Neoplasias hepáticas benignas: variantes histológicas, problemas diagnósticos y diagnóstico diferencial / Diagnosis and Differential Diagnosis Problems with Histological Variants of Benign Liver Neoplasms

Un principio básico de la patología es que las neoplasias se diferencian según sus células de origen y en el hígado semejan sus constituyentes, sean las células hepáticas, del epitelio biliar, endoteliales, mesenquimales o una combinación de estas. Es importante recordar aquí que son las metástasis el tumor maligno más frecuente del hígado, con una relación de 30:1 en pacientes sin enfermedad hepática crónica o cirrosis subyacente; es rara la presencia de las mismas en hígados cirróticos. Las neoplasias gastrointestinales del colon, páncreas, vía biliar extrahepática, estómago, tumores neuroendocrinos y GIST, o extraintestinales del pulmón, mama, melanoma o tumores de cabeza y cuello, son las más frecuentes (1). En este artículo solo revisaremos las más frecuentes. Iniciaremos con las neoplasias benignas y las lesiones pseudotumorales haciendo especial énfasis en aquellas con dificultades diagnósticas, en la utilidad de estudios especiales de inmunohistoquímica o moleculares para su adecuada clasificación y diagnóstico diferencial.

A basic principle of pathology is that neoplasms differ according to their cells of origin. Neoplasms of the liver resemble its constituent liver, biliary, epithelial, endothelial, mesenchymal cells or some combination of these different types of cells. It is important to remember here that metastases are the most frequent malignant liver tumor occurring at ratio of 30: 1 in patients without underlying chronic liver disease or cirrhosis. Metastases are rare in cirrhotic livers. The most common primary sites are the colon, pancreas, common bile duct, stomach, neuroendocrine tumors and GIST, or extraintestinal tumors from the lung, breast, head, neck and skin (1). In this article we review only the most frequent benign neoplasms and tumor-like lesions with particular emphasis on diagnostic difficulties, special studies, and the usefulness of immunohistochemical or molecular studies for proper classification and differential diagnosis.

A Case of Nodular Regenerative Hyperplasia of the Liver Combined with Toxic Hepatitis / 대한소화기학회지

Nodular regenerative hyperplasia (NRH) is an uncommon liver condition characterized by diffuse transformation of the hepatic parenchyma into regenerative nodules without fibrosis. Portal vasculopathy caused by abnormal hepatic venous flow may induce hepatocyte hyperplasia, which forms regenerative nodules. Underlying diseases or certain drugs may also be the cause of NRH. This condition is often underdiagnosed as the patients remain asymptomatic until development of portal hypertension, and histopathologic confirmation by liver biopsy is the only way of making a definite diagnosis. The management mainly involves prevention and treatment of the complications of portal hypertension. The frequency of diagnosis of NRH has increased rapidly in recent years, however, only a few cases have been reported in Korea. Here, we report on a case of NRH of the liver combined with toxic hepatitis.

A Case of Pulmonary Arterial Hypertension Associated with Congenital Extrahepatic Portocaval Shunt

Congenital extrahepatic portocaval shunt (CEPS) is a rare anomaly of the mesenteric vasculature in which the intestinal and splenic venous drainage bypasses the liver and drains directly into the inferior vena cava, the left hepatic vein or the left renal vein. This uncommon disease is frequently associated with other malformations and mainly affects females. Here we report a case of pulmonary arterial hypertension associated with CEPS (Abernethy type 1b shunt) in a 20-yr-old man who was incidentally diagnosed during evaluation of multiple nodules of the liver. The patient was treated by inhalation of iloprost (40 microg/day) with improved condition and walking test. Physicians should note that congenital portocaval shunt may cause pulmonary hypertension.

