Hemangioendotelioma epitelioide: un tumor hepatico infrecuente / Epithelioid hemangioendothelioma: an uncommon liver tumor

Resumen Presentamos un caso de una paciente, remitida a nuestra unidad por presentar una lesión hepática sólida, sugestiva de metástasis, que tras biopsia, fue diagnosticada de hemangioendotelioma epitelioide hepático.ResumenSe trata de una entidad infrecuente, de curso clínico y pronóstico impredecibles, con un potencial letal difícil de prever. Destacamos su importancia por ser una entidad poco frecuente, en la que hay que pensar cuando detectamos una lesión hepática única, debiendo ser incluida en el diagnóstico diferencial con las metástasis hepáticas. Subrayamos la rareza del tumor, su presentación como lesión hepática única y la indicación de tratamiento quirúrgico.ResumenDescribimos las características clínico-patológicas de esta lesión, aportando un nuevo caso de hemangioendotelioma epitelioide hepático y analizando las distintas opciones terapéuticas(AU)

Abstract We report the case of a female patient who was referred to our unit because of a solid liver tumor, suggestive of metastasis. After biopsy, the patient was diagnosed with epithelioid hemangioendothelioma of the liver.AbstractEpithelioid hemangioendothelioma is a rare entity with an unpredictable, potentially fatal, clinical course and outcome. Due to its rarity, this entity should be considered when a solitary hepatic lesion is detected and should be included in the differential diagnosis with liver metastases. We highlight the infrequency of this tumor, its presentation as a solitary hepatic lesion and the indication of surgical treatment.AbstractWe describe the clinical and pathological characteristics of epithelioid hemangioendothelioma of the liver and report a new case of this entity. The distinct therapeutic options are discussed(AU)

[Epithelioid hemangioendothelioma: an uncommon liver tumor]. / Hemangioendotelioma epitelioide: un tumor hepatico infrecuente.

We report the case of a female patient who was referred to our unit because of a solid liver tumor, suggestive of metastasis. After biopsy, the patient was diagnosed with epithelioid hemangioendothelioma of the liver. Epithelioid hemangioendothelioma is a rare entity with an unpredictable, potentially fatal, clinical course and outcome. Due to its rarity, this entity should be considered when a solitary hepatic lesion is detected and should be included in the differential diagnosis with liver metastases. We highlight the infrequency of this tumor, its presentation as a solitary hepatic lesion and the indication of surgical treatment. We describe the clinical and pathological characteristics of epithelioid hemangioendothelioma of the liver and report a new case of this entity. The distinct therapeutic options are discussed.

Leiomiosarcoma de la vena cava inferior. Resección completa con reconstrucción vascular / Leiomyosarcoma of the inferior vena cava. Complete resection with vascular reconstruction

Los leiomiosarcomas de la vena cava inferior son tumores tan raros que se estima por debajo de los 200 pacientes bien documentados y publicados. Su incidencia es mayor en mujeres y con frecuencia aparecen entre los 50-60 años. Se originan en las células musculares de la capa media de la pared venosa y tienen, en general, una progresión lenta y un mal pronóstico. El diagnóstico se realiza mediante pruebas de imagen y biopsia guiada, pero el origen exacto del tumor se suele descubrir durante el acto quirúrgico y especialmente tras el estudio histológico definitivo. La cirugía es el único tratamiento que ha descrito modificaciones en la supervivencia. Presentamos el caso de un varón de 39 años con diagnóstico de leiomiosarcoma de la vena cava inferior, en su porción infrahepática y suprarrenal, tratado mediante cirugía y radioterapia postoperatoria, con una supervivencia libre de tumor a los 5 años (AU)

Combined liver transplantation plus imatinib for unresectable metastases of gastrointestinal stromal tumours.

Therapeutic options for treating unresectable hepatic metastases of leiomyosarcomas were scarce until a few years ago. Recent advances in the study of the biology of intestinal tumours have radically changed our knowledge of their pathogenesis. Many of the tumours previously considered as leiomyosarcomas are now identified as gastrointestinal stromal tumours (GISTs). The introduction of imatinib (an antineoplasic drug that specifically acts on the pathogenesis of these tumours) has shown promising results in patients with advanced GISTs. We present three patients with the initial diagnosis of unresectable hepatic metastases of leiomyosarcomas. They received liver transplants. All three had tumour recurrences after transplantation. Histological re-evaluation identified a stromal origin of the tumours, and the patients were treated with imatinib therapy (400 mg/day). Recurrence occurred in all patients after a mean of 38.3 months, but imatinib treatment achieved control of the tumours. The current survival times with the combination of transplantation and imatinib are 92, 48 and 46 months for the three patients. This series is small and inconclusive, but imatinib treatment showed promising results. The treatment options for patients with unresectable metastases of GISTs must be defined, as in these three patients liver transplantation achieved a disease-free status but all had tumour recurrences before starting the imatinib treatment.

