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)

Our personal experience with 172 patients, the results from the European Liver Transplant Registry and a review of the recent literature are summarized and discussed to define present indications for liver transplantation in hepatobiliary malignancy. The following conditions should be considered contraindications: advanced primary liver tumors with any extrahepatic spread, cholangiocellular carcinoma, hemangiosarcoma and liver metastases from nonendocrine primary tumor. Currently, "favorable" indications include uncommon tumors such as fibrolamellar carcinoma, epithelioid hemangioendothelioma, hepatoblastoma and metastases from endocrine tumors. Further indications may be nonresectable hepatocellular and proximal bile duct carcinoma in tumor stage II. Borderline indications are hepatocellular and proximal bile duct carcinoma in tumor stage III. In advanced tumors confined to the liver, transplantation should be restricted to multimodality treatment protocols. Although there are strong arguments for transplantation in early resectable hepatocellular carcinoma with underlying cirrhosis, it remains an open issue requiring further investigation in a controlled study using the same tumor classification. With regard to limited resources of donor organs, split-liver transplantation permits transplantation in tumor patients without neglecting those with benign diseases.
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PMID:Indications for liver transplantation in hepatobiliary malignancy. 800 78

Pediatric tumors which are conventionally considered to be small round cell tumors (SRCTs) include the prototypical neuroblastoma as well as rhabdomyosarcoma (RMS), Ewing's sarcoma (ES), malignant lymphoma, and primitive neuroectodermal tumor (PNET). Other malignancies may be considered in the differential diagnosis such as small-cell osteogenic sarcoma, undifferentiated (anaplastic) hepatoblastoma, granulocytic sarcoma, blastemal type Wilms' tumor, and desmoplastic small-cell tumor of the peritoneum. The cytomorphologic features of conventional SRCTs is presented as well as the utility of ancillary studies performed on the aspirated material in making a specific and correct diagnosis. The role of the immediate cytologic assessment of the aspirate is stress, since this is a critical step in formulating an initial diagnostic impression that should prompt the need for additional material for pertinent ancillary studies. Although challenging, FNA cytology of SRCTs of childhood can be diagnostic in the majority of cases, allowing specific therapy to be given to patients with unresectable SRCTs without a tissue biopsy as well as documenting recurrent and/or metastatic disease.
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PMID:FNA biopsy of small round cell tumors of childhood: cytomorphologic features and the role of ancillary studies. 805 Mar 33

A significant proportion of children presenting with pediatric solid tumors will have disease distant from the primary site at diagnosis while still more will develop metastases. In this article an appropriate role for the surgical oncologist was determined by extracting surgical and survival data from the literature. It is concluded that aggressive pulmonary metastasectomy is indicated in metastatic osteogenic sarcoma but not in most embryonal soft tissue sarcomas. Wilms' tumor pulmonary metastases are probably best treated by chemotherapy and whole-lung irradiation except for very young children with solitary or localized metastases. However, resection of hepatic metastases from Wilms' tumor may result in long-term disease-free survival. Finally, available data support resection of hepatoblastoma metastases to lung. Surgery maintains an important role in the treatment of metastatic disease but surgeons must remain objective in the reporting and interpretation of results.
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PMID:The surgical management of metastases in pediatric cancer. 806 25

We applied cytologic and architectural diagnostic criteria to the aspiration smears of six cases of hepatoblastoma (HBL) in order to establish whether it is possible to obtain a reliable cytologic diagnosis of this neoplasm and to subclassify it based on cytologic evidence alone. We describe two groups of HBL, undifferentiated and differentiated. The undifferentiated group comprises anaplastic and embryonal subtypes, and the differentiated group comprises fetal and macrotrabecular subtypes. Our findings suggest that the fetal and macrotrabecular subtypes have a rather characteristic cytologic pattern, permitting us to differentiate between the two and to distinguish them from the other two subtypes. The differential diagnosis between the anaplastic and embryonal subtypes is more complex and can be difficult to carry out using cytologic criteria exclusively. The immunophenotypic pattern reflects the degree of maturity of each subtype and helps with cytologic subclassification. The cytologic differential diagnosis of undifferentiated HBL must include hepatic metastases of small round cell tumors of childhood. The cytologic differential diagnosis between differentiated HBL and hepatocellular carcinoma may be very difficult. We suggest that the cytologic subclassification of HBL is possible with the reservation that the predominant pattern may mask other, associated patterns.
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PMID:Hepatoblastoma. An attempt to apply histologic classification to aspirates obtained by fine needle aspiration cytology. 814 8

Hepatoblastomas (HBs) and hepatocellular carcinomas (HCCs) constitute the majority of hepatic tumors in children. Although most children who have HB or HCC present with unresectable disease, chemotherapy may reduce the size of the primary tumor and metastases, thus allowing subsequent successful removal. Clinical trials conducted over the past decade in the United States have demonstrated that chemotherapy regimens consisting of continuous-infusion doxorubicin and cisplatin or high-dose cisplatin alone effectively reduce tumor size. The prognosis of children with HB is better than that of those with HCC. Over half the patients who initially have unresectable HB can be rendered disease free with chemotherapy and subsequent surgery.
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PMID:Pediatric hepatic tumors: clinical trials conducted in the United States. 838 59

