Type I acquired cutis laxa: Report of a unique progressive case and short review.

Cutis laxa is a rare connective tissue disorder, characterized by a reduced number and abnormal properties of elastic fibers throughout the dermis, creating a clinical appearance of premature aging. It can be subdivided into inherited and acquired, the latter rarer than the former, and skin involvement may be localized or generalized. The etiology of acquired cutis laxa (ACL) remains unknown and there is no definitive treatment. We present the case of a 30-year-old man diagnosed with type I ACL with progressive systemic involvement at the renal, pulmonary, and digestive levels. Histological analysis of the skin revealed reduction and fragmentation of elastic fibers. Immunosuppressive treatment was started with prednisone, cyclophosphamide, and rituximab, with which a complete response to proteinuria was achieved and the progression of lung damage was limited. Autoimmune, infectious, and neoplastic diseases were ruled out.

A survivor woman after three years of a cardiac tamponade.

Primary pericardial mesothelioma is an extremely rare tumor, of unclear etiology, nonspecific presentation, with a delay in diagnosis, and a poor prognosis. We present the case of a woman with pericardial mesothelioma, whose main manifestation was cardiac tamponade, currently alive three years after diagnosis and undergoing chemotherapy treatment. .

[Renal cell carcinoma: pathological prognostic factors, staging and histopathological classification of 355 cases]. / Carcinoma de células renales: factores patológicos pronósticos, estadificación y clasificación histopatológica.

BACKGROUND: New morphologic entities for Renal Cell Carcinoma (RCC) that influence the prognosis have been described. Clinical staging has also undergone several modifications, the last one published in 2010 7th edition of the American Joint Committee on Cancer. The aim of this article is to determine the prevalence of histological subtypes, Fuhrman grading and clinical staging of renal cell carcinomas. METHODS: This is a retrospective, descriptive and comparative study, from January 2008 to June 2013. 355 cases of RCC were reclassified according to nuclear grading and new histopathological diagnostics and staging according to the TNM. A Kappa index was used for the diagnostic concordance and nuclear grading. RESULTS: Conventional renal cell carcinoma corresponded to 84.51 %, followed by chromophobe carcinoma and papillary. Less common subtypes were: multilocular cystic carcinoma, papillary clear cell carcinoma and others. Nuclear grading was directly related to the tumoral size and clinical staging (p < 0.001). The predominant stage was pT1b N0 M0, followed by pT3a N0 M0. CONCLUSIONS: The most frequent tumor was clear cell RCC, followed by chromophobe carcinoma and papillary carcinoma. Nuclear grading, necrosis, eosinophilic cells areas with areas of sarcomatoid and rhabdoid differentiation are prognostic factors associated with an increased aggression and risk of metastases.

Introducción: recientemente se han descrito nuevas entidades morfológicas de carcinomas de células renales (CCR) que influyen en el pronóstico. La estadificación clínica también ha sufrido modificaciones. El objetivo de este trabajo es conocer la prevalencia de los subtipos histológicos, el grado nuclear de Fuhrman y el estadio clínico del CCR. Métodos: estudio retrospectivo, descriptivo y comparativo de enero de 2008 a junio de 2013. Se analizaron 355 casos de CCR, fueron reclasificados de acuerdo con el grado nuclear y nuevos diagnósticos histopatológicos (clasificación de Vancouver) y estadificados de acuerdo con el TNM. Se realizó índice de Kappa para la concordancia diagnóstica y gradación nuclear, la asociación de variables cualitativas fue comparada con chi cuadrada. Resultados: el CCR claras convencional fue del 84.5 %, seguido del cromófobo y papilar. Otros subtipos menos frecuentes fueron: el carcinoma quístico multilocular, el CCR papilar de células claras y otros. El grado nuclear estuvo directamente relacionado con el tamaño tumoral y con el estadio clínico (p < 0.001). El estadio que predomino fue el pT1b N0 M0, seguido del pT3a N0 M0. Conclusiones: el tumor más frecuente fue el CCR claras, seguido del cromófobo y papilar. El grado nuclear, necrosis, áreas eosinófilas, sarcomatoides y rabdoides son factores pronósticos asociados a mayor agresividad y riesgo de metástasis.

