Consenso chileno de diagnóstico y manejo de neoplasias quísticas pancreáticas. Club de páncreas Chile-SCHGE

Pancreatic cystic neoplasms (PCN) are frequently detected on abdominal images performed for non-pancreatic indications. Their prevalence in asymptomatic population ranges from 2.7 to 24.8%, and increases with age. There are several types of pancreatic cysts. Some may contain cancer or have malignant potential, such as mucinous cystic neoplasms, including mucinous cystadenoma (MCN) and intraductal papillary mucinous neoplasms (IPMN). In contrast, others are benign, such as serous cystadenoma (SCA). However, even those cysts with malignant potential rarely progress to cancer. Currently, the only treatment for pancreatic cysts is surgery, which is associated with high morbidity and occasional mortality. The Board of the Chilean Pancreas Club of the Chilean Gastroenterology Society developed the first Chilean multidisciplinary consensus for diagnosis, management, and surveillance of PCN. Thirty experts were invited and answered 21 statements with five possible alternatives: 1) fully agree; 2) partially agree; 3) undecided; 4) disagree and 5) strongly disagree. A consensus was adopted when at least 80% of the sum of the answers "fully agree" and "partially agree" was reached. The consensus was approved by the Board of Directors of the Chilean Pancreas Club for publication.

Hemoptisis como manifestación inicial de la enfermedad de Behçet: casos clínicos / Hemoptysis as the initial manifestation of Behçet's disease: report of two cases

Arterial involvement in Behçet's disease (BD) is less common than venous lesions. The most commonly affected arteries are: the aorta, lower extremity arteries, mesenteric, femoral, coronary, renal, subclavian and pulmonary arteries. The rupture of pulmonary arteries is the main cause of death of patients with EB and the presence of aneurysms is a bad prognostic factor. We report two patients with arterial involvement in BD. A 14 years old male presenting with hemoptysis lasting three days. A chest computed tomography showed an aneurismal dilatation of the right interlobar artery, bilateral intramural thrombi and alveolar hemorrhage. A right lobar pulmonary resection was performed but hemoptysis recurred. Suspecting a BD, prednisone was started and hemoptysis subsided. A 42 years old male presenting with chest pain and hemoptysis. A chest CT scan showed thrombi in pulmonary veins and anticoagulant therapy was started. Two months later he was admitted again due to a massive hemoptysis. The CT scan showed aneurisms in pulmonary arteries, mural thrombi and pulmonary infarction. With the diagnosis of BD, methylprednisolone, followed by prednisone and cyclophosphamide were used, with a good clinical response.

Tríada de Carney. Una rara asociación de tumores infrecuentes: caso clínico / Carney triad: report of one case

Carney described a disorder characterized by the presence of several uncommon tumors which were pulmonary chondromas, gastric sarcomas and extra-adrenal paragangliomas. We report a 14 year-old girl in whom multiple gastric tumors were discovered during a study of an iron deficiency anemia and was subjected to a partial gastrectomy. At 25 years of age, she developed several pulmonary chondromas and at 33 years, a mediastinal tumor with features of an extra-adrenal paraganglioma was found. At 35 years of age, a total gastrectomy was performed to remove a gastrointestinal stromal tumor with excision of peritoneal and lymph node metastasis. One year later, the patient died due to liver failure secondary to liver metastases.

Tratamiento con sirolimus y prednisona en enfermedad de Erdheim-Chester con compromiso cardiaco: caso clínico / Sirolimus and prednisone for the treatment of Erdheim-Chester disease with cardiac involvement: report of one case

Erdheim-Chester disease (ECD) is a rare non-Langerhans histiocytic disorder. We report a 76-years-old man who suffered a cardiac tamponade secondary to ECD. A pericardial window was made and during the operation the surgeons observed that the myocardium was diffusely infiltrated. Twenty-eight months before, ECD was clinically diagnosed and prednisone and methotrexate were prescribed. Due to disease progression which culminated in the cardiac tamponade, methotrexate was changed to sirolimus aiming to obtain plasma levels between 5-8 ng/ml. This treatment stabilized his cardiac function allowing a survival of 52 months after its initiation, with fewer side effects.

