Membranous obstruction of the inferior vena cava and invasion of the inferior vena cava by tumour in hepatocellular carcinoma

A 28 yr old Zulu presented with a painful swelling in the right hypochondrium and severe swelling of the legs of short duration. The serum alpha-fetoprotein concentration was over 2 X 10(5) ng/ml and imaging showed a large hepatic mass-lesion. Radionuclide venography revealed no flow through the inferior vena cava but flow through a large collateral vessel. Contrast venography showed the upper portion of the inferior vena cava to be occluded: large collateral vessels arose from the lower vena cava and the iliac veins. The histological features were those of longstanding hepatic venous outflow obstruction with irregular centrizonal and portal fibrosis: severe acute centrizonal congestion was not seen. This combination of findings indicates the presence of both membranous obstruction of the inferior vena cava, a rare developmental abnormality which predisposes to hepatocellular carcinoma formation, and invasion by the tumour of the inferior vena cava via the hepatic veins, an uncommon complication of hepatocellular carcinoma.     

Inferior Vena Caval and Right Atrial Thrombosis Complicating an Amebic Hepatic Abscess

We describe a 50-yr-old black laborer who presented with right lower chest pain, weight loss, and pedal edema. Ultrasonography and computed tomograms showed a large abscess cavity in the right lobe of the liver which extended very close to the inferior vena cava. The lumen of the adjacent inferior vena cava was partially occluded by thrombus, which could be traced up into the cavity of the right atrium. The hepatic veins were normally patent. Sterile blood-stained pus was aspirated from the abscess. Antibodies against Entamoeba histolytica were present in high titer in the patient's serum. Although propagation of hepatocellular carcinoma into the inferior vena cava and even up into the right atrium is well recognized, inferior vena caval thrombosis extending up into the right atrium has not hitherto been reported as a complication of amebic hepatic abscess.     

Aggressive surgical resection for hepatocellular carcinoma with tumor thrombus extending to inferior vena cava and synchronous pulmonary metastasis

We describe a 66-year-old man having hepatocellular carcinoma with tumor thrombus extending into the inferior vena cava and synchronous pulmonary metastasis. He was referred to Chiba University Hospital on May, 2000, complaining of emaciation. Radiological findings showed a huge hepatocellular carcinoma in the entire right lobe and tumor thrombus extended into the intrapericardial inferior vena cava. He also had a solitary pulmonary metastasis in the left pulmonary lobe (stage IVB). Right hemihepatomy was performed under total hepatic vascular exclusion without cardiopulmonary bypass, and tumor thrombus was completely removed. Thoracoscopic wedge resection of pulmonary metastasis was also performed. The patient had an uneventful postoperative course. Histopathological examination revealed that the tumor was moderately differentiated hepatocellular carcinoma The patient is still alive after 26 months with pulmonary recurrence, but without hepatic recurrence. To our knowledge, there has been no reported case of resection for both hepatocellular carcinoma invading the inferior vena cava and synchronous pulmonary metastasis. In conclusion, aggressive surgical resection for advanced hepatocellular carcinoma concomitant with pulmonary resection may bring about better prognosis in highly selected patients.     

Retrohepatic vena cava deroofing in living donor liver transplantation for caudate hepatocellular carcinoma

The removal of tumor together with the native liver in living donor liver transplantation for hepatocellular carcinoma is challenged by a very close resection margin if the tumor abuts the inferior vena cava. This is in contrast to typical deceased donor liver transplantation where the entire retrohepatic inferior vena cava is included in total hepatectomy. Here we report a case of deroofing the retrohepatic vena cava in living donor liver transplantation for caudate hepatocellular carcinoma. In order to ensure clear resection margins, the anterior portion of the inferior vena cava was included. The right liver graft was inset into a Dacron vascular graft on the back table and the composite graft was then implanted to the recipient inferior vena cava. Using this technique, we observed the no-touch technique in tumor removal, hence minimizing the chance of positive resection margin as well as the chance of shedding of tumor cells during manipulation in operation.     

