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.

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.

IMPORTANCE OF LIVER BIOPSY IN THE DIAGNOSIS OF LYSOSOMAL ACID LIPASE DEFICIENCY: A CASE REPORT. / IMPORTÂNCIA DA BIÓPSIA HEPÁTICA NO DIAGNÓSTICO DA DEFICIÊNCIA DE LIPASE ÁCIDA LISOSSOMAL: RELATO DE CASO.

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.

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.

Bartonella henselae AS A PUTATIVE CAUSE OF CONGENITAL CHOLESTASIS.

Severe anemia and cholestatic hepatitis are associated with bartonella infections. A putative vertical Bartonella henselae infection was defined on the basis of ultrastructural and molecular analyses in a three-year-old child with anemia, jaundice and hepatosplenomegaly since birth. Physicians should consider bartonellosis in patients with anemia and hepatitis of unknown origin.

Hepatic involvement in paediatric patients with paracoccidioidomycosis: a histological study.

AIM: Paracoccidioidomycosis is a systemic mycosis that is endemic to certain countries in Latin America. This study aimed to describe the histological features of liver involvement in patients with paracoccidioidomycosis aged <16 years of age who were treated between 1980 and 2010, with a diagnosis that was confirmed by detection of the fungus by pathological examination. METHODS AND RESULTS: Liver tissue was obtained from one necropsy and 12 biopsies. Throughout 2007, biopsies were taken from patients with persistent jaundice or portal hypertension, after which biopsies became indicated due to elevated aminotransferase and low albumin levels. Using haematoxylin and eosin (H&E), Masson's trichrome and immunohistochemical (CK7 and CK19) staining, we noted degenerative alterations in bile duct cells and inflammatory injury to the bile ducts in 10 biopsies. Using immunohistochemistry for CK7 and CK19, we observed ductal proliferation in all 12 samples. CONCLUSIONS: Bile duct injuries by inflammatory cells might explain the predominant increase in canalicular enzymes; immunohistochemistry is more sensitive in demonstrating ductular reactions and might show changes that are not apparent on H&E staining.

Steatosis of indeterminate cause in a pediatric group: is it a primary mitochondrial hepatopathy?

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 ß-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.

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.

Acute hepatotoxicity caused by enalapril: a case report.

A case of enalapril-induced acute hepatotoxicity with an unusual morphology is described. This morphology was characterized by macro- and microvesicular steatosis associated with neutrophil infiltration and Mallory bodies, occasionally with satellitosis. These alterations were most abundant in zone 1 of the periportal region, less common in zone 2 and rare in zone 3. There was also confluent periportal necrosis with sinusoidal fibrin deposits associated with intense ductal metaplasia and an infiltrate of inflammatory cells that included plasmocytes and a few eosinophils, as well as focal biliary damage. This morphology, that may be referred as "predominantly periportal steatohepatitis", was distinct from that associated with non-alcohol and alcohol-induced steatohepatitis, both initiated in acinar zone 3 and subsequently extended to other zones.

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...

Mitochondrial alterations in nonalcoholic fatty liver disease. Pediatric case description of three submitted sequential biopsies.

Nonalcoholic fatty liver disease (NAFLD) is a clinical-pathological syndrome that encompasses a wide spectrum of morphologic alterations, ranging from simple hepatic steatosis to a more severe stage, known as nonalcoholic steatohepatitis (NASH). The purpose of this clinical report was to contribute to the understanding of mitochondrial alterations in NAFLD. The child (13-month-old) underwent initial biopsy in the year 2000 and was diagnosed with diffuse macro and microvesicular steatosis. Two additional biopsies were performed in 2001 and 2004. A high percentage of microvesicular steatosis was observed in the biopsies performed in 2000 and 2001. Mitochondrial size was slightly increased in the biopsy performed in the year 2000, significantly increased in 2001 and decreased in 2004. The presence of "mitochondrial hypertrophy" in the hepatocytes of an asymptomatic pediatric patient whose disease presentation was typical of NAFLD, excluding other pathological processes, allowed us to suspect that such a defect was considered the primary mitochondrial disorder.

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.

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.

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.

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