Post-COVID-19 Secondary Sclerosing Cholangitis: A Rare but Severe Condition with no Treatment Besides Liver Transplantation.

BACKGROUND The incidence of abnormal liver function, mainly aspartate aminotransferase and alanine aminotransferase elevations, in patients with COVID-19 is not uncommon, but persistent liver damage after the acute phase of the disease is uncommon and has been recently recognized as a new entity named post-COVID-19 cholangiopathy. CASE REPORT We report a clinical case with progressive cholestatic disease following severe COVID-19. AST and ALT peaked at hospital admission and while its serum concentration went down, bilirubin and cholestatic liver enzymes started to increase, reaching the maximum at day 122. Magnetic resonance imaging (MRI) revealed a diffuse irregularity of intra- and extrahepatic bile ducts, with multiple focal strictures alternating with mild focal dilations of the biliary tree, suggesting a sclerosing cholangiopathy. A transjugular liver biopsy showed a prominent bile ductular reaction, cholangiocyte injury, inflammatory infiltrate rich in neutrophils, biliary infarctions, marked cholestasis, and portal fibrosis, suggesting the diagnosis of post-Covid-19 secondary sclerosing cholangitis. The patient evolved with a continuous deterioration of liver functions, but liver transplantation was not performed due to his poor clinical condition. CONCLUSIONS Post-COVID-19 SSC is a severe disease with no effective clinical treatment and has liver transplantation as the only treatment for a few selected patients.

Hepatocellular carcinoma in an adult with Alagille syndrome: case report and literature review.

Alagille syndrome (AS) is an autosomal dominant multisystem disorder which can lead to hepatopathy and the development of focal hepatic lesions. The majority of the hepatic lesions are benign, including regenerative nodules, focal hyperplasia, and adenoma. Hepatocellular carcinoma (HCC) is extremely rare in AS, with very few cases reported in the literature. A 38-year-old man complaining of acute right upper quadrant pain with long-standing diagnosis of Alagille syndrome. On imaging, the patient had a large hepatic mass in the right lobe, with arterial hyperenhancement, washout appearance, and areas of internal hemorrhage. The patient underwent a right hepatectomy and histopathology demonstrated HCC. The patient passed away 3 months after the surgery due to infectious complications. HCC is a rare complication of AS, although rare, it should be considered. This case also emphasizes the need of HCC screening in patients with AS in order to allow an early diagnosis and treatment, which can improve patients' outcome.

Hemangioendotelioma hepático multifocal e metastático: relato de caso e revisão da literatura / Multifocal and metastatic hepatic hemangioendothelioma: case report and literature review

O hemangioendotelioma epitelioide é uma neoplasia rara de origem vascular, cujo acometimento de órgãos como o pulmão, o baço, o osso, a mama e o fígado já foi relatado. O quadro clínico e o prognóstico deste tumor são bastante variáveis. A partir de um relato de caso, procuramos contribuir com os dados existentes na literatura, revisando características clínicas e radiológicas que podem ser úteis para inseri-lo no diagnóstico diferencial das lesões hepáticas.

Epithelioid hemangioendothelioma is a rare neoplasm of vascular origin whose involvement of organs such as lung, spleen, bone, breast and liver has already been reported. The clinical signs and the prognosis of this tumor are quite variable. From the present case report, the authors have sought to contribute with literature data, by reviewing clinical and radiological features which may be useful to include such a condition in the differential diagnosis of liver lesions.

Multifocal and metastatic hepatic hemangioendothelioma: case report and literature review.

Epithelioid hemangioendothelioma is a rare neoplasm of vascular origin whose involvement of organs such as lung, spleen, bone, breast and liver has already been reported. The clinical signs and the prognosis of this tumor are quite variable. From the present case report, the authors have sought to contribute with literature data, by reviewing clinical and radiological features which may be useful to include such a condition in the differential diagnosis of liver lesions.

O hemangioendotelioma epitelioide é uma neoplasia rara de origem vascular, cujo acometimento de órgãos como o pulmão, o baço, o osso, a mama e o fígado já foi relatado. O quadro clínico e o prognóstico deste tumor são bastante variáveis. A partir de um relato de caso, procuramos contribuir com os dados existentes na literatura, revisando características clínicas e radiológicas que podem ser úteis para inseri-lo no diagnóstico diferencial das lesões hepáticas.

