Multiple hepatic metastases of cardiac angiosarcoma

The differential diagnosis of hepatic focal lesions is challenging because the etiology can be inflammatory, infectious, and even neoplastic. A rare cause of metastatic liver nodules is cardiac angiosarcoma. We report a case of this tumor, which was diagnosed only after autopsy. A 26-year-old Caucasian man was admitted for progressive dyspnea and cough over the past 3 weeks. Physical examination showed only hypophonetic heart sounds. Laboratory analysis demonstrated anemia and elevated inflammatory markers, despite normal biochemical parameters and liver function. Transthoracic echocardiography revealed massive pericardial effusion. Abdomen computed tomography (CT) showed multiple hepatic nodules, the largest of which measured 3 cm, but the percutaneous biopsy revealed only lobular necrosis and perisinusoidal fibrosis without granulomas or neoplastic cells. During hospitalization, the patient had fever and night sweats with weight loss, and empiric treatment for extrapulmonary tuberculosis associated with corticosteroids was initiated. The outpatient follow-up revealed complete improvement of the pericardial effusion, but maintenance of the liver lesions. After 2 months of hospital discharge, the patient was readmitted with hemorrhagic shock due to bleeding liver lesions, which were evidenced by CT. Embolization of the right hepatic artery was performed, but the patient soon died. The autopsy revealed a primary cardiac angiosarcoma with multiple hepatic metastases, rupture of the Glisson's capsule and laceration of the liver. The case shows how important and difficult the diagnosis of focal liver lesions is, since it may result in an unexpected fatal outcome.

Importância da biópsia hepática no diagnóstico da deficiência de lipase ácida lisossomal: relato de caso / Importance of liver biopsy in the diagnosis of lysosomal acid lipase deficiency: a case report

RESUMO Objetivo: Descrever a doença de depósito de ésteres de colesterol (DDEC) e a importância da biópsia hepática na realização do diagnóstico. Descrição do caso: Paciente feminina, dois anos e dez meses de idade, com queixa de aumento do volume abdominal secundário à hepatomegalia há quatro meses. Ultrassonografia abdominal demonstrou hepatomegalia e esteatose hepática. Exames laboratoriais mostraram aumento de enzimas hepáticas e dislipidemia. A biópsia hepática foi compatível com DDEC. Comentários: Embora a medida da atividade enzimática seja o padrão-ouro para o diagnóstico de DDEC, a biópsia hepática é muito útil na investigação de casos suspeitos, particularmente quando há outros diagnósticos diferenciais a serem considerados.

ABSTRACT Objective: To describe a case of cholesteryl ester storage disease (CESD) and discuss the importance of liver biopsy for diagnosis. Case description: A female patient, aged two years and ten months, presented with an increased abdominal volume following hepatomegaly for four months. Abdominal ultrasound demonstrated hepatomegaly and hepatic steatosis. Laboratory tests showed elevated liver serum enzymes and dyslipidemia. Liver biopsy was consistent with CESD. Comments: Although measuring enzyme activity is the gold standard for CESD diagnosis, liver biopsy is very helpful when investigating suspected cases of CESD, particularly upon other differential diagnoses to be considered.

Steatosis of indeterminate cause in a pediatric group: is it a primary mitochondrial hepatopathy? / Esteatose de causa não determinada em grupo pediátrico: hepatopatia mitocondrial primária?

CONTEXT AND OBJECTIVE: In children, hepatic steatosis may be related to inborn errors of metabolism (IEMs) or to non-alcoholic fatty liver disease (NAFLD). The aim of this study was to assess and characterize steatosis of indeterminate cause through morphological and morphometric analysis of liver tissue. DESIGN AND SETTING: Cross-sectional study at the Departments of Pathology of Faculdade de Ciências Médicas, Universidade Estadual de Campinas (FCM-Unicamp) and Faculdade de Medicina de Botucatu, Universidade Estadual Paulista (FMB-Unesp). METHODS: Eighteen consecutive liver biopsies obtained from 16 patients of ages ranging from 3 months to 12 years and nine months that were inserted in a database in the study period were analyzed using optical microscopy and transmission electron microscopy. Through electron microscopy, the mitochondrial density and mean mitochondrial surface area were determined in hepatocytes. Ten patients ranging in age from 1 to 14 years were used as a control group. RESULTS: "Pure" steatosis was detected, unaccompanied by fibrosis or any other histological alteration. Microvesicular steatosis predominated, with a significant increase in mean mitochondrial surface area. CONCLUSION: Microvesicular steatosis may be related to primary mitochondrial hepatopathy, especially due to reduction of β-oxidation or partial stagnation of oxidative phosphorylation. For these reasons, this form of steatosis (which should not be called "pure") is likely to represent an initial stage in the broad spectrum of NAFLD. We have drawn attention to cases of steatosis in the pediatric group, in which the microvesicular form predominates, since this may be associated with mitochondrial disorders.