Patrones de calidad en el manejo del carcinoma hepatocelular mediante resección hepática: criterios de selección y resultados en una unidad de referencia de cirugía hepatobiliar / Quality standards in the management of hepatocellular carcinoma with liver resection: selection and outcome criteria in a liver surgery reference unit

Introducción. Los resultados de la resección hepática parcial (RH) como tratamiento definitivo del carcinoma hepatocelular (CHC) pueden depender en gran medida de la adecuada selección de los pacientes y de la técnica quirúrgica. Para una mejor aplicación de estos métodos se han constituido en los últimos años unidades de referencia de cirugía hepática (UR).Objetivo. Evaluar los resultados de la RH en el CHC en una UR con pautas de selección y manejo definidos, orientados a la consecución de resultados estandarizados. Pacientes y método. Seleccionamos a 51 pacientes para tratamiento quirúrgico mediante RH. Los criterios de indicación fueron distintos para el grupo A (no cirróticos, 24 pacientes) y el grupo B (cirróticos, 27 pacientes).La técnica quirúrgica estuvo estandarizada. Utilizamos como criterios de calidad: la morbilidad, la mortalidad, la supervivencia total y libre de enfermedad y la recidiva. Resultados. La morbilidad fue del 18 por ciento (9 pacientes), no significativa en el número y tipo de complicaciones entre los 2 grupos. La mortalidad fue del 25,5 por ciento (13 pacientes), un 4 por ciento operatoria y un 16 por ciento por recidiva, no significativa entre los 2 grupos. La mediana de seguimiento fue de 20,5 meses. La supervivencia acumulada fue del 87, el 64 y el 48 por ciento a 1, 3 y 5 años (sin significación estadística entre los grupos).La supervivencia acumulada libre de enfermedad fue del 82, el 46 y el 41 por ciento a 1, 3 y 5 años (sin significación estadística entre los grupos). La recidiva se produjo en 14 pacientes (27,5 por ciento), sin diferencias significativas entre los grupos A y B. La recidiva apareció en un tiempo medio de 19 ñ 45 meses (rango, 5-40 meses). La acumulada a 5 años fue del 48 por ciento. Conclusiones. El esquema de actuación quirúrgica con relación a la RH para el CHC dentro de una UR ha permitido obtener unos resultados equiparables a los estándares de excelencia. Unas adecuadas selección e indicación, junto con las técnicas quirúrgicas disponibles, nos han permitido obtener unos resultados similares en pacientes cirróticos y no cirróticos (AU)

De novo internal neoplasms after liver transplantation: increased risk and aggressive behavior in recent years?

The goal of the study was to determine the incidence and variables associated with post-liver transplantation (LT) de novo internal neoplasms development, excluding skin tumors and hepatocellular carcinoma. Medical records were reviewed for recipient/donor demographics, viral serology, cause of liver disease, interval from LT to tumor diagnosis, predisposing factors, immunosuppression and survival. Forty-one neoplasms (31 solid and 10 hematologic) developed in 772 recipients (5.3%) transplanted between 1991 and 2001. Time to tumor diagnosis was longer in patients transplanted before 1995 than in those transplanted afterwards (58 vs. 22 months; p<0.05). Hematologic neoplasms (HN) appeared earlier than solid (2 vs. 21 months; p<0.001), were more prevalent in those transplanted after 1995 than before (32% vs. 12.5%), and had lower survival than solid (2 vs. 21 months, p<0.001). While HCV was the most frequent indication in HN (70%), alcohol was that of solid tumors (71%). Overall, risk factors for de novo neoplasms included alcohol and immunosuppression (p<0.01). In patients undergoing LT in recent years, there is a higher incidence of HN with de novo internal neoplasms developing at earlier time-points than in those transplanted years ago. Risk factors for tumor development include alcohol, HCV and possibly strong immunosuppression.

Delayed onset of severe hepatitis C-related liver damage following liver transplantation: a matter of concern?

