Hirotsugu Ikawa

Epithelioid hemangioendothelioma with marked liver deformity and secondary Budd–Chiari syndrome: Pathological and radiological correlation

A case of malignant epithelioid hemangioendothelioma of the liver in a 48‐year‐old woman with severe portal hypertension and marked deformity of the liver is presented. This woman had a history of mild liver dysfunction since the age of 30 years, and abdominal distention, esophageal varices, splenomegaly and ascites since October 1996. Imaging examinations revealed liver deformity with severe atrophy of the left lobe and the anterior segment of the right lobe. Celiac arteriography showed narrowing and upward deviation of the proper hepatic artery, and occlusion of the left and right anterior hepatic arteries. Since March 1997, hepatic venography showed stenosis in the right hepatic vein truncus. Budd–Chiari syndrome was clinically diagnosed. She died in June 1997. The autopsy disclosed massive tumor embolism in the left and right anterior portal branches, few in the hepatic artery, and occlusion of the left and right anterior hepatic arteries. The extensive tumor embolism resulted in portal hypertension, and atrophy of the left lobe. The anterior segment of the right lobe was probably caused by the occlusion of both the hepatic arteries and the portal veins. The posterior segment of the right lobe, without massive tumor embolism in its portal branch, appeared hypertrophic.

Superiority of CT Arterioportal Angiography to Contrast-Enhanced CT and MRI in the Diagnosis of Hepatocellular Carcinoma in Nodules Smaller than 2 cm

To evaluate the effectiveness of computed tomography (CT) arterioportal angiography in the diagnosis of hepatocellular carcinoma (HCC) in nodules smaller than 2 cm, we compared the findings of CT during arteriography (CTA) and CT during arterial portography (CTAP) with those of enhanced CT and enhanced magnetic resonance imaging (MRI). Sixty-eight nodules smaller than 2 cm in 53 patients with liver cirrhosis were classified into three groups of CTA and CTAP: (group 1) hyperattenuation on CTA, and hypoattenuation on CTAP (56 nodules, 41 patients); (group 2) hypoattenuation on CTA, and hypoattenuation on CTAP (10 nodules, 10 patients); (group 3) hypoattenuation on CTA, and hyperattenuation on CTAP (2 nodules, 2 patients). Histologically, 96% (54/56), 80% (8/10), and 100% (2/2) of the nodules in groups 1, 2 and 3, respectively, were diagnosed as HCC. In group 1, enhanced CT or enhanced MRI confirmed hypervascularity in only 77% (30/39) and venous washout in 21% (8/39). In groups 2 and 3, enhanced CT or enhanced MRI on 7 and 2 nodules, respectively, revealed no hypervascularity (0%). The results suggested that CT arterioportal angiography is superior to enhanced CT and MRI in nodules smaller than 2 cm for diagnosing HCC (p < 0.01 group 1, p < 0.01 group 2).

Autopsy case of alcoholic hepatitis and cirrhosis treated with corticosteroids and affected by Pneumocystis carinii and cytomegalovirus pneumonia

A case of the very early phase of Pneumocystis carinii pneumonia in a human immunodeficiency virus (HIV)‐negative man with alcoholic hepatitis and cirrhosis treated with steroids is presented. A 40‐year‐old man with a 10‐year history of alcohol abuse was admitted to hospital with jaundice, fever and macrohematuria. Laboratory examinations revealed neutrophilic leukocytosis and a serum bilirubin level of 13.9 mg/dL. The serum bilirubin level rose to 28.5 mg/dL over 1 month. Prednisolone administered orally for 10 days produced a slight improvement in the jaundice and fever. After an interval of a week, it was resumed and maintained for 22 days (total dose, 1555 mg) until the patient died of a massive hemorrhage from ruptured vessels of a gastric ulcer. An autopsy disclosed P. carinii pneumonia in the lower lobe of the left lung, cytomegalovirus infection in both lungs and the esophagus, and esophageal candidiasis. To our knowledge, this is the first report of P. carinii pneumonia together with cytomegalovirus infection in an HIV‐negative alcoholic patient. The present case suggests that a rare opportunistic infection such as P. carinii pneumonia might be caused by treating cirrhosis and alcoholic hepatitis with corticosteroids, even if only for a relatively short period.

CEA producing primary hepatic carcinoid

Imaging studies of a hepatic tumor in a 53-year-old woman with elevated serum levels of neuron-specific enolase (NSE), carcinoembryonic antigen (CEA) and 5-hydroxyindole acetic acid (5HIAA) revealed a hypervascular tumor in the right lobe. Grossly, the brownish tumor was measured 13.5x12 cm with four daughter nodules. Microscopically, the majority of these columnar and round tumor cells had ribbon-or rosette-like patterns with the expression of neuroendocrine marker proteins, such as Grimelius, NSE, chromogranin A, and synaptophysin, and moderate expression of CEA but without the expression of cytokeratin nos 7,8,14,18,19 and OV-6; the minority had glandular patterns with a strong expression of CEA but without the expression of cytokeratin nos 7,8,14,18,19 and OV-6. Ultrastructurally, most tumor cells contained populations of electron-dense core granules ranging between 100 and 200 nm in diameter. After hepatectomy, serum CEA, NSE, and 5HIAA reverted to normal ranges and persisted for 19 months. These findings suggested that the diagnosis of primary hepatic carcinoid was tenable and that the tumor might derive from hepatic stem cells which acquired the additional nature of producing CEA without cytokeratins characteristic of hepatocytes or bile duct cells. Some molecular based approaches have attributed unique biological behavior and histogenesis to this carcinoid tumor.

Development of multicentric hepatocellular carcinoma after completion of interferon therapy

6 international units (IU) of IFNα, 3 days a week for a total of 24 weeks. After the IFN therapy, the patient demonstrated a normal serum ALT level, and was continuously negative for HCV-RNA, and histology improved from chronic active hepatitis to chronic persistent hepatitis. Follow-up studies with ultrasonography (US) every 3 months and computed tomography (CT) every 6 months revealed no space-occupying lesion (SOL) for 3 years after IFN treatment.US-guided biopsies of two 15-mm hypoechoic SOLs in segments eight (S8) and seven (S7) 34 and 74 months, respectively, after IFN treatment showed well-differentiated hepatocellular carcinoma (HCC). Clinical data, imaging studies, and histologic examinations showed that both tumors were multicentric HCC. Further studies may provide insights into the possible role of HCV in hepatocarcinogenesis in patients demonstrating HCV eradication by IFN treatment.

Small hepatocellular carcinoma presenting with massive metastasis in the peritoneum, mimicking sarcomatous tumor

The case of a 51‐year‐old man with hepatitis C virus (HCV)‐related hepatocellular carcinoma metastasizing to the peritoneal cavity and mimicking a sarcomatous tumor is presented. A 12 × 12 cm mass, disclosed by computed tomography (CT), in the peritoneal cavity was predominantly isodense to muscle but had hypodense areas that suggested necrosis. T1‐weighted magnetic resonance imaging (MRI) showed a large mass, slightly hyperintense to muscle, with local hyperintense areas of suspected hemorrhagic necrosis.T2‐weighted MRI of the same region revealed a markedly non‐homogeneous and hyperintense mass with inner high signals and peripheral brush‐like linear striations. From such imaging studies, sarcomatous tumors, such as fibrosarcoma, leiomyosarcoma, and gastrointestinal stromal tumors, can be distinguished. Pathological findings at autopsy revealed necrotic tissue with a small portion of moderately differentiated HCC. Further studies may provide insights into the metastatic modes of HCC.