CONTEXTO E OBJETIVO: Em crianças, a esteatose hepática pode se relacionar a erros inatos do metabolismo (EIMs) ou à doença hepática gordurosa não-alcoólica (DHGNA). O objetivo deste estudo foi avaliar e caracterizar esteatose de causa indeterminada por meio de análises morfológica e morfométrica em tecido hepático. TIPO DE ESTUDO E LOCAL: Estudo transversal nos Departamentos de Patologia da Faculdade de Ciências Médicas da Universidade Estadual de Campinas (FCM-Unicamp) e Faculdade de Medicina de Botucatu da Universidade Estadual Paulista (FMB-Unesp). MÉTODOS: Foram utilizadas 18 biópsias hepáticas consecutivas obtidas de 16 pacientes com idade variando de 3 meses a 12 anos e 9 meses, inseridas num banco de dados no período do estudo, que foram analisadas por microscopia óptica e eletrônica. Na microscopia eletrônica, foi realizada determinação da densidade mitocondrial e da área superficial média das mitocôndrias nos hepatócitos. Dez pacientes com idade variando de 1 a 14 anos foram usados como grupo controle. RESULTADOS: Foi detectada esteatose "pura", não acompanhada por fibrose ou outra alteração histológica. Foi verificado que, na predominância de esteatose microvesicular, houve aumento significativo da área mitocondrial média. CONCLUSÃO: A esteatose microvesicular pode estar relacionada à hepatopatia mitocondrial primária, principalmente devido à redução na β-oxidação ou parcial estagnação da fosforilação oxidativa. Por essas razões, esta forma de esteatose (que não pode ser chamada de "pura") possivelmente represente uma fase inicial no amplo espectro da DHGNA. Chamamos a atenção para casos de esteatose no grupo pediátrico com predomínio da forma microvesicular, uma vez que pode haver associação com desordens mitocondriais.

Doença hepática gordurosa não alcoólica: patogênese e achados histológicos com ênfase nas alterações mitocondriais: [revisão] / Nonalcoholic fatty liver disease: pathogenesis and histological findings with emphasis on mitochondrial alterations: [review]

A esteatose hepática não relacionada ao alcoolismo pode ocorrer isoladamente ou como parte da doença hepática gordurosa não alcoólica, abrangendo amplo espectro de alterações morfológicas e variando de esteatose até estádios mais graves (acompanhados por fibrose), podendo chegar à cirrose. A esteatose hepßtica não relacionada ao alcoolismo tem grande importância clínica na atualidade não só por sua elevada prevalência (15 por cento a 25 por cento na população geral e superior a 80 por cento entre obesos e diabéticos), mas também pela diversidade de condições relacionadas, tais como fatores nutricionais, hepatite pelo virus C, doenças metabólicas, toxicidade por drogas etc...

Sarcoma indiferenciado (embrionário) do fígado: relato de caso e revisão da literatura / Undifferentiated (embryonal) sarcoma of the liver: a case report and review of the literature

O sarcoma embrionário (indiferenciado) é uma neoplasia mesenquimal maligna incomum e primária do fígado, que acomete principalmente crianças na faixa etária entre 6 e 10 anos de idade. Este trabalho tem como objetivo relatar um caso dessa neoplasia, enfatizando os aspectos clínicos e anatomopatológicos, bem como revisar a literatura sobre o tema.

Embryonal (undifferentiated) sarcoma is a primitive and unusual malignant neoplasm of the liver that occurs mainly in children between 6 and 10 years of age. The aim of this case report is to describe one case of this neoplasm, emphasizing clinical and anatomopathological findings as well as review the literature about the theme.