Hiperplasia nodular regenerativa del hígado secundaria a uso de azatioprina por trasplante renal previo, tratado con trasplante combinado hepático y renal / Combined hepatic and renal transplant after nodular regenerative hyperplasia of the liver secondary to azathioprine in renal transplant patient

Nodular regenerative hyperplasia is an uncommon condition, characterized by the presence of regenerative nodules with minimal or absence of fibrosis, which can lead to non-cirrhotic portal hypertension. There are numerous diseases, conditions and drugs that can cause it. Thiopurines, a group of immunosuppressors used in transplanted patients, has been linked to this entity. We report a case of a renal transplant woman, who has been on chronic therapy with azathioprine and that develops portal hypertension and end-stage renal disease, undergoing combined hepatic and renal transplant. Histological examination of the explanted liver was compatible with nodular regenerative hyperplasia. How azathioprine causes this entity is unknown, but endothelial vascular damage in a dose-dependent manner is postulated as the main mechanism. To our knowledge, this is the first case report of a renal transplant patient who develops nodular regenerative hyperplasia of the liver in association with azathioprine, and undergoes combined hepatic and renal transplant, with a favorable outcome 5 years post procedure.

La hiperplasia nodular regenerativa es una entidad infrecuente, que se caracteriza por la presencia de nódulos hepáticos con ausencia o mínima fibrosis y que puede llevar a hipertensión portal de origen no cirrótico. Existen diversas enfermedades, condiciones y medicamentos que la causan, destacando entre estos últimos las tiopurinas, inmunosupresores utilizados habitualmente en trasplantados. Se presenta el caso de una paciente trasplantada renal usuaria crónica de azatioprina, que desarrolla hipertensión portal además de deterioro de la función renal, requiriendo de un doble trasplante hepático y renal, destacando en la biopsia del explante hallazgos histológicos compatibles con hiperplasia nodular regenerativa. Los mecanismos de daño por azatioprina en esta entidad son desconocidos, pero se postula al daño endotelial dosis-dependiente como principal causa. La revisión de la literatura demuestra que este caso corresponde al primero de hiperplasia nodular regenerativa secundaria a azatioprina en trasplantado renal, que requiere de doble trasplante hepático y renal con evolución favorable hasta 5 años post trasplante.

Pseudocirrhosis as a complication after chemotherapy for hepatic metastasis from breast cancer

No abstract available.

Síndrome hepatopulmonar en paciente con hipertensión portal no cirrótica. Reporte de caso / Case report of hepatopulmonary syndrome in patients with non-cirrhotic portal hypertension

Presentamos el caso de una paciente con diagnóstico de síndrome hepatopulmonar (SHP) secundario a hipertensión portal no cirrótica subyacente, en el contexto de trombosis crónica de la vena porta y la mesentérica, por trombofilia primaria (deficiencia de proteína C-S). Se hizo el diagnóstico de SHP al encontrar una ecocardiografía contrastada con solución salina agitada positiva para un shunt extracardíaco, una gammagrafía con macroagregados de albúmina con evidencia de captación a nivel cerebral e hipoxemia persistente con gradiente alvéolo-arterial elevado para la edad de la paciente.

We report the case of a female patient diagnosed with hepatopulmonary syndrome (HPS) which occurred in the context of chronic thrombosis of the portal and mesenteric veins resulting from primary thrombophilia (protein C and protein S deficiencies). This case of HPS was diagnosed when echocardiography with agitated saline contrast showed the patient was positive for extra cardiac shunt. Scintigraphy using albumin macro-aggregates showed evidence of uptake in the brain and persistent hypoxemia with elevated alveolar-arterial gradient for the age of the patient.

Liver pathology in collagen vascular disorders highlighting the vascular changes within portal tracts.

