Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019204 (hepatocellular carcinoma)
 71,386 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Fibrolamellar hepatocellular carcinoma (FLHC) is recognized as a distinct clinicopathologic variant of hepatocellular carcinoma. Ten consecutive patients with FLHC undergoing operative management at our institution were reviewed. At the initial presentation seven patients had stage II disease (pT2N0M0), whereas three patients were in stage III (pT2N0M0 or pT3N0M0). Initial procedures included formal right or left hepatectomy in four patients, right or left trisegmentectomy in two patients, left lateral segmentectomy or nonanatomic resection in three patients, and in one patient considered for liver transplantation, only exploration with biopsy of positive nodes was performed. Four stage II patients required a second procedure for resection of recurrent disease from 8 months to 6 years after the initial resection and one patient required a third procedure after 13 years. Reoperations included hepatic re-resection, resection of extrahepatic disease, and liver transplantation. Overall 5- and 10-year Kaplan-Meier survival was 70%. There were no deaths among stage II patients (follow-up 96 to 180 months). All stage III patients (i.e., lymph node involvement, vascular invasion, or multiple tumors) died within 5 years. Patients with stage II disease had better survival than patients with stage III disease (P = 0.011, log-rank test). Aggressive treatment of FLHC including reoperation and liver transplantation is justified, especially in patients with stage II disease.
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PMID:Aggressive surgical management of fibrolamellar hepatocellular carcinoma. 983 68

Fibrolamellar hepatocellular carcinoma (FLHCC), which is quite uncommon in Japan, is known to be frequently associated with lymph node metastasis in Western countries. Herein, we describe a case of a 25 year-old Japanese woman with recurrent FLHCC in the lymph nodes after undergoing right hepatic lobectomy. She underwent a second operation for removal of a recurrent celiac lymph node tumor 23 months after the initial operation. In Japan, the frequency of lymph node metastasis in ordinary hepatocellular carcinoma is only 1.6%, whereas 3 out of 9 (33%) reported domestic FLHCCs including this case had lymph node metastasis. The surgical management of lymph node metastasis in FLHCC is discussed.
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PMID:Surgical management for lymph node recurrence of resected fibrolamellar hepatocellular carcinoma: a case report. 1037 Jun 85

Fibrolamellar hepatocellular carcinoma (FHCC) is a variant of hepatocellular carcinoma, which mainly affects a young age group and carries a relatively good prognosis. It is widely accepted that aggressive curative resection is still the best option for FHCC. We report here a case of successful arterialisation of the portal vein with an aortoportal jump graft for portal vein thrombosis, which developed postoperatively in an already comprised portal vein with tumour invasion following an extensive liver resection for FHCC.
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PMID:Arterialisation of the portal vein with an aortoportal jump graft for portal vein thrombosis following liver resection for malignancy. 1046 21

Fibrolamellar hepatocellular carcinoma (FHCC) is a unique histologic variant of HCC that occurs in a younger subset of patients than classical HCC, and is associated with a better prognosis. Wilms tumor (WT) is a malignant embryonal neoplasm of the kidney and is one of the most common solid tumors of childhood, occurring at an estimated frequency of 1 in 8000 to 10,000 births. Although second malignant neoplasms (SMNs) following therapy for WTs have been reported in the liver, the coexistence of HCC and WT is extremely rare. We present the first report of a synchronous anaplastic WT and FHCC in a previously healthy 4-year-old girl. Despite the presence of focal immunohistochemical positivity for p53 in the WT, molecular analysis failed to reveal a germline or somatic p53 mutation, and was inconclusive in establishing a clonal relation between the two tumors.
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PMID:Synchronous wilms tumor and fibrolamellar hepatocellular carcinoma: report of a case. 1089 Sep 35

