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)

Congenital extrahepatic portosystemic shunt (CEPS) or Abernethy malformation is a rare condition in which splanchnic venous blood bypasses the liver draining directly into systemic circulation through a congenital shunt. Patients may develop hepatic encephalopathy (HE), pulmonary hypertension (PaHT), or liver tumors, among other complications. However, the actual incidence of such complications is unknown, mainly because of the lack of a protocolized approach to these patients. This study characterizes the clinical manifestations and outcome of a large cohort of CEPS patients with the aim of proposing a guide for their management. This is an observational, multicenter, international study. Sixty-six patients were included; median age at the end of follow-up was 30 years. Nineteen patients (28%) presented HE. Ten-, 20-, and 30-year HE incidence rates were 13%, 24%, and 28%, respectively. No clinical factors predicted HE. Twenty-five patients had benign nodular lesions. Ten patients developed adenomas (median age, 18 years), and another 8 developed HCC (median age, 39 years). Of 10 patients with dyspnea, PaHT was diagnosed in 8 and hepatopulmonary syndrome in 2. Pulmonary complications were only screened for in 19 asymptomatic patients, and PaHT was identified in 2. Six patients underwent liver transplantation for hepatocellular carcinoma or adenoma. Shunt closure was performed in 15 patients with improvement/stability/cure of CEPS manifestations. Conclusion: CEPS patients may develop severe complications. Screening for asymptomatic complications and close surveillance is needed. Shunt closure should be considered both as a therapeutic and prophylactic approach.
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PMID:Congenital Extrahepatic Portosystemic Shunts (Abernethy Malformation): An International Observational Study. 3121 75

Hepatocellular carcinoma (HCC) is among the five most frequent causes of cancer death worldwide, according to the WHO. The disease is related to alcohol abuse, viral infections, and other causes of cirrhosis, and unfortunately, in some developed countries, the incidence shows an increasing trend. Although the diagnosis of the HCC often relies upon the context of a chronic hepatopathy, some cases may present a silent course, and the initial symptoms ensue when the disease is in an advanced stage with no chance for any therapeutic attempt. The clinical picture of the HCC is varied, and unexpected forms may surprise the clinician. One of the unusual presentations of the HCC is shock by the blockage of the venous return to the right atrium by the inferior vena cava infiltration. Herein we present a case of an old patient who sought medical care complaining of dyspnea. The clinical workup disclosed a right thorax pleural effusion and imaging exams depicted a mass in the right hepatic lobe, invasion of the inferior vena cava (IVC) and the right atrium (RA). During the attempts of clinical investigation, the patient passed away. The autopsy disclosed an HCC involving the right hepatic lobe, with the invasion of the IVC and the RA. The authors highlight the importance of recognizing the bizarre presentation of not so rare diseases.
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PMID:Progressive dyspnea and a right atrial mass in an 80-year-old man. 3180 38

Hepatocellular carcinoma (HCC) with cardiac metastasis represents a group of rarity and poor prognosis. Few case reports have described this patient entity, and it remains unclear regarding the optimal treatment and predicted outcome for these patients. In our case, we represented a 67-year-old female patient with presentations of dyspnea and heart murmur, which conducted to the diagnosis of advanced HCC with isolated right ventricular metastasis. Because of multiple comorbidities regarding cardiac and pulmonary systems, she received best supportive care and survived 2 months after initial diagnosis. A systemic literature review of 80 published cases (including our patient) since the 1950s was also performed from PubMed, and the data were gathered from the medical record and literature reviews. In our review, patients with advanced HCC and cardiac metastasis involved the right heart mainly with a predominance of right atrium (53%). Meanwhile the overall 3-month survival rate in our review was 70.7%. In subgroup analysis, the overall 3-month survival was highest (97%) in patients treated with surgery and other therapies, and lowest (27%) in patients with best supportive care. To our knowledge, this is the first comprehensive literature review addressing the epidemiology, optimal treatment, and prognosis for advanced-stage HCC with cardiac metastasis. We suggest that abnormal cardiac murmur in patients with history of HCC should prompt investigation for tumor involvement of the heart. We also emphasize individualized treatment as well as prognostic measurement accordingly.
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PMID:Isolated Right Ventricular Metastasis in a Woman with Advanced Hepatocellular Carcinoma after Palliative Therapy. 3191 61

