant should be considered. Superior survival rates in hepatoblastoma patients who have received a primary transplant after a good response to chemotherapy support the strategy of avoiding partial hepatectomy in cases where radical resection appears difficult and doubtful. We recommend early referral to a transplant surgeon in cases of: (i) multifocal or large solitary PRETEXT IV (PRE Treatment EXTent of disease scoring system) hepatoblastoma involving all four sectors of the liver and (ii) unifocal, centrally located tumours involving main hilar structures or main hepatic veins. Because complete tumour resection is a prerequisite for cure, any strategy leading to an increased resection rate will result in improved survival. We advise the more frequent use of orthotopic liver transplant, as well as the standardisation of techniques for partial liver resection. These guidelines should not be seen as final, but rather as a starting point for further discussion between the various national and international liver tumour study groups.
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PMID:Guidelines for surgical treatment of hepatoblastoma in the modern era--recommendations from the Childhood Liver Tumour Strategy Group of the International Society of Paediatric Oncology (SIOPEL). 1586 52

Parthenolide, a sesquiterpene lactone, shows antitumor activity in vitro, which correlates with its ability to inhibit the DNA binding of the antiapoptotic transcription factor nuclear factor kappaB (NF-kappaB) and activation of the c-Jun NH(2)-terminal kinase. In this study, we investigated the chemosensitizing activity of parthenolide in vitro as well as in MDA-MB-231 cell-derived xenograft metastasis model of breast cancer. HBL-100 and MDA-MB-231 cells were used to measure the antitumor and chemosensitizing activity of parthenolide in vitro. Parthenolide was effective either alone or in combination with docetaxel in reducing colony formation, inducing apoptosis and reducing the expression of prometastatic genes IL-8 and the antiapoptotic gene GADD45beta1 in vitro. In an adjuvant setting, animals treated with parthenolide and docetaxel combination showed significantly enhanced survival compared with untreated animals or animals treated with either drug. The enhanced survival in the combination arm was associated with reduced lung metastases. In addition, nuclear NF-kappaB levels were lower in residual tumors and lung metastasis of animals treated with parthenolide, docetaxel, or both. In the established orthotopic model, there was a trend toward slower growth in the parthenolide-treated animals but no statistically significant findings were seen. These results for the first time reveal the significant in vivo chemosensitizing properties of parthenolide in the metastatic breast cancer setting and support the contention that metastases are very reliant on activation of NF-kappaB.
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PMID:The sesquiterpene lactone parthenolide in combination with docetaxel reduces metastasis and improves survival in a xenograft model of breast cancer. 1595 58

Hepatoblastoma is the most common malignant live tumor in childhood. Multicenter trials have made it clear that pre- and/or postoperative chemotherapy are necessary to improve the outcome of the treatment of hepatoblastoma. The results of the Japanese Pediatric Liver Tumor (JPLT)-1 study since 1991 have been compatible with those of other group study trials in Europe and the USA. However, patients with unresectable primary tumors or with lung metastasis still have a poor prognosis. The JPLT has developed a new protocol that includes four courses of treatment based on the PRETEXT staging system. These treatments include intensive chemotherapy with stem cell transplantation for patients with unresectable primary tumors or with pulmonary metastases.
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PMID:[Strategy for the treatment of hepatoblastoma in children]. 1604 86

Major progress has been achieved during the last decades in the treatment of malignant liver tumors in children, both in chemotherapy and surgical management. Chemosensitivity varies between tumor types, and radical resection remains essential to effect a cure. In tumors extensively involving a normal liver, in a diffuse or multifocal manner, radical resection cannot be accomplished with a partial hepatectomy. This has been the case for some instances of advanced hepatoblastoma and epithelioid hemangioendothelioma. In hepatoblastoma, current experience shows that results of primary liver transplantation with neoadjuvant chemotherapy are excellent with around an 80% 5-to-10-year disease-free survival rate. Epithelioid hemangioendothelioma is very rarely seen in children and may have a more malignant behavior than in adult patients, and liver transplantation may not be the best management option. In nonresectable hepatocellular carcinoma (HCC) developed on an otherwise normal liver, the results of liver transplantation are similarly poor to those obtained in adult patients, except in a few highly selected series fulfilling the Milano criteria. The experience with HCC is still very scarce in children. Incidental HCC associated with chronic liver disease does not seem to impact posttransplant survival. When they are symptomatic, however, indications for transplantation should be very selective regarding tumor size, multi-focality, vascular invasion and distant metastases.
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PMID:The contribution of transplantation to the treatment of liver tumors in children. 1622 98