Alteraciones de la vena porta en pacientes con atresia de vías biliares extrahepáticas / Portal vein alterations in patients with extrahepatic biliary atresia

Introducción. Las lesiones vasculares del hígado son diversas y pueden observarse en varias condiciones. En la atresia de vías biliares extrahepáticas (AVBE) se han descrito lesiones arteriales; sin embargo, hasta donde sabemos, alteraciones en las venas portales no han sido descritas con anterioridad. El propósito de este trabajo es el de informar de algunas lesiones peculiares de la vena porta observadas en un grupo de pacientes con AVBE. Métodos. Estudio retrospectivo, descriptivo de 62 biopsias consecutivas de pacientes con AVBE a los que se les practicó cirugía derivativa. Se estudiaron los vasos del hilio hepático con tinciones de hematoxilina eosina, Masson, fibras elásticas, mucicarmín, azul alciano, PAS, hierro coloidal, CD20, CD3, CD68, y actina. Se evaluó: proliferación fibrosa o celular subendotelial, daño a la elástica, edema, calcificación, trombosis y depósito de glicosaminoglicanes. La extensión del daño se graduó como leve (menos de 25% de la circunferencia), moderado (26 a 50%), o extenso (más de 50%). De acuerdo a su diámetro, los vasos fueron clasificados como de tamaño medio o grande; se evaluaron la vena portal o sus ramas mayores. Los explantes hepáticos (n =20) de pacientes sin AVBE se utilizaron como testigos, y los datos clínicos y demográficos se obtuvieron de los expedientes. Resultados. La edad promedio fue de 3 meses; 45 fueron niñas. En 24 casos se obtuvo vena porta o sus ramas principales. Se encontraron alteraciones vasculares en 35 casos: arteriales en 13, venosas en 10, y daño en ambas en 12. Los cambios arteriales principales fueron: ruptura de la capa elástica (n =19) y proliferación celular subendotelial (n =20); todos los cambios fueron de leves a moderados. Los cambios venosos principales fueron: depósito de glicosaminoglicanes (n =7), proliferación fibrosa subendotelial (n =8) y ruptura de las fibras elásticas (n =9); todos fueron cambios leves. La proliferación subendotelial estuvo compuesta por macrófagos, linfocitos CD20 y CD3 y miofibroblastos. Las alteraciones se observaron desde edades tempranas y no estuvieron presentes en los testigos. Conclusión. Las alteraciones vasculares fueron comúnmente observadas en la AVBE, los cambios en la vena porta que se observaron en un tercio de nuestros casos, parecen ser parte del proceso y no una condición adquirida; podrían explicar algunas complicaciones quirúrgicas del trasplante hepático y apoyar las teorías vascular y autoinmune sobre el origen de la AVBE.

Introduction. Vascular lesions of the liver are varied and may be observed in different conditions; arterial lesions have been observed in extrahepatic biliary atresia (EBA). To our knowledge, alterations of the portal vein or its main branches have not been described previously. The aim of this article is to inform peculiar portal vein lesions found in a group of patients with EBA. Methods. This is a descriptive, retrospective study of 62 consecutive biopsies of patients with EBA who underwent portoenteral anastomosis over a 10 year period. Vessels from the hepatic hilium were studied with hematoxylin & eosin, Masson's trichrome, elastic fibers, mucicarmine, alcian blue, PAS, coloidal iron, CD20, CD3, CD68, and actin stains. Cellular or fibrous subendothelial proliferation, tunica elastica damage, edema, calcification, thrombosis, and glycosaminoglycan deposits were searched; the extension of damage was graded as mild (less than 25% of circumference), moderate (26 to 50%), or severe (over 50%). Vessels were classified according to their circumference as medium sized (less than 300 microns) and large; when possible the portal vein and/or its larger branches were evaluated. Hepatic explants (n =20) of patients without EBA were used as controls. Demographic data were obtained from clinical charts. Results. Age averaged 3 months; 45 patients were female. In 24 cases, portal vein or its larger branches were present. Vascular alterations were observed in 35 cases: arterial changes in 13, vein lesions in 10, and both types of damage in 12. The main arterial changes found were: elastic rupture (n =19), glycosaminoglycans deposits (n =19), and subendothelial cellular proliferation (n =20); all changes were mild to moderate. On the other hand, glycosaminoglycans deposits (n =7), sub-endothelial fibrous proliferation (n =8), and elastic fibers rupture (n =9) were the main alterations observed in the portal vein or its branches; these changes were mild. Subendothelial cellular proliferation was composed of macrophages, B and T lymphocytes, and myofibroblasts. These alterations were present since early ages and not observed in control subjects. Conclusions. Vascular alterations were commonly observed in EBA. Portal vein changes were present at least in one third of cases studied, and they appear to be part of the process and not an acquired condition. These changes may explain some vascular complications of liver transplantation of patients with EBA as well as support vascular and/or autoimmune theories on EBA origin; likewise, they may provide data for future insights on EBA pathogenesis.