Protein induced by vitamin K absence or antagonist II (PIVKA-II) producing large cell neuroendocrine carcinoma (LCNEC) of lung with multiple liver metastases: A case report.

A 78-year-old man was admitted to our hospital for multiple lung and liver tumors. Initial clinical diagnosis was hepatocellular carcinoma (HCC) with lung metastases because of a high value of serum protein induced by vitamin K absence or antagonist II (PIVKA-II) (6,705 mAU/mL). However, a review of a prior CT showed the lung tumor had existed 6 months before liver tumors were detected. The tumors progressed rapidly and the patient died 37 days after admission. Autopsy revealed that both lung and liver tumors exhibited the histology of large cell neuroendocrine carcinoma (LCNEC). Immunohistochemistry revealed that the tumor cells expressed not only neuroendocrine markers but also PIVKA-II diffusely. Hepatoid differentiation was not detected. Background liver did not show any chronic liver disease. The final diagnosis was PIVKA-II producing LCNEC of the lung with multiple liver metastases. PIVKA-II producing tumors other than HCC are extremely rare. To our best knowledge, this is the first case report of PIVKA-II producing neuroendocrine tumors of the lung.

Integrated fibrosis scoring by ultrasonography predicts the occurrence of hepatocellular carcinoma in patients with chronic hepatitis C virus infection.

PURPOSE: This study was performed to elucidate whether evaluating the liver surface, edge, and texture by high-resolution ultrasonography is useful for predicting the occurrence of hepatocellular carcinoma (HCC) in patients with hepatitis C virus (HCV)-associated chronic liver diseases (CLDs) METHODS: The integrated fibrosis stage (a comprehensive value of scores for liver edge, surface, and texture) of 337 patients with HCV-associated CLDs was evaluated, at entry, by ultrasonography (US), as a US score. The patients were followed up prospectively (mean observation period was 16.4 months; range 2.8-36.2 months) for the occurrence of HCC by US or helical CT at 3-month intervals. A total of 140 patients received interferon therapy, and the occurrence of HCC was compared between those with and without interferon therapy RESULTS: The annual incidence of HCC was 1.1, 5.5, and 10.2% in low, middle, and high US score groups, respectively. Univariate analysis showed that age, serum levels of total bilirubin, alpha-fetoprotein (AFP), platelet count, albumin, total cholesterol, and the US score were associated with HCC occurrence in the patients. A multivariate proportional hazard model revealed that only the middle and high US scores (p = 0.0922, hazard ratio 4.006, 95% CI 0.796-20.153 and p = 0.008, hazard ratio 7.991, 95% CI 1.721-37.10, respectively) and elevated AFP (p = 0.031, hazard ratio 2.774, CI 1.097-7.014) were independently associated with HCC occurrence. Our US scoring based on evaluation of the liver surface, edge, and texture was clearly and strongly associated with the occurrence of HCC in patients with HCV-associated CLDs, and with the higher occurrence rate of HCC in patients with higher US scores CONCLUSION: Thus, US is a good tool for evaluating the fibrosis stage of the liver, and may therefore be useful in designing an optimum follow-up interval for each patient with HCV-associated CLD.

Acute dissecting thoracic aortic aneurysm in a patient with polymyalgia rheumatica.

Giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) are closely related disorders found in older patients, and vasculitis has been proposed as a part of the pathogenesis of PMR. We describe a female patient with PMR plus aortitis, both of which were well controlled on maintenance steroid therapy. Six months after the onset of her condition, however, she suddenly presented with chest pain. A diagnosis of dissecting aortic aneurysm was confirmed, and the aorta was successfully resected. Histology revealed infiltration of mononuclear cells including giant cells around the vaso vasorum with disruption of elastic lamina of the resected aorta. PMR or GCA may indicate an increased risk for aortic dissection in patients with normal erythrocyte sedimentation rate or C-reactive protein, and prompt recognition and therapy, not only during the active disease but also after symptoms of PMR have resolved, are needed.

CT findings of ruptured intramural hematoma of the aorta extending along the pulmonary artery.

Mediastinal hematoma extending along the pulmonary artery is a rare complication of Stanford type A classic (double-barreled) aortic dissection. Rupture from the posterior aspect of the aortic root penetrates the shared adventitia of the aorta and pulmonary artery. From this location, hematoma can spread along the adventitial planes of the pulmonary arteries out into the lungs. We report a case of ruptured intramural hematoma of the aorta (IMH) extending along the pulmonary artery. To our knowledge, this finding in patients with IMH has not been reported in the literature.

CT analysis of the growth rate of aortic diameter affected by acute type B intramural hematoma.