Fibrolamellar hepatocellular carcinoma (FL HCC) is an uncommon variant of hepatocellular carcinoma occurring usually in non-cirrhotic livers. Hepatic resection or transplantation offers the only chance of cure. We reviewed our experience of surgery for FL HCC from 1985-1998. Twenty patients with FL HCC (13 females and 7 males) median age 27 years (range 12-69) were treated either by hepatic resection [n = 11; extended right hepatectomy (5), extended left hepatectomy (1), right hemihepatectomy (2), left hemihepatectomy (2), left lateral segmentectomy (1)] or, if the disease was non-resectable, by transplantation (n = 9). The median follow up was 25 months (1-63). The prognostic factors analysed included size [less than 5 cm (3 patients), more than 5 cm (17 patients)], number [solitary (16 patients), multiple (4 patients)], capsular invasion (6 patients), vascular invasion (11 patients) and lymph node invasion (6 patients). The overall survival at 1, 3 and 5 years was 89.5, 75 and 50%, respectively. The liver resection survival was better than liver transplantation survival at 3 years 100 vs 76%, respectively (P < 0.025). Although all prognostic factors analysed did not show a significant difference, there is tendency that tumour stage was the most significant for prognosis. Most of the patients in this study are young and presented without specific symptoms, with normal liver function range and had no tumour marker to help in diagnosis. As a result most of our patients were diagnosed late. However the outcome of surgical intervention was favourable.
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PMID:Outcome of liver resection and transplantation for fibrolamellar hepatocellular carcinoma. 1111 43

Fibrolamellar hepatocellular carcinoma (FLC) is a rare entity of hepatocellular carcinoma (HCC) not yet analyzed cytogenetically. By using comparative genomic hybridization (CGH), we looked for chromosome changes in 2 primary FLCs and a recurrent FLC with and without metastases. CGH revealed an amplification of 1q in 1 primary FLC. The other primary FLC and a metastasis revealed no changes. The recurrent FLC showed 18 aberrations, including 1q+, 2p+, 3p+, 3q+, 4p+, 4q+, 5p+, 5q+, 6q+, 8p+, 8q+, 9q+, 12p+, 12q+, 18p+, 18q+, Xp+, and Xq+. In 2 metastases, 9 and 10 aberrations were seen, including 1q+, 3p-, 3q-, 4q+, 5p+, 5q+, 8q+, 10p+, 10q+, Xp+, and Xq+. In 9 cases of other entities of HCC, a mean of 10.2 aberrations per case were detectable affecting 1q (7 cases), 4q (5), 5q (4), 6q (5), 8p (5), 8q (5), 9p (4), 9q (5), 16q (4), 17p (5), and 17q (4). Chromosomes 2p, 2q, 3p, 3q, 4p, 5p, 6p, 7p, 7q, 10q, 11p, 11q, 12p, 12q, 13q, 14q, 16p, 18p, 18q, 20p, 20q, and 21q were altered in up to 3 samples. Our findings indicate striking differences in the number of chromosomal imbalances in primary FLC and recurrent FLC, whereas imbalances seen in the recurrent FLC and the other entities of HCC were similar in number and chromosomes involved. It may be speculated that these aberrations represent secondary events based on a genetic instability and do not mirror the primary alterations in these carcinomas.
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PMID:Cytogenetic aberrations in primary and recurrent fibrolamellar hepatocellular carcinoma detected by comparative genomic hybridization. 1133 75

Fibrolamellar hepatocellular carcinoma (FHCC), a rare variant of hepatocellular carcinoma, is becoming more prevalent; however, up until 1999, only 18 cases had been reported in Japan. We recently diagnosed a case of FHCC in a 46-year-old Japanese man who had visited four hospitals before being finally admitted to our department. On admission, he was diagnosed as having multiple liver tumors, with lymph node metastasis and peritoneal dissemination. Both hepatitis B antigen and hepatitis C antibody were negative, and the levels of serum alpha-fetoprotein and PIVKA-II were within normal limits. Under a provisional diagnosis of atypical hepatocellular carcinoma (HCC), a right hepatic lobectomy with resection of the metastatic lymph nodes and peritoneal dissemination was performed. The histopathological diagnosis made by our pathologist was atypical HCC. He underwent another operation for a recurrence in the left external iliac lymph node. It has been 29 months since his first surgery in this hospital and he is progressing well, which led us to establish the diagnosis of FHCC. Moreover, his serum carbohydrate antigen 125 levels have been well correlated with this condition.
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PMID:Fibrolamellar hepatocellular carcinoma in a Japanese man: report of a case. 1199 50