We report the case of a girl with multi-focal hepatoblastoma in whom chemotherapy alone has resulted in long term event-free survival and possibly cure, without any surgical procedure apart from biopsy for initial diagnosis. At presentation she had a large tumour arising from the left lobe of liver and two other separate masses were noted in the right lobe, but the lungs were free of metastases. Histology showed a foetal type of hepatoblastoma. The serum alpha-feto protein (AFP) level was 44,000 iu/litre. Chemotherapy was started using the triple drug regime recommended for "high risk" (of relapse) patients in the SIOPEL 2 hepatoblastoma protocol of the International Society of Paedaitric Oncology (SIOP). Within a few weeks her abdominal girth decreased, the child became much more comfortable. Drug-induced cardiotoxicity, ototoxicity and nephrotoxicity were not observed. After a total of 4 courses of chemotherapy (completed at the end of August 1998) a CT scan showed that all 3 tumours were smaller but that there were residual multifocal defects in the liver neither hepatic resection nor liver transplantation were considered safe or appropriate. 6.5 years after completion of chemotherapy and now aged 8.5 years the child is in normal health and at school with normal liver size, serum AFP levels and chest imaging.
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PMID:Successful treatment of multifocal unresectable hepatoblastoma with chemotherapy only. 1665 Dec 44

To evaluate clinical and laboratory findings of these patients and the efficacy of liver transplantation in children with hepatocellular carcinoma (HCC) and hepatoblastoma (HB) associated with tyrosinemia. Among 113 children with liver tumors diagnosed between 1972 and 2004 five patients had HCC or HB associated with tyrosinemia. The age at diagnosis of the HCC or HB ranged from 9.5 to 17 yr and male:female ratio was 1:4. During regular clinic visits for tyrosinemia, elevated alpha-fetoprotein (AFP) was detected in all patients. AFP levels ranged between 13.7 and 29 340 IU/mL. Radiological studies including ultrasound, computed tomography and magnetic resonance imaging showed heterogeneous parenchyma and nodules in the liver. The patients did not have any metastatic disease. The time from diagnosis of tyrosinemia to HCC or HB ranged from 9.25 to 15.25 yr. Histopathologically, four patients have been diagnosed as HCC and one patient had HB. All patients were given chemotherapy including cisplatin and adriamycin. In three patients, living-related liver transplantation was performed. They had no treatment after transplantation. All of them are disease free. One patient was treated with chemotherapy and right hepatectomy. She had no suitable donor for living-related liver transplantation. Three months after completing chemotherapy, she had recurrent tumor in the left lobe of the liver and she died with progressive disease. The last patient whose parents were not suitable as donors for living-related liver transplantation is waiting for a deceased donor graft. All patients had limited disease to liver due to close clinical and radiological follow up for tyrosinemia. In these patients liver transplantation is curative both for liver tumor and tyrosinemia.
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PMID:The efficacy of liver transplantation in malignant liver tumors associated with tyrosinemia: clinical and laboratory findings of five cases. 1671 14

Our objective is to review a leading Australian tertiary referral centre's experience in treating hepatoblastoma over two decades. This is a retrospective study of clinical, radiological and pathological data in hepatoblastoma patients treated at our institution between 1984 and 2004. Thirty children (17 male, 13 female) ranging in age from 5 months to 6.5 years (median 19 months) at diagnosis were reviewed. This is the largest case series reported in Australia to date. The median length of follow-up was 6.3 years (range 7 months to 20.9 years). On average, the number of new cases increased by 0.12 each year (P=0.01). A total of 29 patients underwent definitive surgery for primary tumour control. Of these, two (7%) had initial primary surgery, while 27 (93%) had delayed surgery following neo-adjuvant chemotherapy. Chemotherapy regimens included the SIOPEL study protocols. Patients not enrolled in SIOPEL tended to be given more courses of chemotherapy. Ten patients (34%) received an extended right hemi-hepatectomy, six (21%) had right hemi-hepatectomy, seven (24%) had left hemi-hepatectomy, three (10%) had left lateral segmentectomy, and two (7%) had a non-anatomical resection. In addition, two patients required IVC reconstruction at the time of their primary liver resection. Overall survival at 5 years was 89.1% (95% CI 69.8-96.4%). Event-free survival at 5 years was 75.7% (95% CI 53.2-88.5%). There was a clear increase in the risk of recurrence (RR=4.8) and death (RR=4.5) where margins were not microscopically clear. However, neither reached statistical significance in this small cohort. Surgery was still worthwhile even when margins were involved. There was no correlation between mean AFP level at diagnosis, and outcome. Our experience suggests that the achievement of microscopically clear margins at the time of primary tumour resection is an important factor in achieving a positive long-term outcome. In addition, extended courses of neo-adjuvant chemotherapy in patients with pulmonary metastases achieves good long-term outcomes, provided adequate surgical resection of the primary tumour with clear microscopic margins can be achieved.
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PMID:Treatment outcomes for hepatoblastoma: an institution's experience over two decades. 1711 81


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