Tumor de células epiteliales perivasculares (PECOma) de origen pancreático. Descripción de un caso y revisión de la literatura / Pancreatic perivascular epithelial cell tumour (PECOma). Case report and literature review

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Liposarcoma del cordón espermático simulando una hernia inguinal incarcerada / Liposarcoma of the spermatic cord mimicking an incarcerated inguinal hernia

INTRODUCCIÓN: Los tumores paratesticulares representan del 7%-10% de las masas intraescrotales. Los sarcomas abarcan el 90% de las lesiones malignas del cordón espermático y de éstas, aproximadamente, el 3%-7% son liposarcomas. CASO CLÍNICO: Presentamos el caso de un varón de 45 años, que consultó en urgencias por una masa inguinoescrotal derecha no reductible, diagnosticándose de hernia inguinal incarcerada. Se realizó cirugía urgente evidenciando una gran tumoración de aspecto lipomatoso, dependiente de cordón espermático. Se realizó orquiectomía y hernioplastía inguinal. La anatomía patológica, reveló un liposarcoma bien diferenciado de cordón espermático. Posteriormente, se realizó estudio de extensión, sin afectación a distancia y no precisó tratamiento adyuvante. Actualmente, tras dos años de seguimiento no ha presentado recidiva. DISCUSIÓN: Sólo alrededor de 200 casos han sido comunicados previamente en la literatura y sólo 61 de éstos se presentaron simulando una hernia inguinal incarcerada. Debido a la baja incidencia de esta patología es difícil de conocer la historia natural y llegar a conclusiones sobre los resultados del tratamiento, el cual hasta el momento sigue siendo la orquiectomía radical, con escisión amplia de los tejidos locales. El papel de la radio y quimioterapia aun es controvertido. CONCLUSIONES: Los sarcomas del cordón espermático son neoplasias raras con alta tasa de recurrencia local. Su manejo inicial es quirúrgico. Se requiere de un alto índice de sospecha clínica para el diagnóstico ya que las implicaciones oncológicas varían en función del tratamiento que, en ocasiones, es llevado a cabo por cirujanos generales al simular una hernia inguinal.

INTRODUCTION: Paratesticular tumors represent 7%-10% of intraescrotal masses. Sarcomas account for 90% of malignant lesions of the spermatic cord and of these approximately 3%-7% are liposarcomas. CLINICAL CASE: This is the case of a 45 year old male who consulted in the emergency department for a non-reducible right inguino-scrotal mass and was diagnosed with an incarcerated inguinal hernia. Emergency surgery was performed which revealed a large lipomatous tumor, originating from the spermatic cord. Orchiectomy and hernioplasty were performed. Histopathology revealed a well-differentiated liposarcoma of the spermatic cord. Later extension study was conducted, without distant affectation, and did not require adjuvant treatment. Today, after two years of monitoring has been no recurrence. DISCUSSION: Only about 200 cases have been previously reported in the literature and only 61 of these were presented mimicking an incarcerated inguinal hernia. Due to the low incidence of this disease it is difficult to know the natural history and draw conclusions on the results of treatment, which so far remains the radical orchiectomy with wide local excision of the tissue. The role of radiotherapy and chemotherapy is still controversial

Fallo hepático posthepatectomía y fístula biliar: manejo multidisciplinar a propósito de un caso / Liver failure posthepatectomy and biliary fistula: multidisciplinar treatment

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Liver failure posthepatectomy and biliary fistula: multidisciplinar treatment.

The main cause of morbimor-mortality after major liver surgery is the development of liver failure posthepatectomy(LFPH). Treatment must involve multiple options and will be aggressive from the beginning. We report a case of a patient with cholangiocarcinoma perihilar treated with surgery: right hepatectomy extended to sI + IVb with develop of LFPH and biliary fistula and being management successfully in a multidisciplinary way.

Estudio descriptivo de la experiencia sobre carcinoma hepatocelular en hígado no cirrótico / Descriptive study of hepatocellular carcinoma in noncirrhotic liver

