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Nodular regenerative hyperplasia of the liver -a type of non-cirrhotic portal hypertension- is a rare condition of unknown etiopathogenesis that has been associated with multiple disorders, including diverse types of hematologic disease. We report the case of a 36-year-old female patient diagnosed with cutaneous T-cell lymphoma of the mycosis fungoides variety, staged as T2N0M0B0, where a transjugular liver biopsy demonstrated the presence of nodular regenerative hyperplasia with a hepatic venous pressure gradient of 15 mm Hg. The study was triggered by the incidental radiologic finding of hepatomegaly with indirect evidence of portal hypertension. We are not aware of any previous reports on the association of nodular regenerative hyperplasia with mycosis fungoides in the medical literature.

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, mesenquima- les 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.

Introduction: regenerative nodular hyperplasia (RNH) is a rare liver disease with an etiology that is not well understood. Among the etiological factors are purine-analogue drugs such as azathioprine. Case report: we present a case of a 47-year-old patient diagnosed with Crohn's disease in treatment with azathioprine due to corticosteroid dependency who developed RNH with clinical and laboratory signs of portal hypertension one year after starting treatment. After discontinuation of azathioprine, the patient started treatment and, given the poor disease progression, started treatment with adalimumab. This was continued with an excellent response and without deleterious effects on the liver. Discussion: the relevance of this case is twofold: First, this is a rare and early side effect of azathioprine treatment and this is an irreversible disease with potentially serious complications. Second, because treatment was carried out with biological drugs (adalimumab) despite the patient having advance liver disease with portal hypertension without any evidence of its worsening, nor signs of deleterious effects or complications, given that there is scarce or no experience with adalimumab treatment in this type of situation.

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. [ABSTRACT FROM AUTHOR]