Intraductal papillary neoplasia of the bile duct with malignancy: a differentiated entity of cholangiocarcinoma with a better prognosis. A review of three new cases.

INTRODUCTION: Intraductal papillary neoplasms of the bile duct have been recognized as a differentiated entity to other biliary tumors since 2001. They are characterized by intraductal growth, with or without mucus production, and have malignant potential, although they have a better prognosis than other types of cholangiocarcinoma. CASE REPORTS: From January 2010 to August 2015, we included three patients with a confirmed diagnosis of bile duct intraductal papillary neoplasia with malignancy that were treated at our center. Two cases were male and one female with a mean age of 67.3 years. All three patients presented malignancy at the time of diagnosis. One patient was asymptomatic and the diagnosis was reported in the hepatectomy specimen after a liver transplant due to autoimmune hepatitis. The other two patients presented with cholestasis and acute cholangitis and the diagnosis was made based on imaging tests (computed tomography [CT] and magnetic resonance imaging [MRI]) and endoscopic retrograde cholangiopancreatography (ERCP) with brushing and a biopsy. The treatment in both cases was surgical with a left hepatectomy, and resection of the left bile duct and segment I. They did not receive adjuvant treatment. None of the cases had recurrence of the disease. DISCUSSION: Malignant intraductal papillary neoplasias of the biliary tract represent a different entity of cholangiocarcinoma with a better prognosis. Its diagnosis is based on imaging tests and histology by ERCP. The treatment is surgical, achieving a high rate of success with a low relapse rate.

Graft versus host disease as a complication after liver transplantation: A rare but serious association.

UNLABELLED: The graft versus host disease after liver transplant is rare, with an incidence less than 1%, but with a high mortality (75-85%), especially due to infectious complications. It usually affects gastrointestinal tract, skin and blood system in the context of a normal liver graft function. There is no consensus on the most appropriate treatment: some articles support a reduction or even elimination of immunosuppressive drugs, while others published success with a dose increase. CLINICAL CASE: We report a case of a 68 year-old liver transplant recipient with a graft retrieved from an ABO identical cadaveric donor. After an uneventful postoperative period, he was readmitted presenting these symptoms: skin lesions, diarrhea and kidney failure. After ruling out infectious causes or drug toxicity, skin, duodenum and colon biopsies demonstrated characteristic histological changes of graft versus host disease grade III. Initially, supportive treatment along with methylprednisolone bolus were administered with good response. However, as the doses of corticosteroids decreased, the patient worsened again, requiring basiliximab. In spite of that, the patient progressively worsened with hematological involvement and, finally, an alteration of liver function tests prior to decease. The autopsy showed CMV and Herpes virus superinfection. DISCUSSION: We report a new case of graft-versus-host disease after liver transplantation with fatal evolution due to viral superinfection despite the employed measures.

Duodenal involvement by seminomatous tumors.

Testicular germ cell tumors, though rare (1%), represent the most common neoplasm among young men. Gastrointestinal involvement from these malignancies usually presents as bowel obstruction and digestive bleeding, but their frequency is low (5%). The patterns of this involvement are: infiltration from affected retroperitoneal lymph nodes or, less frequently, by peritoneal seeding and direct hematogenous spread. Particularly, infiltration of duodenum is also rare, though its real frequency is not well defined. Moreover, the affinity for GI tract differs among the histological types of GCT, being seminomatous tumors an exceedingly unfrequent cause of duodenal infiltration. We herein present a recent case in our institution of severe anemia due to gastrointestinal bleeding in the context of giant retroperitoneal bulky metastatic mass infiltrating duodenum as first manifestation of a testicular pure seminoma.

Survival analysis and identification of prognostic factors in colorectal liver metastasis after liver resection.

INTRODUCTION: Liver resection is the only curative treatment for colorectal liver metastasis. The identification of predictive factors leads to personalize patient management to enhance their long-term outcomes. This population-based study aimed to characterize factors associated with, and survival impact of patients who received hepatectomy for colorectal liver metastasis. METHODS: A retrospective cohort study of all the hepatectomies for colorectal liver metastasis performed at third-level hospital of Spain (2010-2018) was conducted. The Kaplan-Meier method was used for survival analyses. Multivariable Cox and regression models were used to determine prognostic factors associated with overall survival. RESULTS: The 5-year overall survival and disease-free survival were 42 and 33%, respectively. Survival analysis showed that metastasis features (number, largest size, distribution, and extrahepatic disease) and postsurgical factors (transfusion, major complications, and positive margin resection), as well as non-mutated KRAS, showed a significant association with survival. Otherwise, on multivariate analysis, only 5 independent risk factors were identified: major size metastasis >4 cm, RAS mutation, positive margin resection, intraoperative transfusion, and major complications. CONCLUSIONS: According to our findings, major size metastasis >4 cm, intraoperative transfusion, and major postoperative complications continue to be traditional prognostic factors. Meanwhile, the KRAS biomarker has a powerful impact as a survival prognostic factor.