Calcificaciones pulmonares metastásicas en un trasplantado renal con un injerto con función renal adecuada: Caso clínico / Metastatic pulmonary calcification in a patient with a functioning kidney allograft: Report of one case

In renal transplants patients, metastatic pulmonary calcifications have been reported occasionally when the grafts are dysfunctional and rarely when they are functioning normally. We report a male who received a renal allograft in 1994 at the age of 61 years. Nineteen years later a routine chest X ray showed diffuse infiltrates and a CT scan showed diffuse calcifications in both lungs. These were interpreted as metastatic pulmonary calcifications. The last available laboratory determinations were a serum creatinine of 1.4 mg/dl and urinary protein excretion of 255 mg/24 hours. No further studies were done since the patient experienced a sudden death due to an acute myocardial infarction.

Síndrome hemofagocítico en un trasplantado renal con síndrome de Alport / Hemophagocytic syndrome after kidney transplant in a patient with hereditary nephritis. Report of one case

We report a 28-year-old mole with a hereditary nephritis (Alport Syndrome) on hemodialysis for 5 years, who received a kidney graft from a deceased donor. Cyclosporine (CsA), mycophenolate mofetil (MMF) and steroids were prescribed. In thepostoperativeperiod thepatient had thrombophlebitis and diarrhea. A CTsean showed splenomegaly, ascites, bilateral pleural effusion and bowel edema. Laboratory showed hypoalbuminemia, increased C reactive protein (CRP) and panhypogam-maglobulinemia. At day 32 after transplantation, an acute rejection (Banff II b) was diagnosed and treated with methylprednisolone, replacing CsA by tacrolimus. The acute rejection was controlled but six days later, high fever, pancytopenia and hyperferritinemia appeared. A bone marrow smear showed numerous histiocytes and hemophagocytosis. Hemophagocytic syndrome was diagnosed. MMF and tacrolimus were withdrawn and CsA was reinstituted. Fever fell quickly, CPR normalized at 24 hours and white blood cell count at 72 hours. Days later, the concentrations of albumin, immunoglobulins and hematological parameters normalized. The patient was discharged on day 57 after admission in good condition.

Remisión completa de síndrome nefrótico asociado a carcinoma cervicouterino luego de tratamiento del tumor: Comunicación de 2 casos / Complete remission of nephrotic syndrome associated with carcinoma of the cervix after treatment of the tumor: Report of two cases

Nephrotic syndrome secondary to paraneoplastic glomerulopathies is exceptional. We are aware of only three cases reported of cervical carcinomas associated with nephrotic syndrome. Two women, aged 40 and 79 years, presented with nephrotic syndrome. The first had a membranous nephropathy and the second was not biopsied. The first women had a metrorrhagia after 8 months of unsuccessful therapy with corticosteroids and immunosuppressive drugs. An advanced cervical carcinoma with lymph node metastases was found. In the second patient, a cervical carcinoma and hematometra was discovered two months after diagnosis ofa nephrotic syndrome. The syndrome subsided completely, nine months after radiotherapy and chemotherapy in the first patient and 10 months after hysterectomy in the second patient.

Linfangiectasia renal unilateral: Caso clínico / Unilateral renal limphangiectasia: Report of one case

Background: Renal lymphangiectasia is a rare disorder characterized by the presence of cystic masses in renal parenchyma, renal sinus or perinephric space. This condition, congenital or acquired, is probably caused by an alteration of renal lymphatic drainage to retroperitoneal lymph ducts. We report a 48-year-old woman, who consulted in the emergency room due to left flank pain and microscopic hematuria. Blood pressure and renal function were normal. A CT scan showed a multilocular cystic mass in the left renal sinus without alterations in the renal parenchyma. Renal lymphangiomatosis was diagnosed. The pain subsided with analgesics and did not recur. Annual clinical and ultrasound follow-up was suggested.

