Adenoma suprarrenal gigante con degeneración quística hemorrágica / Giant adrenal adenoma with hemorrhagic cystic degeneration

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[Tumor neuroendocrino ano-rectal: desde un pólipo con buen pronóstico hasta un carcinoma letal]. / Ano-rectal neuroendocrine tumour: from a polyp with a good prognosis to a lethal carcinoma.

INTRODUCCIÓN: Aunque los tumores neuroendocrinos rectales son neoplasias poco frecuentes, su incidencia está aumentando en las tres últimas décadas. El objetivo de este trabajo es el estudio del comportamiento de dichos tumores basándonos en sus variables clínicas, diagnósticas, terapéuticas y anatomopatológicas en un hospital terciario. MÉTODO: Estudio retrospectivo de los tumores neuroendocrinos rectales desde el año 2000 hasta el año 2017 en nuestro hospital. Se realizó una búsqueda informatizada en el programa SNOMED del servicio de anatomía patológica, empleando los criterios «tumor neuroendocrino¼ y «tumor carcinoide¼. RESULTADOS: Se hallaron 16 pacientes con tumor neuroendocrino ano-rectal. El 75% eran varones. La mediana de edad fue de 53 años (rango: 34-83). El diagnóstico fue incidental en el 62,5%. El tratamiento fue endoscópico en 13 (81%) pacientes y quirúrgico en 3 (19%). El 75% de las lesiones fueron T1. El 68,7% de los tumores presentaban un grado de agresividad G1. La media de seguimiento fue de 29 meses; no hubo recidivas. Durante el seguimiento fallecieron 3 (18,5%) pacientes, todos ellos con tumor G3 metastásico, y la supervivencia media fue de 8 meses. CONCLUSIONES: Los tumores neuroendocrinos rectales son cada vez más frecuentes y presentan unos patrones variables, cuyo pronóstico dependerá de su agresividad histológica. El aumento de su incidencia puede deberse a una mayor concienciación de la existencia de estos tumores, a las campañas de cribado del cáncer colorrectal, al aumento del número de colonoscopias realizadas y a la generalización de los estudios histológicos con marcadores neuroendocrinos. BACKGROUND: Although rectal neuroendocrine tumours are rare neoplasms, their incidence is increasing in the last three decades. The objective of this work is to study the behaviour of these tumours based on their clinical, diagnostic, therapeutic, and pathological variables in a tertiary hospital. METHOD: Retrospective study of rectal neuroendocrine tumours from 2000 to 2017 in our hospital. A computerized search was performed in the SNOMED program of the pathological anatomy service, using the criteria "neuroendocrine tumour" and "carcinoid tumour". RESULTS: We found 16 patients with ano-rectal neuroendocrine tumour; 75% were male. The median age was 53 years (range: 34-83). The diagnosis was incidental in 62.5%, with endoscopic treatment in 13 patients (81%) and surgical treatment in 3 (19%). 75% of the lesions were T1. 68.7% of the tumours showed a degree of G1 aggression. The mean follow-up was 29 months; no recurrences. During follow-up, three patients died (18.5%), all of them with metastatic G3 tumour and the mean survival was 8 months. CONCLUSIONS: The rectal neuroendocrine tumours are increasingly frequent and present variable patterns, whose prognosis will depend on their histological aggressiveness. The increase incidence may be due to a greater awareness of the existence of these tumours, to colorectal cancer screening, to the increase in the number of colonoscopies performed and to the generalization of histological studies with neuroendocrine markers.

Mitral valve prosthesis implanted in atrial wall over huge calcified annulus.

We describe an alternative technique for mitral valve replacement in patients with severe mitral annular calcification, in whom conventional techniques are not feasible. A new annulus that allows supra-annular prosthetic implantation is created.

Giant left main coronary artery to right atrium fistula.

Aneurysmal arterial origin of coronary fistulae is an extremely rare combination. We report a case of a giant left main coronary artery to right atrium fistula in a 48-year-old male. We describe the clinical course and management options.

Randomized comparison of long-term carvedilol and propranolol administration in the treatment of portal hypertension in cirrhosis.

Short-term carvedilol administration is more powerful than propranolol in decreasing hepatic venous pressure gradient (HVPG) in cirrhotic patients, but induces arterial hypotension that may prevent its long-term use in portal hypertensive patients. This study compared the HVPG reduction and safety of long-term carvedilol and propranolol. Fifty-one cirrhotic patients were randomly assigned to receive carvedilol (n = 26) and propranolol (n = 25). Hemodynamic measurements and renal function were assessed at baseline and after 11.1 +/- 4.1 weeks. Carvedilol caused a greater decrease in HVPG than popranolol (-19 +/- 2% vs. -12 +/- 2%; P <.001). The proportion of patients achieving an HVPG reduction >/=20% or

Response to steroids in de novo autoimmune hepatitis after liver transplantation.

Graft dysfunction associated with autoimmune phenomena has been recently described in liver transplant recipients without previous autoimmune disease. However, the natural history, diagnostic criteria, and definitive therapeutic approach of de novo autoimmune hepatitis (de novo AIH) are poorly understood. We report 12 cases of de novo AIH 27.9 +/- 24.5 months after liver transplantation: the outcome of 7 patients treated with steroids is compared with a group of 5 nontreated patients. Nontreated patients lost the graft after 5.8 +/- 2.6 months from de novo AIH onset. All treated patients were alive after 48.4 +/- 14 (29-65) months from de novo AIH onset, and none of them lost the graft. However, 5 patients relapsed in relation to steroid tapering. All patients presented an atypical antiliver/kidney cytosolic autoantibody, associated to classical autoantibodies in 10 cases. Histological study showed several degrees of lobular necrosis and inflammatory infiltrate. HLA antigen frequencies and matching were compared with 2 control groups (16 orthotopic liver transplantation [LTX] patients without de novo AIH and 929 healthy blood donors); de novo AIH patients showed a higher prevalence of HLA-DR3 (54.5% vs. 25.9%, P =.04) than healthy controls, which was not observed in LTX patients without de novo AIH. In conclusion, this new disease should be included in the differential diagnosis of unexplained graft dysfunction. In addition, treatment with steroids results in a dramatically improved outcome. However, maintenance therapy is usually required.