The presence of bilobar tumor should be considered as a new limit for transplantation beyond the Milan criteria? / A presença de tumor bilobar deve ser considerado novo limite para exclusão de transplante em pacientes que excedem os critérios de Milão?

BACKGROUND: An imprecise estimate of the tumor's aggressiveness of the hepatocellular carcinoma especially in transplanted patients beyond the Milan criteria has a poor outcome, although a more reliable criteria including microscopic vascular invasion is difficult to be established before transplantation. AIM: To examine a cohort of patients with hepatocellular carcinoma undergoing liver transplantation to evaluate the preoperative predicting factors for microscopic vascular invasion. METHODS: A series of 46 consecutive cirrhotic patients with hepatocellular carcinoma undergoing transplantation based on Milan criteria or similar criteria in a single center were enrolled between 1993 and 2007. The survival was calculated using Kaplan-Meyer's method and a multivariate Cox regression was performed to evaluate survival and factors related to microscopic vascular invasion. RESULTS: Multifocal tumors were present in 39 percent. Microvascular invasion, tumor relapses and hepatocellular carcinoma beyond the Milan criteria were identified in 33 percent, 13 percent and 33 percent, respectively. Overall 1-, 3-, and 5-year actuarial patient survival rates were 64 percent, 59 percent and 45 percent respectively. Patients who exceeded the Milan criteria had a higher incidence of microscopic vascular invasion and bilobar tumor compared to those who met the Milan criteria (53 percent vs. 23 percent and 80 percent vs. 19 percent; p<0.05, respectively). After multivariate analysis, the variable identified as independent risk factor for microscopic vascular invasion was the presence of bilobar tumor (hazard ratio, 3.67; 95 percent confidence interval, 1.01 to 13.34; p<0.05). CONCLUSIONS: The presence of a bilobar tumor is more frequent in hepatocellular carcinoma beyond the Milan criteria and it is an independent predictive factor of a high risk of microscopic vascular invasion. The presence of bilobar tumor in hepatocellular carcinoma beyond the Milan ...

RACIONAL: A recidiva tumoral após o transplante de fígado para o carcinoma hepatocelular tem grande impacto desfavorável na mortalidade e a presença de invasão microvascular desempenha papel importante na recidiva tumoral. OBJETIVO: Avaliar a sobrevida, o risco de recidiva tumoral pós-transplante e os fatores relacionados à invasão microvascular de uma série de transplantados por carcinoma hepatocelular. MÉTODOS: No período entre 1993 e 2007 foi estudada uma série consecutiva de 46 cirróticos com carcinoma hepatocelular submetidos à transplante de fígado baseado nos critérios de Milão a partir de 1996 ou critérios semelhantes no período anterior a esta data. Inicialmente todas as variáveis foram analisadas descritivamente, e as quantitativas através da observação dos valores mínimos e máximos, e do cálculo de médias e desvios-padrão e medianas. Para as variáveis qualitativas calcularam-se frequências absolutas e relativas. Realizou-se a regressão logística com ajuste pelo modelo de Cox para avaliar a sobrevida e os fatores relacionados à recidiva tumoral e invasão microvascular. RESULTADOS: A sobrevida da amostra foi de 64 por cento, 59 por cento e 45 por cento para 1, 3 e 5 anos, respectivamente. Em 13 por cento dos casos, a recidiva tumoral foi verificada. A análise multivariada identificou a chance de um paciente com nódulo bilobar sofrer invasão microvascular é 3,67 vezes maior em relação a um paciente com nódulo unilobar e a presença de um tumor unilobar representar um significativo efeito protetor em relação à invasão microvascular (p = 0,048). CONCLUSÕES: A identificação de um tumor bilobar no estadiamento tumoral é fator preditivo independente de maior risco de invasão microvascular e é necessário ainda confirmar se a presença de tumor bilobar deve ser adicionada aos critérios de Milão para melhor indicação de transplante de fígado em pacientes cirróticos com carcinoma hepatocelular.