Enfermedad de Erdheim-Chester. Una causa rara de derrame pericárdico: Caso clínico / Erdhei-Chester disease: Report of one case

We report a 76-year-old male who was admitted due to progressive congestive heart failure lasting several months. An echocardiogram showed a large pericardial effusion with early signs of pericardial tamponade and an irregular surface suggestive of cancer infiltration. The patient was operated, creating a pericardial window and draining 1,200 ml of a brownish yellow fluid with abundant cellularity. Pericardial biopsy showed infiltration by CD68 (+), CD1a (-) and S100 (-) cells. Twenty-eight months earlier, due to fatigue, dyspnea, and a non-specific inflammatory process, an enhanced-contrast-scan showed that aorta was coated with a hypodense tissue that began near the aortic valve and extended until the inferior mesenteric artery, with stenosis of the left subclavian, celiac axis, renal and upper mesenteric arteries. An angioplasty and stent placing was carried out in the last two arteries. Both kidneys had the appearance of "hairy kidneys". A bone scan showed increased uptake in femurs and tibiae and X-ray examination showed osteosclerosis in metaphysis and diaphysis. The diagnosis of Erdheim-Chester disease (non-Langerhans-cell histiocytosis) was made and the patient was treated with steroids and methotrexate.

Fibrosis retroperitoneal asociada a uso crónico de ergotamina. Tratamiento con colchicina y esteroides: Caso clínico / Retroperitoneal fibrosis associated with chronic use of ergotamine: Report of one case

Retroperitoneal fibrosis (RPF) associated with chronic use of ergotamine is a very rare disorder. We report a 45-year-old woman who presented with a RPf after using, almost daily for 23 years, ergotamine tartrate for migraine relief. FRP presented as a chronicinflammatory state, anemia, abdominal and lumbosacral pain and a hypogastric mass. A CT-Scan showed a periaortic mass and left hydronephrosis. A percutaneous biopsy was obtained and the patient was subjected to a surgical ureterolysis and tissue resection. The biopsy confirmed the presence of RPf. Due to persistent symptoms and increase in the volume of periaortic tissue, treatment with colchicine 1 mg/day and defazacort 30 mg/day was started, resulting in a rapid di-sappearance of symptoms, disappearance ofinflammation and a significant reduction in the volume of the periaortic tissue. The patient remains in complete remission after 29 months of follow up.

Pronóstico de pacientes ingresados a Unidad de Cuidados Intensivos por falla respiratoria asociada a presencia de infiltrados pulmonares / Prognosis of patients with acute respiratory failure and pulmonary infiltrates admitted to intensive care unit

En la unidad de cuidados intensivos (UCI), la insuficiencia respiratoria aguda (IRA) asociada a infiltrados pulmonares de causa desconocida se relaciona con alta morbilidad y mortalidad. El objetivo principal de este trabajo fue conocer el pronóstico de pacientes con IRA, infiltrados pulmonares y necesidad de ventilación mecánica (VM) ingresados a UCI. Como objetivo secundario se planteó evaluar la utilidad de medios diagnósticos de uso habitual en este grupo de pacientes en relación al pronóstico y la conducta terapéutica. Se realizó un estudio observacional retrospectivo que incluyó todos los pacientes ingresados a la UCI del Hospital Naval Almirante Nef por IRA de causa desconocida asociada a infiltrados pulmonares en radiografía de tórax y que tengan necesidad de VM entre los años 2006 y 2010. Se incluyeron 90 pacientes de los cuales 45,6 por ciento fueron de sexo masculino. La edad promedio fue 66 años (DS 17,1). El puntaje promedio de score APACHE II fue 20,6 (DS 7,7). La mortalidad fue 52,2 por ciento y el tiempo promedio de estadía en UCI fueron 14 días (DS 14,4). La principal causa de IRA fue síndrome de distress respiratorio agudo (32 por ciento).Otras causas fueron neumonía adquirida en la comunidad (28,2 por ciento), neumonía aspirativa (12,2 por ciento), neumonía nosocomial (7,8 por ciento) y neumonía criptogénica organizada (6,7 por ciento). La realización de tomografía computarizada de tórax, lavado broncoalveolar y biopsia pulmonar determinó cambio de conducta terapéutica en 31 por ciento, 58 por ciento y 88 por ciento de los casos, respectivamente. En conclusión, los pacientes con IRA tienen alta mortalidad. La causa más frecuente fue el síndrome de distress respiratorio agudo. El uso de procedimientos diagnósticos pueden cambiar las medidas terapéuticas empleadas, en especial la biopsia pulmonar.

