Hepatocellular carcinoma with an unusual papillary vesicular appearance

A resected hepatocellular carcinoma with grossly papillary, whitish cut surfaces and 'vesicles' in a noncirrhotic but hepatitis B surface antigen positive liver is described. This appearance has not previously been reported in the literature. Such an unusual appearance may lead to an erroneous diagnosis by both surgeons and pathologists. Histological features and possible mechanisms for the unusual gross appearance are discussed.     

Hepatocellular carcinoma with unusual metastasis to the esophagus

We describe a rare case of esophageal metastasis from hepatocellular carcinoma. Esophagoscopy showed an oozing semipedunculated polypoid mass. Endoscopic ultrasound showed a mosaic pattern, and angiography and computed tomography showed tumor staining similar to that of the primary tumor in the liver. The present patient underwent superselective transcatheter arterial infusion of anticancer agents and permanent hemostasis was achieved.     

Hepatocellular carcinoma with an unusual medullary-like histology and signs of regression ("medullary-like hepatocellular carcinoma").

The case of a variant of hepatocellular carcinoma is described, which, based on its unique histology, we propose to term, medullary-like hepatocellular carcinoma. It developed in a 56-year-old male patient with liver cirrhosis, and consisted of large, amphophilic cells with a solid growth pattern. The tumour was densely infiltrated with lymphocytes and plasma cells. Lymphocytes formed a mixture of B and T cells, and plasma cells were polytypic. In addition, numerous S-100 protein-reactive stellate cells were observed at the tumour border, where marked apoptosis of hepatocellular carcinoma cells was evident. In areas of dense lymphoplasmacytic infiltration, part of the tumour cells had lost their intercellular connections and their beta-catenin reactivity. Some tumour cells expressed FasL, but not Fas. The tumour exhibited several foci of regression, showing small remnants of damaged tumour cells within dense infiltrations. The patient is alive without evidence of disease 25 months after resection. Medullary-like hepatocellular carcinoma is a lesion which mimics several features known for other medullary carcinomas, including a marked immune response which may be responsible for partial regression of this tumour.     

An unusual encounter of a "cobra" in the heart: rare appearance of an Amplatzer Septal Occluder

This article presents the unusual appearance of a "cobra" due to "acute bending" of the proximal part and partial opening of the distal part of the left atrial disk of an Amplatzer Septal Occluder during the process of transcatheter closure of a secundum atrial septal defect in a 6-year-old boy. The possible reasons and method to overcome this technical problem, which resulted in successful occlusion of the atrial septal defect, are discussed.     

Arterial microembolisation: an unusual presentation of dilated cardiomyopathy

Systemic embolisation is common in patients with dilated cardiomyopathy. Microembolisation as a presenting sign of dilated cardiomyopathy, however, has not been reported before. A 37 year old woman in whom dilated cardiomyopathy presented as arterial microembolisation to the toes is described.     

Hepatocellular carcinoma with polymyositis as an initial symptom: a case report

Patients with hepatocellular carcinoma may manifest paraneoplastic syndromes such as hypercholesterolaemia, hypoglycaemia, hypercalcaemia and erythrocytosis. An exceptional case of hepatocellular carcinoma associated with polymyositis, a rheumatic paraneoplastic syndrome, is reported. A 72-year-old male complained for a recent muscular weakness mainly in the proximal limb muscles. The clinical course, a raised level of serum creatine kinase and electromyographic findings suggested polymyositis, and the pathological findings on muscle biopsy were compatible with this diagnosis. Computed tomography of the upper abdomen revealed a mass lesion in segment IV, V and VII of the liver, and alpha foetoprotein level was high, resulting in the diagnosis of hepatocellular carcinoma.     

Hepatocellular carcinoma: an Asian perspective

Hepatocellular carcinoma (HCC) is one of the most frequently occurring malignancies in Asia. The incidence exceeds 30 cases/100,000/year in the east Asian region. Worldwide, it accounts for almost 1 million deaths/year. The high incidence in Asia is due to the high prevalence of chronic viral hepatitis, mainly chronic hepatitis B. With the introduction of universal vaccination for hepatitis B in some Asian countries in the mid 1980s, some of these countries are experiencing a decline in the incidence of HCC. This probably underscores the point that HCC caused by hepatitis B is a malignancy preventable by vaccine. Due to the relative paucity of symptoms in the early stages and the rapid doubling time of the tumor, most HCCs are discovered late in advanced stages at presentation. Most Asian countries have adopted a screening program for patients at risk. Earlier and smaller HCCs are detected through such programs but these programs have yet to demonstrate improved patient survival. Physicians managing patients with HCC are faced with two main challenges, the malignancy itself and the underlying liver disease. The extent of the tumor and the existing liver function limits the therapeutic choices available. Hepatic resection remains the treatment of choice. However, the majority of patients present with nonresectable tumors. Transarterial chemoembolization, percutaneous ethanol injection and radiofrequency ablation are the other treatment modalities. In patients with small tumors (<5 cm) and poor liver function, liver transplant offers a viable treatment alternative. In summary, the risk factor for HCC in Asia is predominantly chronic hepatitis B. Universal vaccination against hepatitis B is likely to reduce the incidence. The prognosis and outcome of treatment remains poor with a 5-year survival of 35% for patients treated surgically and less than 10% for nonresectable tumors. Current management is aimed at earlier detection and more effective treatment of early HCC. In future, the challenge will be managing HCC in the premalignant stage.     