The Role of Myocardial Contrast Echocardiography to Assess the Origin of a Mass in Right Cardiac Cavities

We report the case of a patient with hepatocellular carcinoma who was admitted to our hospital with fatigue and edema of lower extremities. Transthoracic echocardiographic examination revealed a mobile echogenic cavoatrial mass that infiltrated the inferior vena cava and extended along the vessel protruding into the right cardiac cavities. The differential diagnosis included a tumor mass originating from the liver and subsequently infiltrating the inferior vena cava and extending into the right cardiac cavities or a large thrombus formed on the tumor mass that infiltrated the inferior vena cava.     

Budd-Chiari syndrome as a late complication following closure of an atrial septal defect

A case of a Budd-Chiari syndrome in a 19-year-old female patient is reported who had undergone surgical closure of a secundum atrial septal defect 13 years before. 8 months before the development of the Budd-Chiari syndrome she started to take oral contraceptives. The clinical picture of the Budd-Chiari syndrome developed within several days. The inferior vena cava did not fill with contrast dye when an angiography was performed using the right vena iliaca approach. The contrast dye disappeared through collateral veins (vena azygos, vena hemiazygos). After 4 days of treatment with systemic streptokinase she underwent open-heart surgery. The orifice of the inferior vena cava was occluded to a diameter of 6 mm. No thrombi were found. The lesion was corrected with two patches, one in the right atrium and the other in the inferior vena cava. This case report demonstrates that a Budd-Chiari syndrome is a possible late complication after closure of an atrial septal defect which should be treated by surgery.     

Budd-Chiari syndrome as the primary manifestation of a fibrolamellar hepatocellular carcinoma.

An 18-year-old female patient was admitted with ascites, right upper abdominal tenderness and peripheral edema. Angiography showed complete occlusion of the vena cava inferior up to the level of the right atrium. By open heart surgery, masses of thrombotic material were pulled out of the v. cava inferior/vv. iliacae which histologically contained tumor cell populations consistent with a hepatocellular carcinoma. Celiacography showed a highly vascularized tumor in the right hepatic lobe. Histologically, it proved to be fibrolamellar subtype hepatocellular carcinoma.     

Growth of hepatocellular carcinoma into the right atrium. Report of five cases

Five patients had hepatocellular carcinoma growing into the right atrium. Clinically, all patients had edema in the legs, venous dilatation in the abdominal wall, ascites, and dyspnea. Paroxysmal aggravation of dyspnea and its alleviation by a left decubitus position were noted in three patients. Three patients developed shock after a change in posture. A gallop rhythm in the cardiac murmur was detected in two. Pathologically, all livers had hepatocellular carcinoma and macronodular cirrhosis. At autopsy, a tumor thrombus was found that completely occluded the right hepatic vein and extended into the inferior vena cava and right atrium, partially occluding the inferior vena cava. Antemortem diagnosis of right atrial tumor thrombi in patients with primary hepatocellular carcinoma is difficult, but the condition should be suspected when dyspnea, abnormal cardiac sounds, and shock develop.     

Surgery of hepatoma with intracavitary cardiac extension

Summary A case of primary liver carcinoma with intracavitary cardiac extension is presented. A 36-year-old female was admitted to our surgical clinic with dyspnea and generalized edema. Echocardiography and superior vena cavography demonstrated a large filling defect in the right atrium. After a diagnosis of acute cardiac failure due to an intracardiac tumor, the patient was operated upon immediately. A right atriotomy exposed a large yellow mass within the right atrium, which was not adherent to the atrial wall. The mass was in continuity with similar material in the inferior vena cava and right hepatic vein. With a suspicion of hepatic malignancy, the atrial tumor was removed, and debulking of the mass in the inferior vena cava and right hepatic vein was performed. A postoperative histological examination of the tumor showed hepatocellular carcinoma. Her postoperative course was uneventful, and she was discharged from the hospital.Intracardiac extension of hepatoma is rarely encountered. In this clinical setting, long-term survival cannot be anticipated from any surgery, but palliative clearing of the atrium and inferior vena cava may be of value in preventing cardiac arrest causing sudden death.     

Small hepatocellular carcinoma with intravascular tumor growth into the right atrium

A 66-year-old man with ascites and marked edema in the lower extremities was suspected of having secondary Budd-Chiari syndrome due to primary liver cancer, based on imaging diagnosis, i.e., ultrasonography, computed tomography, and inferior venacavogram. At autopsy, an encapsulated small liver cancer was found to have extended into the inferior vena cava and right atrium. There have been few reports of small hepatocellular carcinoma with intravascular tumor growth into the right atrium.     