The influence of occult infection with hepatitis B virus on liver histology and response to interferon treatment in chronic hepatitis C patients

Occult hepatitis B virus (HBV) infections have been identified in patients with chronic hepatitis C virus (HCV) infection, although the clinical relevance of occult HBV infection remains controversial. We searched for serum HBV DNA in 106 HBsAg negative/anti-HBc positive patients with chronic HCV infection and in 150 blood donors HBsAg negative/anti-HBc positive/anti-HCV negative (control group) by nested-PCR. HCV genotyping was done in 98 patients and percutaneous needle liver biopsies were performed in 59 patients. Fifty-two patients were treated for HCV infection with interferon alone (n=4) or combined with ribavirin (n=48) during one year. At the end and 24 weeks after stopping therapy, they were tested for HCV-RNA to evaluate the sustained virological response (SVR). Among the 106 HCV-positive patients, 15 (14 percent) were HBV-DNA positive and among the 150 HCV-negative blood donors, 6 (4 percent) were HBV-DNA positive. Liver biopsy gave a diagnosis of liver cirrhosis in 2/10 (20 percent) of the HBV-DNA positive patients and in 6/49 (12 percent) of the HBV-DNA negative patients. The degree of liver fibrosis and portal inflammation was similar in HCV-infected patients HBV-DNA, irrespective of HBV-DNA status. SVR was obtained in 37.5 percent of the HBV-DNA positive patients and in 20.5 percent of the HBV-DNA negative patients; this difference was not significant. In conclusion, these data suggested that occult HBV infection, which occurs at a relatively high frequency among Brazilian HCV-infected patients, was not associated with more severe grades of inflammation, liver fibrosis or cirrhosis development and did not affect the SVR rates when the patients were treated with interferon or with interferon plus ribavirin.

Viral hepatitis in patients infected with human immunodeficiency virus

From 1992 to 1995 we studied 232 (69 percent male, 87 percent Caucasian) anti-human immunodeficiency virus (anti-HIV) positive Brazilian patients, through a questionnaire; HIV had been acquired sexually by 50 percent, from blood by 32 percent, sexually and/or from blood by 16.4 percent and by an unknown route by 1.7 percent. Intravenous drug use was reported by 29 percent; it was the most important risk factor for HIV transmission. The alanine aminotransferase quotient (qALT) was >1 for 40 percent of the patients, 93.6 percent had anti-hepatitis A virus antibody, 5.3 percent presented hepatitis B surface antigen, 44 percent were anti-hepatitis B core antigen positive and 53.8 percent were anti-hepatitis C virus (anti-HCV) positive. The anti-HCV test showed a significant association with qALT>1. Patients for whom the probable HIV transmission route was blood had a 10.8 times greater risk of being anti-HCV positive than patients infected by other routes. Among 30 patients submitted to liver biopsy, 18 presented chronic hepatitis

A new method for the experimental induction of secundary biliary cirrhosis in wistar rats

The aim of this study was to describe a method for the induction of experimental secondary biliary fibrosis (SBF). Forty-seven Wistar rats were submitted to hepatic duct obstruction (OB group) for thirty days without ligature, section or cannulization causing interruption of biliary flow. This technique was carried out by simple traction of the bile duct passing it through the xiphoid appendix. Nine rats were submitted to a sham operation for bile duct stricture and seven rats comprised the control group. Blood samples were collected for the measurement of total bilirubin (TB), alkaline phosphatase (AP), alanine aminotransferase (ALT) and aspartate aminotransferase (AST). Liver fragments were removed for morphological study. Thirty days after surgery TB, AP, ALT and AST levels were significantly increased in the hepatic duct ligation group compared to the sham operated group and the presence of SBF in the OB group was confirmed by morphological study of the liver. There was technical failure in 31.92 percent cases. The survival was 100 percent at fifteen days and 82.97 percent at the end of the experiment. We concluded that this simple surgical technique may be used to study the consequence of bile duct obstruction which could be a reversible process depending on the obstruction time. This technique can be carried out from cholestasis to fibrosis.