BACKGROUND Postoperative pulmonary embolism following liver transplantations is still one of the most fatal complications, especially during the early postoperative phase. The use of a thrombolytic agent such as the recombinant tissue-type plasminogen activator (rtPA) is considered a contraindication early after major abdominal surgery such as liver transplantation. However, thrombolysis after major surgery in the early postoperative period carries a substantial risk of surgical site hemorrhage. CASE REPORT A 55-year-old patient presented with a hepatic mass diagnosed as a combined cholangio/hepatocellular carcinoma. Following the criteria of the University of San Francisco, California (UCSF) for liver transplantation, the case was selected for liver transplantation. The patient received neoadjuvant therapy. After the liver transplantation, on the second postoperative day, an acute, severe dyspnea with sudden onset occurred on the surgical ward. A computed tomography angiography showed a drawn-out embolus, which sprawled into both pulmonary main arteries and occluded them subtotally. A thrombolysis with rtPA was started. Within the first 60 minutes of administration of rtPA, the circulation stabilized effectively, so that epinephrine could be tapered down to zero and the patient was promptly extubated. About 6 hours after administration of rtPA, a sudden and pronounced bleeding via one of the intraperitoneal drains occurred, hemoglobin concentration dropped from 9.7 g/dL to 6.4 g/dL. After immediate re-laparotomy, circulation and hemoglobin concentration were absolutely stable. CONCLUSIONS Even with anticipated high risk of bleeding, thrombolysis with rtPA can be used as a life-savings treatment in a case of pulmonary embolism after liver transplantation.
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PMID:Thrombolysis in Postoperative Pulmonary Embolism Following Liver Transplantation: A Case Report. 3206 51

Immunoglobulin G4 (IgG4) -related disease (IgG4-RD) is a recently characterized entity in which lymphocytes and plasma cells infiltrate various anatomical sites. IgG4-hepatopathy, which is the manifestation of IgG4-RD, is a broader term covering various patterns of liver injury. The clinical course including the malignant potential of IgG4-RD remains unclear. Here we report the first case of secondary hemochromatosis and hepatocellular carcinoma (HCC) developed from IgG4-hepatopathy. A 67-year-old man was admitted to our hospital due to deteriorated glucose tolerance. Blood test results showed hypergammaglobulinemia, especially IgG4. He was readmitted 2 months later with dyspnea due to lung disease and pleural effusion, with elevated transaminase levels. He underwent liver and lung biopsies and was diagnosed with IgG4-RD, and received steroid therapy, which improved his serum IgG4 levels and imaging abnormalities. A follow-up computed tomography (CT) scan conducted 38 months later demonstrated a 50-mm-diameter tumor segments 7 and 8 of the liver. The resected specimen revealed HCC and abundant siderosis in the background liver, leading to a diagnosis of hemochromatosis. IgG4-positive cells were scarce, probably due to corticosteroid therapy. In the present case, IgG4-RD was well controlled with prednisolone (PSL) and immunosuppressive agent, and chronic hepatitis was not so severe, even though the patient subsequently developed HCC. However, extensive siderosis consistent with hemochromatosis was unexpectedly noted. It is suggested that secondary hemochromatosis and HCC developed during IgG4-RD with hepatopathy. Here we report the present case because it contributes to our understanding of IgG4-RD.
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PMID:Secondary Hemochromatosis and Hepatocellular Carcinoma Developed from Immunoglobulin G4-Related Disease with Hepatopathy: A Case Report. 3274 4


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