Background: Collagen vascular disorders (CVDs) are autoimmune disorders with multisystem involvement. Clinical liver involvement is not a characteristic feature though histological involvement could be frequent. Liver disease in CVDs could be the consequence of various factors. Aim: The aim was to analyze the histological spectrum of liver in collagen vascular disorders (CVDs) at autopsy. Materials and Methods: Thirty-six autopsy livers negative for hepatitis B or C virus were studied in CVD cases with no known association with chronic liver disease or vascular thrombosis or hematological disorder. Cirrhotic and normal livers were used as controls. The paired t-test, one-way ANOVA, and two-sided Dunnett t-test were used for comparison (< 0.05). None of the control cases showed any abnormal vessels. Results: There were 21 systemic lupus erythematosus (SLE), 7 rheumatoid arthritis (RA), 5 systemic sclerosis (SSc), and 3 polyarteritis nodosa (PAN) cases (M:F = 11:25, age range 23-60 years). Histology: Diffuse nodular regenerative hyperplasia of liver (NRHL) was seen in 10 cases, and 6 (5 SLE and 1 RA) had numerous abnormal thin-walled vessels in intermediate- and small-sized portal tracts with no vascular occlusion or inflammation. Moderate sized portal tracts showed more interface and lobular inflammation. The main portal vein and its major branches were normal. None of these six cases had increased transmainases (P>0.05). Most SLE cases had increased transaminases (P<0.05). No evidence of portal hypertension was seen in all except in one RA. Septicemia is known to be associated with raised transaminases. Conclusion: A rare pathology of conglomerate of abnormal vessels in intermediate- and small-sized portal system was observed co-existing with NRHL in CVDs. Raised liver enzyme with interface hepatitis in CVD may not necessarily warrant an overlap, as a similar feature could be observed in septicemia.

A Case of Nodular Regenerative Hyperplasia of Liver that mimicked Primary Biliary Cirrhosis / 대한간학회지

Nodular regenerative hyperplasia (NRH) of the liver is a rare disease that is characterized by multiple regenerative nodules in the hepatic parenchyma without fibrosis. The exact pathogenesis of NRH has not been established, but it's been suggested that obliteration of portal veins may initiate the nodular transformation. It is also known that this disease is associated with autoimmune disease, myeloproliferative disease, lymphoproliferative disease, primary biliary cirrhosis, and some chemotherapy agents. The patients with NRH are usually asymptomatic, yet if they have symptoms, the most common clinical manifestations are those of portal hypertension, including splenomegaly and esophageal varices with or without bleeding. We report a case of nodular regenerative hyperplasia that presented with clinical manifestations similar to those of primary biliary cirrhosis.

Nodular regenerative hyperplasia of the liver:a clinical study of 12 cases / 中华消化杂志

Objective To review the clinical,histological and diagnostic aspects of 12 documented cases of nodular regenerative hyperplasia of the liver(NRHL),to make this condition be understood and dealt with better. Method Twelve NRHL cases were diagnosed based on liver biopsy from 300 portal hypertension patients who had been underwent splenectomy.Imaging studies were performed as part of the diagnostic evaluation.Clinical manifestation and biochemical tests were recorded at the time of diagnosis.Management and prognosis were also reviewed.Results Most patients were complicated with autoimmune disease,6 cases were diagnosed systemic lupus erythematosus and 1 was Crohn's disease and 1 suspected ulcerative colitis.Six cases were treated by prednisone and 3 cases by immunosuppressant.Eleven cases had suffered from portal hypertension.All cases had no history of viral hepatitis.Biochemical tests showed mild increase of liver enzyme and relative normal synthetic liver function.The histological finding was nodular in the hepatic parenehyma,with mild periportal fibrosis,intraportal lymphocytic infiltration,narrow and obstruction of branch of portal vein,and lack of hepatocyte necrosis.All cases were diagnosed liver cirrhosis and portal hypertension before operation.Management was directed to portal hypertension and varices bleeding with satisfactory results.Most of them keep a stable condition during the follow-up. Conclusion The NRHL was uncommon and its cause and pathogenesis was unclear,may be related with immune and hepatic blood circulation disorder.It should be considered in patients with unexplained portal hypertension and distinguished it from liver cirrhosis.Liver biopsy confirms the diagnosis.Management directed to portal hypertension may improve clinical condition.