Diagnosis of alpha-1-antitrypsin deficiency by DNA analysis of children with liver disease

BACKGROUND: Alpha-1-antitrypsin deficiency is a genetic disorder which is transmitted in a co-dominant, autosomal form. Alpha-1-antitrypsin deficiency affects mainly the lungs and the liver leading, in the latter case, to neonatal cholestasis, chronic hepatitis or cirrhosis. A precise diagnosis of Alpha-1-antitrypsin deficiency may be obtained by biochemical or molecular analysis. OBJECTIVE: The purpose of this study was to use DNA analysis to examine the presence of an alpha-1-antitrypsin deficiency in 12 children suspected of having this deficiency and who showed laboratory and clinical characteristics of the disease. PATIENTS AND METHODS: Twelve patients, aged 3 months to 19 years, who had serum alpha-1-antitrypsin levels lower than normal and/or had hepatic disease of undefined etiology were studied. The mutant alleles S and Z of the alpha-1-antitrypsin gene were investigated in the 12 children. Alpha-1-antitrypsin gene organization was analyzed by amplification of genoma through the polymerase chain reaction and digestion with the restriction enzymes Xmnl (S allele) and Taq-1 (Z allele). RESULTS: Seven of the 12 patients had chronic liver disease of undefined etiology and the other five patients had low serum levels of alpha-1-antitrypsin as well as a diagnosis of neonatal cholestasis and/or chronic liver disease of undefined etiology. Five of the 12 patients were homozygous for the Z allele (ZZ) and two had the S allele with another allele (*S) different from Z. CONCLUSION: These results show that alpha-1-antitrypsin deficiency is relatively frequent in children with chronic hepatic disease of undefined etiology and/or low alpha-1-antitrypsin levels (41.6 per cent). A correct diagnosis is important for effective clinical follow-up and for genetic counseling.

A clinical, epidemological, laboratorial, histological and ultrasonographical evaluation of anti-HCV EIA-2 positive blood donors

Between 1992 and 1997, 790 blood donors with anti-HCV EIA-2 strongly reagent (relationship between the sample optical density/cut-off > 3) detected at the blood bank serological screening, were evaluated in ambulatory environment. They were all negative for Chagas disease, syphilis, hepatitis B (HBsAg) and AIDS. Blood samples were collected at the first ambulatorial evaluation, for hemogram, biochemical tests and new serological tests for HCV (anti-HCV EIA-2). In blood samples of 226 repeatedly reagent anti-HCV EIA-2 blood donors, supplementary "immunoblot" test for HCV (RIBA-2) was used. In 209 donors, the presence of HCV-RNA was investigated by the PCR test. The abdominal ultrasonography was realized in 366 donors. In 269 patients liver biopsy was performed for the histopathological study. The follow-up of blood donors showed that 95.6 percent were repeatedly EIA-2 reagent, 94 percent were symptomless and denied any hepatitis history, with only 2 percent mentioning previous jaundice. In 47 percent of this population at least one risk factor has been detected for the HCV transmission, the use of intravenous drugs being the main one (27.8 percent). Blood transfusion was the second factor for HCV transmission (27.2 percent). Hepatomegaly was detected in 54 percent of the cases. Splenomegaly and signs of portal hypertension have seldom been found in the physical examination, indicating a low degree of hepatic compromising in HCV. Abdominal ultrasound showed alterations in 65 percent of the subjects, being the steatosis the most frequent (50 percent). In 83.5 percent of the donors submitted to the liver biopsy, the histopathological exam showed the presence of chronic hepatitis, usually classified as active (89 percent) with mild or moderate grade in most of the cases (99.5 percent). The histopathological exam of the liver was normal in 1.5 percent of blood donors. The RIBA-2 test and the HCV-RNA investigation by PCR were positive in respectively 91.6 and 75 percent of the anti-HCV EIA-2 reagent donors. The HCV-RNA research was positive in 82 percent of the RIBA-2 positive subjects, in 37.5 percent of the indeterminate RIBA-2 donors and in 9 percent of the negative RIBA-2 donors. Chronic hepatitis has also been observed in 50 percent of the histopathological exams of the anti-HCV EIA-2 reagent donors which were indeterminate RIBA-2...