Introducción y objetivoEl carcinoma hepatocelular (CHC) es la sexta causa de cáncer. En el mundo occidental su incidencia está en aumento y la gran mayoría asientan sobre una cirrosis hepática. El objetivo del estudio fue evaluar las características del CHC en pacientes sin cirrosis.MétodosSe realizó una revisión retrospectiva de 469 pacientes con diagnóstico de CHC durante el periodo de enero de 2003 a diciembre de 2007. Se seleccionaron aquellos pacientes que cumplían criterios histológicos, o la conjunción de criterios clínicos, analíticos, radiológicos y/o de hemodinámica hepática, de ausencia de cirrosis hepática.ResultadosSe identificaron 29 pacientes con diagnóstico de CHC sobre hígado no cirrótico (6,2%). El 86,2% eran varones y con una mediana de edad de 61,6 (23 - 82) años. La enfermedad hepática fue hepatopatía crónica con fibrosis leve-moderada en el 68,9%. El 62,1% de los pacientes se encontraban asintomáticos al diagnóstico. El CHC era un nódulo único en el 86,2%, y la mediana del nódulo mayor fue 46 (20 - 150) mm. Se evidenció invasión vascular macroscópica en dos pacientes y metástasis óseas en otros dos. Se realizó tratamiento activo en 25 pacientes: 15 resección quirúrgica, 6 radiofrecuencia y 4 quimioembolización transarterial. La supervivencia acumulada a 1, 2 y 3 años, fue del 84,3; 67,2 y 50,1%, respectivamente.ConclusionesEl CHC sobre hígado no cirrótico es una patología poco frecuente, siendo tumores de gran tamaño al diagnóstico. A pesar de ello, se pueden aplicar tratamientos con intención curativa y alcanzar una supervivencia superior a la esperable en pacientes con cirrosis(AU)

Introduction and objectiveHepatocellular carcinoma (HCC) is the sixth leading cause of cancer. In western countries its impact is steadily growing and most of these tumors arise in cirrhotic liver. The aim of this study was to evaluate the incidence and characteristics of HCC developing in noncirrhotic patients. Methods: We conducted a retrospective review of 469 patients diagnosed with HCC between January 2003 and December 2007. Patients who met the histological criteria, or a combination of clinical, laboratory, imaging and hemodynamic criteria for the absence of cirrhosis were included.ResultsWe identified 29 patients with a diagnosis of HCC in non-cirrhotic liver (6.2%). Most (86.2%) were men and the median age was 61.6 (23 - 82) years. The most frequent histology of the liver was mild-moderate liver fibrosis (68.9%) and 62.1% of the patients were asymptomatic at diagnosis. HCC was a solitary nodule in 86.2%, and the median size of the main nodule was 46 (20 - 150) mm. Macroscopic vascular invasion was demonstrated in two patients and bone metastases in a further two patients. Active treatment was provided in 25 patients, consisting of tumoral resection in 15, radiofrequency ablation in six and transarterial chemoembolization in four. The overall cumulative survival at 1, 2 and 3years was 84.3%, 67.2% and 50.1%, respectively.ConclusionHCC arising in non-cirrhotic liver is uncommon and there is wide tumor extension at diagnosis. Nevertheless, in most patients, treatment with curative intent can be applied, achieving better survival than that expected patients with cirrhosis(AU)

[Descriptive study of hepatocellular carcinoma in noncirrhotic liver]. / Estudio descriptivo de la experiencia sobre carcinoma hepatocelular en hígado no cirrótico.

INTRODUCTION AND OBJECTIVE: Hepatocellular carcinoma (HCC) is the sixth leading cause of cancer. In western countries its impact is steadily growing and most of these tumors arise in cirrhotic liver. The aim of this study was to evaluate the incidence and characteristics of HCC developing in noncirrhotic patients. METHODS: We conducted a retrospective review of 469 patients diagnosed with HCC between January 2003 and December 2007. Patients who met the histological criteria, or a combination of clinical, laboratory, imaging and hemodynamic criteria for the absence of cirrhosis were included. RESULTS: We identified 29 patients with a diagnosis of HCC in non-cirrhotic liver (6.2%). Most (86.2%) were men and the median age was 61.6 (23 - 82) years. The most frequent histology of the liver was mild-moderate liver fibrosis (68.9%) and 62.1% of the patients were asymptomatic at diagnosis. HCC was a solitary nodule in 86.2%, and the median size of the main nodule was 46 (20 - 150) mm. Macroscopic vascular invasion was demonstrated in two patients and bone metastases in a further two patients. Active treatment was provided in 25 patients, consisting of tumoral resection in 15, radiofrequency ablation in six and transarterial chemoembolization in four. The overall cumulative survival at 1, 2 and 3 years was 84.3%, 67.2% and 50.1%, respectively. CONCLUSION: HCC arising in non-cirrhotic liver is uncommon and there is wide tumor extension at diagnosis. Nevertheless, in most patients, treatment with curative intent can be applied, achieving better survival than that expected patients with cirrhosis.