A 6-year-old Malay boy presented with fever and abdominal pain for 2 months. Computerised tomography showed a nodular mass in the left lobe of the liver. There was also portal vein thrombosis on the left side. Serum alpha-fetoprotein was not elevated and Hepatitis B antigen was negative. Biopsy of the liver mass led to a histological diagnosis of fibrolamellar hepatocellular carcinoma. In view of extensive tumour involvement, he could not be operated on but was treated with chemotherapy. However, the tumour did not respond. While this is expected for fibrolamellar hepatocellular carcinoma, the possibility of the tumour having a component of ordinary hepatocellular carcinoma could not be excluded as the tumour was not resected. Fibrolamellar hepatocellular carcinoma is a rare histological subtype of hepatocellular carcinoma, associated with a better prognosis. It affects the younger age group and has no association with cirrhosis, hepatitis B virus infection or exposure to oral contraceptives, all of which are implicated in ordinary hepatocellular carcinoma. Serum alpha-fetoprotein level is usually within normal limits and other laboratory values are not contributory to the diagnosis. The diagnosis is usually suggested by radiographic studies viz. CT scan of the abdomen, which would show an irregular non-homogenous mass in the liver, and confirmed by histological examination. The most characteristic microscopical feature is fibrosis arranged in a lamellar fashion around polygonal and deeply eosinophilic neoplastic hepatocytes.
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PMID:Fibrolamellar hepatocellular carcinoma: a case report. 1216 92

No more than 11 cases of carcinosarcoma of the liver have been reported in the past 40 years that fulfill the definition of hepatocellular carcinoma combined with differentiated sarcomatous elements. Most cases consist of hepatocellular carcinoma with 1 to 2 heterologous elements. We report a case of a 51-year-old woman with liver carcinosarcoma consisting of 3 carcinomatous components and 4 sarcomatous components. Hepatocellular carcinoma, fibrolamellar type, was accompanied by neuroendocrine carcinoma (neuron-specific enolase and synaptophysin positive) and adenocarcinoma (cytokeratin 7 and 20 positive). The sarcomatous elements consisted of poorly differentiated spindle cell neoplasm (vimentin positive), leiomyosarcoma (smooth muscle actin positive), rhabdomyosarcoma (desmin positive), and osteosarcoma. To our knowledge, this is the first case of liver carcinosarcoma with this many differentiated heterologous features. There are differing views on the pathogenesis of this tumor. Findings in this case support the view that metaplasia of carcinomatous cells gives rise to the sarcomatous elements.
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PMID:Carcinosarcoma of the liver: a case report and review of the literature. 1591 31

Increased expression of epidermal growth factor receptor (EGFR), a transmembrane tyrosine kinase, is associated with tumor progression in many carcinomas. Epidermal growth factor receptor inhibitors have shown promise in treating some of these tumors. Fibrolamellar hepatocellular carcinoma (FL-HCC) is an aggressive neoplasm that occurs in young patients with no history of cirrhosis. This study examines the expression and gene copy number of EGFR in FL-HCC. Formalin-fixed, paraffin-embedded FL-HCC (n = 13) sections were stained with a monoclonal antibody against EGFR. Fluorescence in situ hybridization analysis was performed using probes against EGFR gene and centromeric region of chromosome 7 (CEP 7). Epidermal growth factor receptor and CEP 7 signals were counted in 50 tumor nuclei per case as well as 300 normal hepatocyte nuclei. The EGFR to CEP 7 signal ratio was calculated for each case. Most (92%, 12/13) of FL-HCC showed strong and diffuse staining with anti-EGFR antibody. Fluorescence in situ hybridization was informative in 11 cases, 10 of which showed extra EGFR gene copy numbers (mean, 3.69; range, 3.13-5.0). Epidermal growth factor receptor was overexpressed in all these cases. The mean number of EGFR signals per cell in FL-HCC was double that of normal hepatocytes (3.69 versus 1.80); the mean EGFR/CEP 7 ratio in tumor cells was 1.05. In conclusion, EGFR is strongly overexpressed on the cell membrane in nearly all cases of FL-HCC. Similar gains of chromosome 7 are observed, indicating that the extra EGFR gene copies are due to polysomy rather than gene amplification. The strong expression of EGFR in FL-HCC tumors suggests that they may respond to treatment with EGFR antagonists.
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PMID:Epidermal growth factor receptor expression and gene copy number in fibrolamellar hepatocellular carcinoma. 1656 14


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