Crystal structure of peanut lectin, a protein with an unusual quaternary structure.

The x-ray crystal structure of the tetrameric T-antigen-binding lectin from peanut, M(r) 110,000, has been determined by using the multiple isomorphous replacement method and refined to an R value of 0.218 for 22,155 reflections within the 10- to 2.95-A resolution range. Each subunit has essentially the same characteristic tertiary fold that is found in other legume lectins. The structure, however, exhibits an unusual quaternary arrangement of subunits. Unlike other well-characterized tetrameric proteins with identical subunits, peanut lectin has neither 222 (D2) nor fourfold (C4) symmetry. A noncrystallographic twofold axis relates two halves of the molecule. The two monomers in each half are related by a local twofold axis. The mutual disposition of the axes is such that they do not lead to a closed point group. Furthermore, the structure of peanut lectin demonstrates that differences in subunit arrangement in legume lectins could be due to factors intrinsic to the protein molecule and, contrary to earlier suggestions, are not necessarily caused by interactions involving covalently linked sugar. The structure provides a useful framework for exploring the structural basis and the functional implications of the variability in the subunit arrangement in legume lectins despite all of them having nearly the same subunit structure, and also for investigating the general problem of "open" quaternary assembly in oligomeric proteins.     

Arterial microembolisation: an unusual presentation of dilated cardiomyopathy.

Systemic embolisation is common in patients with dilated cardiomyopathy. Microembolisation as a presenting sign of dilated cardiomyopathy, however, has not been reported before. A 37 year old woman in whom dilated cardiomyopathy presented as arterial microembolisation to the toes is described.     

Double left innominate vein: an unusual cross-sectional echocardiographic appearance

A patient is described with ventricular septal defects and two innominate veins which gave rise to an unusual cross-sectional echocardiographic finding thought to be due to a left superior caval vein without connexion to the coronary sinus.     

Hepatocellular carcinoma and cholangiocarcinoma: an update

Hepatocellular carcinoma (HCC) is the third most common cause of cancer worldwide and is rising in incidence. Ultrasound is the preferred modality for screening high-risk patients for HCC because it detects clinically significant nodules, widespread availability and lower cost. HCC does not require a biopsy for diagnosis if specific imaging criteria are fulfilled. Transarterial chemoembolization (TACE) is the most common modality used to treat HCC followed by ablation. Cholangiocarcinoma (CCA) is increasing in incidence and the second most common primary malignancy of the liver. There is no effective screening strategy for CCA although magnetic resonance imaging and carbohydrate antigen 19-9 (CA 19-9) are commonly used without proven benefit. Therapy for CCA is challenging and resection, when possible, is the mainstay of therapy. Gemcitabine in combination with cisplatin or biologics may offer a modest survival benefit. Liver transplantation for CCA is associated with reasonable survival in select cases. Molecular diagnostics offer the potential to develop personalized approaches in the management of HCC and CCA.     

Hepatocellular carcinoma with nonalcoholic steatohepatitis

Nonalcoholic steatohepatitis (NASH) was originally believed to be a benign disease. However, it has been recently revealed that NASH could lead to irreversible liver disease in some patients. We report an unusual case of hepatocellular carcinoma (HCC) in a 76-year-old man with NASH. He had no history of alcohol consumption, drug use, or blood transfusion. He was negative for all serological viral markers and autoantibodies. In addition, he was obese (body mass index [BMI], 30.75kg/m²) and had type 2 diabetes mellitus. A liver biopsy specimen showed moderate steatosis with necroinflammatory changes, ballooning degeneration, Mallory bodies, pericellular fibrosis, and evidence of nodular regeneration. He was diagnosed with NASH with cirrhosis. Simultaneously, a liver tumor, measuring 19mm in diameter, was detected in segment 6. A tumor biopsy specimen revealed well-differentiated HCC, and imaging modalities confirmed the characteristics of HCC. To our knowledge, ten patients who had HCC with NASH were reported. In all patients with NASH and HCC, cirrhosis was present. Patients with NASH and cirrhosis may progress to HCC, and regular screening, based on tumor markers and imaging modalities, is needed to detect HCC in patients with NASH and cirrhosis.     

Exogastric cystic gastric leiomyoblastoma with unusual CT appearance

Gastric leiomyoblastomas are rare tumors between leiomyomas and leiomyosarcomas in terms of their malignant potential. This article describes 2 cases of exogastric cystic gastric leiomyoblastoma with unusual location and computed tomographic (CT) appearance. The CT findings of these cases were unlike those of usual smooth muscle tumors, but closely simulated cystadenomatous tumor of the ovary or pancreas.     

Hepatocellular carcinoma with cutaneous metastases

Two cases of cutaneous metastases from hepatocellular carcinoma (HCC) are reported. Both patients had been diagnosed with HCC at least one year before the appearance of skin lesions. The lesions presented as small reddish nodules in both patients, with a large additional vascular lesion in one of the patients. Cutaneous metastases from HCC are very rare. However, these two cases suggest that patients with HCC and presenting with skin nodules should have biopsies performed to confirm the diagnosis.