Although histological hepatitis occurs in the majority of hepatitis C virus (HCV)-infected liver transplant recipients, the natural history is highly variable. Whereas progression to cirrhosis occurs in up to 30% after 3 to 7 years, the disease remains stable in another third of patients, in whom protocol liver biopsies might be avoided. However, there is recent concern that with prolonged follow-up, some patients with initial benign recurrence may develop a late-onset aggressive course. Aims of the study are to determine the incidence and factors associated with this event. Based on yearly protocol biopsies (median, five biopsies; range, three to seven biopsies), we evaluated the histological outcome of 57 HCV type 1b-infected transplant recipients with initial benign recurrence, defined as stable histological state (fibrosis stage F0 or F1) during the first 3 years posttransplantation. Severe late-onset liver damage is defined as progression to F3 or F4 in patients with previous benign recurrence. Potential predictors of this event include demographics, donor-related factors, liver enzyme levels at 1 and 3 (or baseline) years posttransplantation, activity grade and fibrosis stage at 1 and 3 years posttransplantation, nonalcoholic steatohepatitis-related variables occurring within the first 3 years posttransplantation (diabetes, hyperlipidemia, obesity), use of some drugs (renin-angiotensin inhibitors, ursodeoxycholic acid), and the advent of any unusual event. The incidence of severe late-onset liver damage was 35% (n = 20). Twelve transplant recipients progressed to F3, whereas 8 transplant recipients progressed to F4. Sudden histological deterioration was observed on postoperative biopsy 5 in 12 patients; biopsy 6 or 7, in 7 patients; and biopsy 4, in 1 patient. Variables associated with this event in univariate analysis were fibrosis stage and activity grade (and its components) at baseline (P <.0001), recipient female gender (P =.04), alanine aminotransferase (ALT) level at 1 year posttransplantation (P =.02), and aspartate aminotransferase (AST) and ALT levels at baseline (P =.008 and P =.005, respectively). By multivariate analysis, only one variable was retained in the model: fibrosis stage at baseline (relative risk, 11; 95% confidence interval, 3 to 41; P =.0007), whereas AST level almost reached statistical significance (P =.07). In conclusion, delayed HCV-related severe liver damage is not infrequent in transplant recipients with initial benign recurrence, occurring in approximately one third of them. The presence of some degree of fibrosis at baseline appears to predict this sudden change in the natural history of recurrent hepatitis C. Based on these findings, we recommend continuing protocol biopsies and evaluating potential antiviral therapy in transplant recipients with evidence of some fibrosis (even if it is only portal).

Hepatocellular carcinoma: Can it be considered a controversial indication for liver transplantation in centers with high rates of hepatitis C?

Hepatocellular carcinoma (HCC) is still considered a controversial indication for liver transplantation (LT), mainly because of long waiting times and underlying viral cirrhosis. The goal was to evaluate the outcome of LT in 104 patients with HCC and cirrhosis, mainly hepatitis C virus (HCV)-related, in a center with a short waiting time (median, 105 days). Four groups were formed according to the HCC and HCV status: HCV positive with HCC (group 1, n = 81), HCV negative with HCC (group 2, n = 23), HCV positive without HCC (group 3, n = 200), and HCV negative without HCC (group 4, n = 207). Predictive factors of tumor recurrence were demographics, tumor related (size or number of nodules, capsule, bilobar involvement, vascular or lymphatic invasion, clinical and pathologic TNM staging, pre-LT percutaneous ultrasound-guided ethanol injection or transarterial chemoembolization, alpha-fetoprotein levels), donor and surgery related, and year of transplantation. The same variables and "tumor recurrence (yes/no)" were applied to evaluate the effect on survival. The median follow up was 29 months (range, 0 to 104 months). Patient survival was 70% at 1 year and 59% at 5 years for group 1, 87% at 1 year and 77% at 5 years for group 2, 81% at 1 year and 64% at 5 years for group 3, and 88% at 1 year and 77% at 5 years for group 4 (P =.013). Survival was significantly lower in patients with HCC than in those without (74% and 63% versus 85% and 70%, at 1 and 5 years, respectively; P =.05). The causes of death in those with and without HCC were tumor recurrence (24%) and recurrent HCV (8%) versus sepsis (34%) and recurrent HCV (14%). HCC recurrence occurred in 12 patients (11.5%) at a median of 14 months (range, 3 to 60 months) with a probability increasing from 8% at 1 year to 16% at 5 years. In patients with HCC, tumor recurrence was associated with vascular invasion (P =.0004) by multivariate analysis; variables predictive of survival were donor old age (P =.01), viral-related etiology (P =.02), and tumor recurrence (P =.001). Although LT still remains an adequate indication for HCC in centers with high prevalence of HCV infection and short waiting times, both tumor and HCV-related recurrent diseases hamper significantly the outcomes of these patients.