Íleo biliar: reporte de casos / Biliary ileus: cases report

Resumen Introducción: El íleo biliar es una causa poco frecuente de obstrucción intestinal mecánica, siendo más frecuente en pacientes mayores de 65 años y sexo femenino. Casos Clínicos: Se exponen cuatro casos clínicos diagnosticados de íleo biliar, intervenidos en nuestro centro. Discusión: El íleo biliar es una enfermedad que se produce a consecuencia del paso de un cálculo al lumen intestinal. Esto se produce por la presencia de una fístula entre la vesícula y el intestino, localizada mayormente a nivel duodenal. Se manifiesta con una clínica muy inespecífica, lo que condiciona un retraso en su diagnóstico. La tomografía computarizada es actualmente el examen de elección. El tratamiento consiste en la enterotomía con extracción del cálculo, realizándose la reparación de la fístula en un primer o segundo tiempo. Tiene un mal pronóstico debido a comorbilidad de los pacientes y la demora diagnóstica y terapéutica.

Introduction: Gallstone ileus is a rare cause of mechanical intestinal obstruction, being more frequent in patients over 65 years and females. Clinical Cases: Four clinical cases diagnosed with gallstone ileus, operated on in our center, are reported. Discussion: Gallstone ileus frequently occurs as a result of a fistula that communicates the digestive tract with the gallbladder. Their clinic is very unspecific, which determines a delay in its diagnosis. Computed tomography is currently the gold standard. The treatment is enterolithotomy and fistula repair in a first or second stage. Prognosis is poor due to patient comorbidities and diagnostic and therapeutic delay.

Análisis de supervivencia e identificación de factores pronósticos de metástasis hepáticas de cáncer colorrectal tras resección hepática / Survival analysis and identification of prognostic factors in colorectal liver metastasis after liver resection

Introducción: El único tratamiento curativo para las metástasis hepáticas de cáncer colorrectal es la cirugía. La identificación de factores pronósticos nos permite individualizar el tratamiento de estos pacientes, mejorando sus resultados oncológicos a largo plazo. El objetivo de nuestro estudio es identificar la supervivencia y los factores pronósticos de pacientes sometidos a tratamiento quirúrgico de metástasis hepáticas de cáncer colorrectal. Métodos: Se trata de un estudio retrospectivo de todos los casos de hepatectomías por metástasis hepáticas de cáncer colorrectal, operadas en un hospital de tercer nivel en España, entre los años 2010 y 2018. Se realizó análisis de supervivencia univariante con las curvas de supervivencia Kaplan-Meier. Mediante el modelo multivariante regresión de Cox se determinaron los factores pronósticos asociados a la supervivencia global. Resultados: La supervivencia global y supervivencia libre de enfermedad de nuestra serie, a los 5 años fue del 43% y del 33%, respectivamente. En el análisis de supervivencia, las características de las metástasis hepáticas (números, tamaño mayor, distribución bilobar y enfermedad extrahepática) y postoperatorias (transfusión sanguínea, complicaciones graves y margen afecto), así como el KRAS no mutado resultaron estadísticamente significativos. No obstante, después de aplicar la regresión de Cox, solo se identificaron 5 factores de riesgo independientes: mutación del KRAS, metástasis hepáticas >4cm, transfusión intraoperatoria, complicaciones graves y margen afecto. Conclusiones: En nuestro grupo de pacientes hemos observado que el tamaño>4cm de la metástasis hepáticas, la transfusión intraoperatoria, las complicaciones posquirúrgicas graves y el margen afecto se mantienen como factores pronósticos tradicionales vigentes. Mientras que el biomarcador KRAS cobra un valor predictivo importante como factor pronóstico de supervivencia. (AU)

Introduction: Liver resection is the only curative treatment for colorectal liver metastasis. The identification of predictive factors leads to personalize patient management to enhance their long-term outcomes. This population-based study aimed to characterize factors associated with, and survival impact of patients who received hepatectomy for colorectal liver metastasis. Methods: A retrospective cohort study of all the hepatectomies for colorectal liver metastasis performed at third-level hospital of Spain (2010–2018) was conducted. The Kaplan–Meier method was used for survival analyses. Multivariable Cox and regression models were used to determine prognostic factors associated with overall survival. Results: The 5-year overall survival and disease-free survival were 42 and 33%, respectively. Survival analysis showed that metastasis features (number, largest size, distribution, and extrahepatic disease) and postsurgical factors (transfusion, major complications, and positive margin resection), as well as non-mutated KRAS, showed a significant association with survival. Otherwise, on multivariate analysis, only 5 independent risk factors were identified: major size metastasis>4cm, RAS mutation, positive margin resection, intraoperative transfusion, and major complications. Conclusions: According to our findings, major size metastasis>4cm, intraoperative transfusion, and major postoperative complications continue to be traditional prognostic factors. Meanwhile, the KRAS biomarker has a powerful impact as a survival prognostic factor. (AU)