Membranous obstruction of the inferior vena cava and hepatocellular carcinoma in South Africa

This study was undertaken to establish the frequency of membranous obstruction of the inferior vena cava in South Africa, to characterize the pathology of the lesion and to define its relationship to hepatocellular carcinoma. Over a 9-yr period 101 cases of membranous obstruction of the inferior vena cava into the right atrium was occluded in all cases in which it was examined, and two basic patterns of the abnormality in the hepatic portion of the inferior vena cava have been described. The histologic picture in the liver was a chronic congestive fibrosis. In 44 cases of congestive fibrosis, diagnosed from liver biopsy specimens from black patients over a 5-yr period, vena caval membranes were demonstrated on cavography in 38 (86.4%). Hepatocellular carcinoma developed in 48 of the 101 cases (47.5%), indicating the importance of this defect in the pathogenesis of hepatocellular carcinoma in South Africa.     

Living-donor liver transplantation in Budd-Chiari syndrome with inferior vena cava complete thrombosis: A case report and review of the literature

BACKGROUND Budd-Chiari syndrome(BCS) is a challenging indication for liver transplantation(LT) due to a combination of massive liver,increased bleeding,retroperitoneal fibrosis and frequently presents with stenosis of the inferior vena cava(IVC).Occasionally,it may be totally thrombosed,increasing the complexity of the procedure,as it should also be resected.The challenge is even greater when performing living-donor LT as the graft does not contain the retrohepatic IVC;thus,it may be necessary to reconstruct it.CASE SUMMARY A 35-year-old male patient with liver cirrhosis due to BCS and hepatocellular carcinoma beyond the Milan criteria underwent living-donor LT with IVC reconstruction.It was necessary to remove the IVC as its retrohepatic portion was completely thrombosed,up to almost the right atrium.A right-lobe graft was retrieved from his sister,with outflow reconstruction including the right hepatic vein and the branches of segment V and VIII to the middle hepatic vein.Owing to massive subcutaneous collaterals in the abdominal wall,venovenous bypass was implemented before incising the skin.The right atrium was reached via a transdiaphragramatic approach.Hepatectomy was performed en bloc with the retrohepatic vena cava.It was reconstructed with an infra-hepatic vena cava graft obtained from a deceased donor.The patient remains well on outpatient clinic follow-up 25 mo after the procedure,under an anticoagulation protocol with warfarin.CONCLUSION Living-donor LT in BCS with IVC thrombosis is feasible using a meticulous surgical technique and tailored strategies.     

Curative resection by superior hepatectomy for advanced hepatoblastoma facilitated by the presence of a large inferior right hepatic vein

BackgroundDespite the success of neoadjuvant chemotherapy some patients with hepatoblastoma remain unresectable due to the proximity of important vascular structures. We report an unconventional surgical resection via a superior hepatectomy in a 16-month-old infant with hepatoblastoma.Case outlineStaging CT scan revealed extensive replacement of the superior portion of the liver with complete occlusion of the three hepatic veins, and with extension into the inferior vena cava and right atrium. Following chemotherapy the tumour was confined to the superior portion of the liver with obstruction of the right, middle and left hepatic veins, but with a large patent inferior hepatic vein draining the inferior liver segments. Superior hepatectomy was performed without complication.DiscussionComplete surgical resection offers the only chance of cure for patients with hepatoblastoma. This case illustrates that careful preoperative planning facilitated aggressive surgical clearance with superior hepatectomy for curative resection of an otherwise non-resectable tumour.     

An approach to intrapericardial inferior vena cava through the abdominal cavity, without median sternotomy, for total hepatic vascular exclusion.

In surgical resection for hepatocellular carcinoma with tumor thrombus extending into the inferior vena cava and other malignancies involving the retrohepatic inferior vena cava, the usefulness of total hepatic vascular exclusion has been reported by several authors. Total hepatic vascular exclusion usually consists of clamping at three points; at the infrahepatic inferior vena cava, at the suprahepatic inferior vena cava, and in Pringles' maneuver. Tumor thrombus extending into the inferior vena cava at the intrapericardial level below the right atrium can be resected without the use of cardio-pulmonary bypass. The inferior vena cava at the intrapericardial level has been reported to be usually approached by median sternotomy such as Chevron incision. We herein demonstrate an approach to the intrapericardial inferior vena cava through the abdominal cavity without median sternotomy.     