A child with stage 4 hepatoblastoma failed to respond to treatment with cisplatin and adriamycin. She then showed a response to carboplatin with complete clearing of pulmonary metastases. Bilobar liver disease persisted, although significantly reduced in size. A liver transplant was subsequently performed and she remains in complete remission 36 months later. After the first course of carboplatin, there was a dramatic rise in alpha-feto protein which then fell exponentially. Carboplatin warrants further study in hepatoblastoma.
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PMID:Cisplatin-resistant metastatic hepatoblastoma: complete response to carboplatin, etoposide, and liver transplantation. 839 11

Hepatoblastoma is a rare but highly malignant liver tumour, predominantly occurring during early childhood. Clinical data of 71 children treated from 1988 to 1993 according to the protocol of the multicentre liver tumour study (HB89) of the German Society for Paediatric Oncology and Haematology were evaluated to assess the effects of surgical therapy in these patients. The protocol prescribed an initial laparotomy for all children with a liver tumour, except for infants with distant metastases and a high serum-alpha-fetoprotein; for small hepatoblastomas confined to one liver lobe primary resection by a lobectomy; and for those extending into both lobes and metastasizing tumours tumour reductive chemotherapy including ifosfamide, cisplatin and adriamycin (IPA), preparatory to resection at second-look surgery. All patients received adjuvant IPA chemotherapy after tumour resection. In 20 of the 71 cases, the hepatoblastomas were completely resected at initial laparotomy (stage I), while in 6 cases microscopic residues were left (stage II). In 38 of 45 children with extended hepatoblastomas (stage III, 38 patients) or distant metastases (stage IV, 7 patients) the tumour was resected during later surgery, and 1 patient received a liver transplant. Thus, the overall resection rate was 92% (65 of 71 hepatoblastomas). After a median follow-up of 3.8 years (range 1.0-5.9 years), 53 (75%) of the patients were tumor-free and 18 (25%) had died. Recurrence-free survival was 100% for stage I, 50% for stage II, 71% for stage III and 29% for stage IV patients (P = 0.0009).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Surgical therapy of hepatoblastoma in childhood]. 855 99

Liver transplantation has been performed in five children with unresectable hepatic tumors who did not have extrahepatic metastases at the time of surgery. Two of the children had hepatoblastomas, one had an infantile hemangioendothelioma, and two had a hepatoma. The two children who had hepatoblastoma are well (37 and 25 months posttransplant) and have no evidence of recurrence. The child with infantile hemangioendothelioma had a successful operation, with good quality of life, but died of tumor recurrence 41 months after transplantation. Both children with hepatomas died, one of graft failure owing to chronic rejection and the other of tumor recurrence 5 months posttransplant. These results suggest that liver transplantation may be successful in children with unresectable hepatic tumors without extrahepatic spread and should be considered particularly for the treatment of hepatoblastoma.
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PMID:Unresectable hepatic tumors in childhood and the role of liver transplantation. 894 24

Hepatoblastoma is an embryonal tumour of the liver, which often contains tissue components with multidirectional differentiation. The occurrence of cell surface antigens in this tumour has not been studied systematically, and we therefore investigated 20 hepatoblastomas for the expression of common acute lymphoblastic leukaemia antigen (CALLA) and cell adhesion molecules (CAMs) in their different tissue components. Epithelial tumour cells of fetal differentiation contained E-cadherin. This protein did not occur in tumour areas with embryonal or mesenchymal differentiation. In contrast, immature embryonal and anaplastic cells expressed CALLA and the hyaluronate receptor (HCAM, CD44). Both fetal and embryonal areas stained irregularly positive for ICAM-1, which, in contrast, was not present on anaplastic cells. Immature fibrous tissue did not contain any of these molecules except for ICAM-1. However, some cells adjacent to, or enclosed in, osteoid were positive for HCAM and NCAM. Like small undifferentiated hepatoblastoma cells, primitive mesenchymal spindle-shaped cells also expressed CALLA, HCAM, and the polysialylated embryonic form of NCAM strongly. This last is not present on other epithelial or mesenchymal tumour cells. Hepatoblastoma cells of varying differentiation express distinct patterns of CAMs and CALLA. Our results give further insight into their histogenesis and cellular interactions and may explain their variable ability for invasive growth and formation of metastases.
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PMID:Expression of cell adhesion molecules and common acute lymphoblastic leukaemia antigen in hepatoblastoma. 897 59

A 26-month-old female with trisomy 18 and repaired omphalocele died of metastatic disease after resection of hepatoblastoma (HB) at 21 months of age. Four other cases (three of them published) suggest that the association of trisomy 18 and HB may be nonrandom. Karyotype abnormalities of the tumor in our case included duplication of 2q and +20, reported previously in HB arising in patients with normal karyotype. Antecedent growth disturbance of liver, either intrinsic (abnormal lobation) or related to contiguous extrinsic anomalies such as omphalocele or local diaphragmatic hypoplasia and possibly augmented by unusual sensitivity to noxious environmental agents, may predispose to hepatoblastoma in trisomy 18. Longevity in trisomy 18 predisposes to both hepatoblastoma and Wilms tumor, possibly by a shared pathway.
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PMID:Hepatoblastoma in a child with trisomy 18: cytogenetics, liver anomalies, and literature review. 902 31


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