In the intensive care unit (ICU), acute respiratory failure (ARF) associated with unexplained pulmonary infiltrates is associated with high morbidity and mortality. The main objective of this study was to determine the prognosis of patients with ARF and pulmonary infiltrates admitted to ICU. The secondary objective was proposed to evaluate the usefulness of diagnostic methods frequently used in this patient group and correlated them with prognosis and therapeutic management. We performed a retrospective observational study that included all patients admitted to ICU of Almirante Nef Naval Hospital with ARF of unknown cause associated with pulmonary infiltrates on chest radiography during the years 2006-2010. We included 90 patients of which 45 percent were male. The average age was 66 years. The average APACHE II score was 20.6. Mortality was 52.2 percent and the average length of stay in the ICU was 14 days (SD 14.4). The main cause of ARF was acute respiratory distress syndrome (32 percent). Other causes were community-acquired pneumonia (28.2 percent), aspiration pneumonia (12.2 percent), nosocomial pneumonia (7.8 percent) and cryptogenic organized pneumonia (6.7 percent. The performance of chest computed tomography, bronchoalveolar lavage and lung biopsy determined therapeutic behavior change in 31 percent 58 percent and 88 percent of cases, respectively. In conclusion, patients with ARF have high mortality. The most common cause was acute respiratory distress syndrome. The use of diagnostic procedures may change the therapeutic measures used, particularly lung biopsy.

Rol de la TC multicorte en las hernias diafragmáticas: Ensayo iconográfico / Role of Multislice Computed Tomography in the evaluation of diaphragmatic hernias: Pictorial essay

Las hernias diafragmáticas consisten en la migración de estructuras abdominales hacia el tórax a través de un defecto del diafragma. Ellas pueden tener origen congénito (de Morgagni y de Bochdalek) o adquirido, incluyendo las traumáticas o no traumáticas (del hiato, defectos diafragmáticos posteriores). Debido a que en algunas hernias diafragmáticas está indicada la reparación quirúrgica, los métodos de diagnóstico por imágenes cumplen un rol fundamental. La TC multicorte, con su capacidad multiplanar y posibilidad de efectuar cortes finos, nos permite valorar y caracterizar adecuadamente el defecto diafragmático y sus complicaciones. En este ensayo iconográfico realizamos un breve repaso de la embriología y anatomía del diafragma, revisamos los distintos tipos de hernias diafragmáticas y la utilidad de la TC multicorte.

Diaphragmatic hernias are characterized by the migration of abdominal structures into the chest through a diaphragmatic defect. These may have either a congenital etiology (e.g., Morgagni and Bochdalek), or an acquired etiology, including traumatic and nontraumatic hernias (hiatal, posterior diaphragmatic defects). Since a surgical repair is indicated in certain types of hernias, imaging diagnostic methods play a key role. Multislice Computed Tomography (MSCT) allows multiplanar views and thin section evaluation, thus providing a useful tool for the assessment and characterization of the diaphragmatic defect and its complications. In this pictorial essay we briefly review the diaphragm anatomy and embryology, the different types of diaphragmatic hernias and the role of MSCT.