Renal cell carcinoma presenting as right atrial mass

Approximately 190 new cases of renal cell carcinoma infiltrate into the inferior vena cava and right atrium every year. Echocardiography often plays a major role in establishing the diagnosis and in guiding surgical therapy. This report describes a patient who presented with shortness of breath but had no classic signs or symptoms of renal cell carcinoma other than chronic anemia. Transthoracic two-dimensional echocardiography and transesophageal echocardiography established the diagnosis of a right atrial mass extending from the inferior vena cava that was later discovered to be renal cell carcinoma.     

Recurrent hepatocellular carcinoma presenting with superior vena cava syndrome

A 45-year-old male received wedge resection for his small hepatocellular carcinoma in April 1989 and extended right lobectomy for tumor recurrence 8 months later. Unfortunately, recurrent hepatic tumor with lung metastases were found 18 months after the second operation. Both the hepatic and pulmonary recurrent tumors were resected and transcatheter arterial embolization was added for the residual hepatic tumors. He remained symptom free for another 18 months. However, mediastinal lymphadenopathy, superior vena cava thrombus with superior vena cava syndrome, cardiac and brain metastases developed subsequently. He died of increased intracranial pressure. It is rare for hepatocellular carcinoma to have mediastinal metastases, superior vena cava thrombus and superior vena cava syndrome.     

Fatal thrombotic complications of hepatic cystic compression of the inferior vena： A case report

Of 5% of patients who develop liver cysts, only 10-15% of them come for medical attention, typically because of dull right upper quadrant pain, abdominal bloating or early satiety. We treated a 77-year-old female with a rare complication of inferior vena cava thrombosis. The patient expired due to septic shock and multiple organ failure.     

Growth of hepatocellular carcinoma into the right atrium. A case of antemortem diagnosis with magnetic resonance imaging of the heart

We report the unusual case of a 54-year-old man with a right atrial mass (detected by two-dimensional echocardiography) associated with hepatocellular carcinoma. The cardiac mass, following magnetic resonance imaging of the heart, was proved to be due to a direct extension of the liver tumour, via the inferior vena cava, up to the right atrial cavity. We wish to stress that the availability of magnetic resonance imaging renders possible the antemortem diagnosis of cardiac metastasis due to malignant tumours.     

Advanced adenosquamous carcinoma of the gallbladder with bilio-biliary fistula: an uncommon case treated by hepatopancreatoduodenectomy.

A 70 year-old female, who presented with jaundice and abdominal pain, was found to have an advanced gallbladder cancer involving the liver parenchyma, duodenum, and transverse colon. This was complicated by a bilio-biliary fistula between the gallbladder and both the right and left hepatic ducts. After obtaining an accurate pre-operative diagnosis, the patient underwent hepatopancreatoduodenectomy (HPD) with lymph node dissection around the hepatic pedicle, celiac trunk, aorta, and inferior vena cava. Histologic examination revealed adenosquamous carcinoma. This rare variant accounts for 3.5% of gallbladder cancers, and is associated with a worse prognosis than adenocarcinoma. The patient is in good condition without any signs of recurrence 42 months after the HPD. In this case report, we discuss the histological type and internal biliary fistula with regard to the literature, and the usefulness of an aggressive surgical procedure such as HPD with extended lymph node dissection which can improve survival and quality of life in selected patients.     

Multiple aortocaval fistulas associated with a ruptured abdominal aneurysm in a patient with Ehlers-Danlos syndrome.

Aortocaval fistula (ACF) is a rare complication of spontaneous abdominal aortic aneurysm (AAA) rupture, with an incidence of 2-4%. A unique case of ruptured AAA complicated by multiple aortovenous fistulas involving the inferior vena cava and left internal iliac vein is presented, and is the first published report of a patient with Ehlers-Danlos syndrome undergoing surgical treatment for an ACF.