OBJECTIVE: The purpose of this study was to evaluate the growth rate of aortic diameter affected by acute type B intramural hematoma and the factors that influence its enlargement. MATERIALS AND METHODS: Fifty-four patients were entered into this study, and regular follow-up CT studies (mean +/- SD, 46.9 +/- 27.2 months; range, 5-136 months) were performed. The affected aortas and iliac arteries were divided into five segments. A total of 127 segments were evaluated (aortic arch, n = 47; descending thoracic aorta, n = 51; suprarenal abdominal aorta, n = 24; infrarenal abdominal aorta, n = 3; and iliac artery, n = 2). The growth rate of each segment was obtained on CT. The factors influencing increase in the diameter and growth rate--age, sex, diabetes mellitus, atherosclerotic disease, history of smoking 20 years, chronic renal failure, blood pressure, initial diameter of the lumen, the presence of blood flow in the false lumen--were evaluated by univariate analysis and a multivariate logistic regression model. RESULTS: Twenty (37.0%) of 54 patients had one or more segments that increased in size during the follow-up period. Of a total of 127 segments, 35 (27.6%) increased in size, and for all, the mean growth rate was -0.5 +/- 2.9 mm/year. This negative growth rate represents shrinkage. The mean growth rates within the first year and after 1 year from onset were -2.2 +/- 5.7 and 0.4 +/- 3.2 mm/year, respectively, and a significant difference was observed between them (p < 0.0001). An initial diameter of 40 mm or greater and the presence of blood flow in the false lumen were significant risk factors for an increase in the diameter, as confirmed by univariate and multivariate analyses. CONCLUSION: In patients with type B intramural hematoma, the affected aortas did not show a high incidence of enlargement during the follow-up period, but the affected aortas tended to increase in size after 1 year from onset. An initial diameter of 40 mm or greater and the presence of blood flow in the false lumen were important risk factors for enlargement during the follow-up period.

Catastrophic transverse myelitis in a patient with systemic lupus erythematosus.

A 24-year-old Japanese woman was admitted to our hospital suffering from high fever and progressive paralysis in both legs. Magnetic resonance imaging of the spinal cord showed high-intensity signals from C5 to Th4 and from Th7 to L1 on T2-weighted images. The patient was diagnosed as having acute transverse myelitis trade mark, which was a complication of systemic lupus erythematosus based on the serological findings. Despite aggressive immunosuppressive treatments including corticosteroid pulse therapy, plasmapheresis, and intravenous cyclophosphamide, the paralysis of her lower extremities did not improve. In the catastrophic type of lupus-associated TM, which develops extensively and longitudinally along the spinal cord, the prognosis still seems to be poor despite intensive treatments.

Intrahepatic biloma formation (bile duct necrosis) after transcatheter arterial chemoembolization.

OBJECTIVE: The purpose of our study was to discuss the incidence, predisposing factors, and clinical course of intrahepatic biloma after transcatheter arterial chemoembolization for hepatic tumors including hepatocellular carcinoma and metastatic liver tumor. MATERIALS AND METHODS: Nine hundred seventy-two patients with hepatocellular carcinoma (n = 920) or metastatic liver tumor (n = 52) underwent chemoembolization during a 12-year period beginning in January 1989. We retrospectively reviewed the medical records and follow-up radiographs of chemoembolization and analyzed the risk factors associated with the development of intrahepatic biloma. RESULTS: Intrahepatic biloma developed after chemoembolization in 35 patients (3.6%, 35/972) in our series. The incidence of intrahepatic biloma formation in patients with metastatic liver tumor (9.6%, 5/52) was higher than that in patients with hepatocellular carcinoma (3.3%, 30/920) (p < 0.05, Fisher's exact test). The incidence of intrahepatic biloma formation in patients with hepatocellular carcinoma was statistically higher in patients with main tumor size of less than 5 cm and in those with the presence of intrahepatic bile duct dilatation. Technique-related risk factors such as injection site of drugs, repeated chemoembolization with frequency of less than 3 months, and regimen of chemoembolization significantly influenced the incidence of biloma formation in patients with hepatocellular carcinoma. No patient died of infected biloma or septicemia, but one patient died of hepatic failure 2 months after chemoembolization. CONCLUSION: Biloma formation was significantly more prevalent in the metastatic lesion group than in the hepatocellular carcinoma group. Significant prognostic factors for biloma formation in patients with hepatocellular carcinoma were tumor size of less than 5 cm, bile duct dilatation, proximal injection site, repeated injection with frequency of less than 3 months, and injection of a suspension of anticancer drugs.