Neoplasia papilar intraductal de los conductos biliares con malignización: una entidad diferenciada del colangiocarcinoma con mejor pronóstico. A propósito de tres nuevos casos / Intraductal papillary neoplasia of the bile duct with malignancy: a differentiated entity of cholangiocarcinoma with a better prognosis. A review of three new cases

Introducción: las neoplasias papilares intraductales de la vía biliar se han reconocido como una entidad diferenciada de otros tumores biliares desde 2001. Se caracteriza por un crecimiento intraductal, puede o no producir moco y tiene potencial de malignización, aunque con mejor pronóstico que otros tipos de colangiocarcinoma. Casos clínicos: desde enero de 2010 a agosto de 2015, incluimos tres pacientes con diagnóstico confirmado de neoplasia papilar intraductal de la vía biliar con malignización tratados en nuestro centro. Dos casos eran hombres y el tercero, una mujer con una edad media de 67,3 años. Los tres pacientes presentaban malignización en el momento del diagnóstico. Un paciente era asintomático y el hallazgo fue en la pieza de hepatectomía tras un trasplante hepático por virus de la hepatitis C (VHC). Los otros dos pacientes se presentaron con colestasis y colangitis aguda y el diagnóstico se estableció en base a las pruebas de imagen (tomografía axial computarizada [TAC] y resonancia magnética [RMN]) y colangiopancreatografía retrógrada endoscópica (CPRE) con cepillado y biopsia. El tratamiento en ambos fue quirúrgico con hepatectomía izquierda y resección del conducto biliar izquierdo y del segmento I. o recibieron tratamiento adyuvante. Ninguno de los tres pacientes presenta recidiva de la enfermedad. Discusión: las neoplasias papilares intraductales de la vía biliar malignizadas representan una entidad diferenciada de los colangiocarcinomas con un mejor pronóstico. Su diagnóstico se basa en las pruebas de imagen y en la histología por CPRE. El tratamiento es quirúrgico consiguiendo altas tasas de curación con baja recidiva (AU)

Introduction: Intraductal papillary neoplasms of the bile duct have been recognized as a differentiated entity to other biliary tumors since 2001. They are characterized by intraductal growth, with or without mucus production, and have malignant potential, although they have a better prognosis than other types of cholangiocarcinoma. Case reports: From January 2010 to August 2015, we included three patients with a confirmed diagnosis of bile duct intraductal papillary neoplasia with malignancy that were treated at our center. Two cases were male and one female with a mean age of 67.3 years. All three patients presented malignancy at the time of diagnosis. One patient was asymptomatic and the diagnosis was reported in the hepatectomy specimen after a liver transplant due to autoimmune hepatitis. The other two patients presented with cholestasis and acute cholangitis and the diagnosis was made based on imaging tests (computed tomography [CT] and magnetic resonance imaging [MRI]) and endoscopic retrograde cholangiopancreatography (ERCP) with brushing and a biopsy. The treatment in both cases was surgical with a left hepatectomy, and resection of the left bile duct and segment I. They did not receive adjuvant treatment. None of the cases had recurrence of the disease. Discussion: Malignant intraductal papillary neoplasias of the biliary tract represent a different entity of cholangiocarcinoma with a better prognosis. Its diagnosis is based on imaging tests and histology by ERCP. The treatment is surgical, achieving a high rate of success with a low relapse rate (AU)

Enfermedad de injerto contra huésped como complicación después de un trasplante hepático: una asociación rara pero grave / Graft versus host disease as a complication after liver transplantation: a rare but serious association

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Embolismo pulmonar por fistulización de quiste hidatídico hepático a vena cava inferior / Pulmonary embolism due to a liver hydatid cyst fistulized into the inferior cava vein

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Manifestación clínica atípica de un cáncer oculto de mama / Atypical clinical manifestations in occult breast cancer

El cáncer de mama oculto se define actualmente como aquella neoplasia que se expresa con una adenopatía metastásica, sin manifestación clínica ni radiológica del tumor primario en la mama. Su frecuencia es baja, del orden del 0,3 al 0,8% de todos los cánceres de mama. Presentamos el caso de una paciente con una adenopatía supraclavicular izquierda secundaria a carcinoma oculto de mama. A este diagnóstico se llegó tras múltiples estudios de imagen y después de detectar la mamaglobina en el estudio inmunohistoquímico de la adenopatía. Revisamos la literatura médica, sin que hayamos encontrado ningún caso publicado en el que un cáncer oculto de mama se manifieste a través de un ganglio supraclavicular izquierdo(AU)

Occult breast cancer is currently defined as that which is expressed in metastatic lymph node tumors without a clinical or radiographic finding of a primary tumor in the breast. The frequency of this entity is low, representing between 0.3 and 0.8% of all breast cancers. We report the case of a patient with left supraclavicular lymphadenopathy secondary to occult carcinoma of the breast. The diagnosis was reached after multiple imaging studies and detection of mammaglobin in the immunohistochemical analysis of the adenopathy. We reviewed the medical literature and found no published case of an occult breast tumor detected in a left supraclavicular node(AU)