Esclerose hepatoportal associada à trombose de veia porta em crianças: relato de três casos / Hepatoportal sclerosis associated with portal vein thrombosis in children: report of 3 cases

Esclerose hepatoportal é o termo utilizado para designar a condiçäo clínico-patológica responsável por hipertensäo portal näo-cirrótica. Säo apresentados três casos de crianças com esclerose hepatoportal associada à trombose de veia porta. Os dois primeiros pacientes apresentaram, como queixa principal, hemorragia digestiva alta e o terceiro veio encaminhado para investigar hepatoesplenomegalia. O exame ultra-sonográfico evidenciou alteraçöes indicativas de trombose de veia porta extra-hepática nos três casos. A biopsia hepática foi realizada nestes pacientes por apresentarem enzimas hepáticas alteradas (aminotransferase e Gama GT). Os achados histopatológicos principais foram: esclerose subintimal, fibrose portal e proliferaçäo telangiectásica de ramos portais intra-hepáticos, compatíveis com o diagnóstico de esclerose hepatoportal. Os três pacientes apresentaram boa evoluçäo, sendo controlada a hemorragia nos dois primeiros casos por escleroterapia das varizes esofágicas.

Injetável trimestral de açäo prolongada como anticoncepcional: efeitos sobre o ciclo menstrual e endométrio / Medroxyprogesterone acetate as long action contraceptive: effects on menstrual cycle and endometrium

O acetato medroxiprogesterona injetável AMP é um progestogênio sintético de açao prolongada que produz efeitos marcantes sobre o ciclo menstrual e endométrio. Neste estudo foram avaliadas as características do ciclo menstrual de 1.136 mulheres que utilizaram o AMP como anticoncepcional por um período de até cinco anos. Em 148 usuárias, estudaram-se também as características histológicas do endométrio. Os resultados mostraram que apenas 25 por cento das usuárias mantiveram menstruaçoes periódicas com o uso do método. A amenorréia foi a alteraçao menstrual mais freqüente, em aproximadamente 70 por cento das usuárias. O diagnóstico histológico revelou um endométrio com sinais de atrofia em 90 por cento das biópsias. Nao foram observadas atipias endometriais no material analisado. Os autores salientaram algumas vantagens do AMP como anticoncepcional e enfatizaram a importância da orientaçao prévia aos efeitos colaterais para um bom desempenho e aceitaçao do método.

Valor da ultra-sonografia abdominal e da biópsia hepática percutânea no diagnóstico diferencial da colestase neonatal / The role of the abdominal ultrasonography and the percutaneous liver biopsy in differential diagnosis of neonatal cholestasis

A colestase neonatal representa para o médico um desafio do ponto de vista de diagnóstico anatômico e etiológico. É fundamental diferenciar colestase intra-hepática (CIH), pois, nesta última, o tratamento é cirúrgico e o prognóstico depende da idade em que a criança é submetida à cirurgia. Diversos exames subsidiários têm sido propostos na elucidaçäo. Entre estes, a biopsia hepática é o mais largamente utilizado e a ultra-sonografia um dos mais recentes. O objetivo do presente trabalho é relatar a experiência de quatro anos e meio com a utilizaçäo da biopsia hepática e ultra-sonografia, na diferenciaçäo entre CIH e CEH. Foram estudados 35 pacientes com colestase neonatal, no período de janeiro de 1989 a julho de 1993, sendo submetidos a um protocolo de investigaçäo diagnóstica, incluindo biopsia hepática percutânea e ultra-sonografia abdominal. Este último procedimento foi realizado após jejum de quatro horas e considerado indicativo de CEH, quando a vesícula biliar näo era visualizada, era hipoplásica ou näo funcionante, ou se havia evidência de estrutura cística na árvore biliar extra-hepática. Para análise dos resultados calculou-se a sensibilidade, especificidade, valor preditivo positivo, valor preditivo negativo e acurácia da biópsia e ultra-sonografia no diagnóstico de CEH e CIH. O diagnóstico final foi de 17 pacientes com CEH, todos portadores de atresia das vias biliares extra-hepáticas, e 18 pacientes com CIH. A sensibilidade da ultra-sonografia foi de 100 por cento e da biopsia de 76 por cento, no diagnóstico de CEH. A acurácia da ultra-sonografia foi de 83 por cento e da biopsia de 86 por cento, com elevaçäo para 96 por cento quando os exames foram associados. Diante desses resultados, recomendar-se a utilizaçäo da ultra-sonografia abdominal com critérios definidos, associada à biopsia hepática, na investigaçäo diagnóstica de colestase neonatal