Hiponatremia sintomática como forma de presentación clínica de un macroadenoma hipofisiario: Comunicación de tres casos / Hyponatremia associated to pituitary adenomas: Report of three patients

Severe hyponatremia occurring as the presenting feature of hypopituitarism secondary to pituitary adenomas is rare. We report three patients with this condition: Two elderly males (74 and 78 year-old) presenting with impaired consciousness and low plasma sodium after an episode of diarrhea and a 56-year-old male presenting with impaired consciousness after an episode of vomiting. All had clinical features of hypopituitarism and pituitary adenomas were found on imaging studies. Two were subjected to a trans sphenoidal resection of the adenoma.

Estadificación del cáncer de pulmón no células pequeñas: estado del arte / Cancer staging non-small cell lung: state of the art

El cáncer de pulmón es la neoplasia maligna más frecuente a nivel mundial, siendo la principal causa de muerte por cáncer en ambos sexos en Argentina. El pronóstico se correlaciona con el estadio clínico y la estirpe histológica tumoral. La clasificación del estadio clínico de cáncer de pulmón está basado en el sistema TNM y permite agrupar a pacientes con similares pronóstico y tratamiento. Los estudios de diagnóstico por imágenes juegan un rol muy importante en la detección, diagnóstico y estadificación del cáncer de pulmón, como así también en el monitoreo de recurrencias y respuesta al tratamiento, provocando un gran impacto en el manejo del paciente. Este trabajo de revisión tratará la estadificación del cáncer de pulmón de células no pequeñas, haciendo énfasis en el rol de los distintos métodos de diagnóstico por imágen.

Hemangiopericitoma intradural de la columna lumbar / Intradural hemangiopericytoma of the lumbar spine

El hemangiopericitoma es un raro tumor que puede compromeeter a la columna espinal, siendo la localización intradural extremadamente infrecuente. Se presenta un caso de un paciente de sexo masculino, de 54 años de edad, que consulta por lumbocitalgia de meses de evolución, con diagnóstico de hemangiopericitoma intradural de la columna lumbar. Se describen los hallazgos obtenidos en la RM y en la anatomía patológica

Schwannoma retroperitoneal: comunicación de 5 casos / Retroperitoneal schawannoma: diagnostic imaging findings in 5 patients

Propósito: Interpretar los distintos hallazgos imagenológicos a fin de llegar a una aproximación diagnóstica en este tipo de tumores. Material y método: Se estudiaron retrospectivamente 5 pacientes (3 de sexo masculino, 2 de sexo femenino), con schwannoma retroperitoneal. Presentaron un rango de edades de 33 a 66 años (media 54 años). Se interpretaron los hallazgos clínicos y fueron estudiados con ecografía, TC y/o RM. Las imágenes obtenidas fueron correlacionadas con los resultados histopatológicos. Resultados: El hallazgo clínico más frecuente fue de dolor abdominal (60 por ciento). Sólo los tumores que presentaron bordes bien delimitados y gran componente quístico permitieron sospechar el diagnóstico previo a la cirugía. A todos los pacientes se les realizó resección de la masa tumoral. Sólo 2 pacientes presentaron recidiva en un lapso de estudio de 3 años. Conclusión: El schwannoma retroperitoneal es un tumor muy infrecuente. En nuestra corta serie de casos, los distintos métodos de imágenes, en especial la RM, permitieron caracterizar la lesión y aproximar el diagnóstico

Edema periportal en TC: revisión de causas / Periportal halo on CT: spectrum of causes