Alteraçöes morfológicas endometriais associadas ao uso do dispositivo intra-uterino (DIU) inerte e liberador de cobre / Endometrials morphological alterations associated with used of the intrauterine contraceptive device (IUD)

Curetagens e biópsias endometriais de 40 usuárias de DIUs (28 TCu e 12 alça de Lippes) foram examinadas, juntamente com grupo controle de 44 casos, pareado pela dataçäo morfológica. A idade variou de 21 a 52 anos, e o tempo de inserçäo do DIU, de 1,5 a 240 meses. Foram diagnosticados 11 casos com quadros patológicos, sendo 3 hiperplasias endometriais e 6 decorrentes de corpo lúteo persistente. Entre as 27 pacientes nas quais se pode realizar a dataçäo da mucosa, 55,6 por cento exibiram assincronismo endometrial, representado tanto por avanço como retardo de maturaçäo. Foram pesquisadas 18 variáveis morfológicas, sendo que erosöes e/ou ulceraçöes superficiais, atipias epiteliais superficiais regenerativas, aplainamento da mucosa, infiltrado eosinofílico, metaplasia do tipo "hobnail" e aumento do número de luzes vasculares superficiais, mostraram-se significativamente associadas ao uso do DIU, sem relaçäo com o tempo de uso

Multiple biopsies in bladder urothelial carcinomas: correlation of atypical lesions with histological grade, clinical staging and number of tumors

Twenty-eight cases of transitional cell carcinomas, (19 papillary cell carcinomas, 9 nonpapillary invasive carcinomas) with concomitant mucosal biopses are reported. Multiple biopses were obtained cystoscopically at diagnosis (during ressection or biopsy of the main tumor) or afterwards, during post-operative evaluation. Fifteen patients (53.6%) wre positive for dysplasia, carcinoma "in situ" or micro-invasive carcinoma in the biopsies. These lesions were correlated with the primary neoplasm in regard to: 1) histological grade. Atypical lesions were more frequent the higher the grade (0.01 < p < 0.05); 2) clinical staging. The possibility of finding atypical lesions was higher in cases with more advanced staging (0.01 < < 0.05) and 3) presence of one or more tumors visible cystoscopically. The results were not statistically significant (0.10 < p < 0.50) but there was a trend toward a highr incidence of atypical lesions among patients with more than one tumor at cystoscopy. Performance of multiple mucosal biopsies is the only means of diagnosing for atypical lesions of the bladder because, due to their plane configuration, they are not detected cystoscopically. The presence of these lesions is very important because they influence the prognosis and the therapeutic measures

Grande cisto luteinizado solitário do ovário; próprio do período gravídico ou puerperal: descriçäo de dois casos / Large solitary luteinized ovarian cyst: a report of 2 cases

Säo descritos dois casos de cisto luteinizado gigante do ovário, próprio do período gravídico ou puerperal. É entidade anatomoclínica pouco freqüênte, de patogênese discutida, provavelmente relacionada a estímulo pelas gonadotrofinas coriônicas (HCG) ou hipofisárias (FSH e LH). A importância do seu conhecimento está principalmente relacionada ao diagnóstico diferencial com as neoplasias ovarianas

Intuscepçao intestinal por lipoma de colon: confusao diagnostica com carcinoma colorretal obstrutivo / Intestinal intussusception by colonic lipoma: confusion diagnostc with obstrutive colon-rectal carcinoma

Os autores apresentam um caso de intuscepçao por lipoma de colon que se manisfestou como obstruçao intestinal baixa, cujo diagnostico clinico foi de carcinoma colorretal disseminado. Devido as pessimas condiçoes gerais, o paciente acabou indo a obito, e o diagnostico so foi feito por necropsia. A literatura a respeito e revista. Discutem-se as formas de se diagnosticar e tratar essa manifestaçao pouco comum do lipoma de colon, e a importancia do diagnostico diferencial com o carcinoma colorretal obstrutivo.