Propósito: la presencia de un halo hipodenso periportal es un hallazgo de relativa frecuencia. Este halo es atribuido generalmente a edema o linfáticos dilatados. A través de esta muestra iconográfica nos proponemos ilustrar el aspecto del edema periportal y establecer sus causas. Material y métodos: se estudiaron retrospectivamente 78 pacientes por TC de abdomen con contraste iodado endovenoso, que presentaron edema periportal, en el lapso comprendido entre enero de 1998 y enero de 2003. Resultados: de los pacientes estudiados, 49 presentaron insuficiencia cardíaca congestiva (62,8 por ciento), 14 presentaron hepatitis viral (18 por ciento), 5 postransplante hepático (6,4 por ciento), 3 colangitis infecciosa (3,34 por ciento), 3 pacientes con trauma abdominal (3,8 por ciento), 2 neoplasias (2,6 por ciento) y 2 pacientes hepatitis medicamentosa (2,6 por ciento). Conclusión: el edema periportal es un hallazgo frecuente e inespecífico en pacientes con trastornos sistemáticos y enfermedades hepáticas. La integración de los distintos hallazgos asociados en TC permite en la mayoría de los casos establecer la etiología

Liposarcoma retroperitoneal: reporte de seis casos / Retroperitoneal liposarcoma: report of 6 cases

Propósito: Determinar la utilidad de la TC y RM en el diagnóstico del liposarcoma retroperitoneal en nuestra serie de casos. Material y métodos: Estudio retrospectivo de seis casos que fueron estudiados con TC y/o RM en pacientes con liposarcoma retroperitoneal diagnosticados en los últimos 4 años en nuestra institutción. Se analizaron los síntomas, tratamiento y pronóstico, con un enfoque especial en los hallazgos en la TC y RM, y su correlación anatomopatológica. Resultados: Los principales subtipos histológicos de nuestro grupo de pacientes, fueron tres liposarcomas pleomórficos, dos bien diferenciados, y uno de células redondas. Los distintos tipos de liposarcomas retroperitoneales, en especial los bien diferenciados, presentaron ciertos patrones imagenológicos que permitieron sospechar el subtipo tumoral. Se presentaron recidivas en un 50 por ciento (n=3). La mortalidad fue de un 33,3 por ciento (n=2). Conclusión: La TC y RM son métodos que ayudan a la detección de esta rara patología, a su aproximación diagnóstica, y a su posterior seguimiento

Leiomiosarcoma pelviano extraperitoneal: comunicación de un caso / Extraperitoneal pelvic leiomyosarcoma

El leiomiosarcoma pelviano extraperitoneal es una patología extremadamente infrecuente. Es un tumor altamente agresivo y el pronóstico es desfavorable. La presentación clínica es inespecífica y tardía, y generalmente se lo diagnostica en un estadio avanzado. Se presenta el caso de un leiomiosarcoma pelviano situado en el tabique rectovaginal en una paciente de 34 años de edad que consulta por tumoración vulvar e impotencia funcional del miembro inferior izquierdo. Se describen los hallazgos obtenidos por ecografía, Tomografía Computada (TC), Resonancia Magnética Nuclear (RM) y anatomía patológica

Linfangiomatosis musculoesquelética / Skeletal lymphangiomatosis

La linfangiomatosis musculoesquéletica ha sido descripta esporádicamente en la bibliografía radiológica y ortopédica. Este caso documenta el espectro imagenológico de una enfermedad poco frecuente y ayuda a identificar un potencial patrón diagnóstico. Se presenta una paciente de 35 años de edad que consulta por dolor dorsolumbar inespecífico, es estudiada mediante Rx simple, Tomografía Computada (TC) y Resonancia Magnética (RM). Los hallazgos radiológicos evidenciados permitieron confirmar la naturaleza benigna de la enfermedad. La linfangiomatosis musculoesquelética es una entidad benigna poco frecuente de etiología desconocida, que compromete a los vasos sanguíneos y linfáticos, produce lesiones quísticas difusas a nivel del esqueleto axial y apendicular y se acompaña de afectación visceral. El empleo de los distintos métodos de imágenes permite realizar el diagnóstico correcto