ABSTRACT
Primary liver tumors are an increasing indication for pediatric liver transplantation. Here we report the cases of 10 patients who underwent liver transplantation for primary liver tumors in our hospital, from 2001 to date. Up to 2011, 1 transplant due to hepatoblastoma was done out of 117 liver transplants (0.8%). Since 2012, there were 9 patients out of 141 (6.4%) (5 due to hepatoblastoma, 2 due to hepatocellular carcinoma, 1 due to hepatic epithelioid hemangioendothelioma, and 1 due to hepatic mesenchymal hamartoma). Follow-up: 13.2 months (median); age at transplantation: living 4.7 years (median); weight: 17.6 kg (median). Eighty percent of patients received grafts from living donors. No tumor recurrence was observed. Survival was 100% in the follow-up period. In our series, patients with primary liver tumors requiring transplantation showed an adequate course, even in the case of hepatocellular carcinoma, Related living donors liver transplantation shortened the time between the indication and the surgery.
Los tumores hepáticos primarios son indicación creciente de trasplante hepático pediátrico. Reportamos los 10 pacientes con trasplantes hepáticos por tumores hepáticos primarios en nuestro centro desde 2001 hasta la actualidad. Hasta el año 2011, se realizó un trasplante por hepatoblastoma de 117 trasplantes hepáticos (0,8 %). Desde 2012, fueron 9 pacientes de 141 (6,4 %) (5 hepatoblastomas, 2 hepatocarcinomas, 1 hemangioendotelioma epitelioide hepático y 1 hamartoma mesenquimático hepático). Seguimiento 13,2 meses (media), edad al trasplante 4,7 años (media), peso 17,6 kg (mediana). El 80 % recibió injertos desde donantes relacionados. No hubo recurrencia tumoral y la sobrevida fue del 100 % en el período de seguimiento. En nuestra serie, los pacientes con tumores hepáticos primarios que requirieron trasplante presentaron buena evolución, aun en hepatocarcinoma. El trasplante hepático con donante relacionado acortó los tiempos entre la indicación y la realización.
ABSTRACT
The severity of non-alcoholic fatty liver disease (NAFLD) ranges from simple steatosis to steatohepatitis, and it is not yet clearly understood which patients will progress to liver fibrosis or cirrhosis. SPARC (Secreted Protein Acidic and Rich in Cysteine) has been involved in NAFLD pathogenesis in mice and humans. The aim of this study was to investigate the role of SPARC in inflammasome activation, and to evaluate the relationship between the hepatic expression of inflammasome genes and the biochemical and histological characteristics of NAFLD in obese patients. In vitro studies were conducted in a macrophage cell line and primary hepatocyte cultures to assess the effect of SPARC on inflammasome. A NAFLD model was established in SPARC knockout (SPARC-/-) and SPARC+/+ mice to explore inflammasome activation. A hepatic RNAseq database from NAFLD patients was analyzed to identify genes associated with SPARC expression. The results were validated in a prospective cohort of 59 morbidly obese patients with NAFLD undergoing bariatric surgery. Our results reveal that SPARC alone or in combination with saturated fatty acids promoted IL-1ß expression in cell cultures. SPARC-/- mice had reduced hepatic inflammasome activation during the progression of NAFLD. NAFLD patients showed increased expression of SPARC, NLRP3, CASP1, and IL-1ß. Gene ontology analysis revealed that genes positively correlated with SPARC are linked to inflammasome-related pathways during the progression of the disease, enabling the differentiation of patients between steatosis and steatohepatitis. In conclusion, SPARC may play a role in hepatic inflammasome activation in NAFLD.
Subject(s)
Non-alcoholic Fatty Liver Disease , Obesity, Morbid , Animals , Humans , Mice , Inflammasomes/metabolism , Liver/metabolism , Liver Cirrhosis/metabolism , Non-alcoholic Fatty Liver Disease/genetics , Non-alcoholic Fatty Liver Disease/complications , Obesity, Morbid/metabolism , Osteonectin/genetics , Osteonectin/metabolism , Prospective StudiesABSTRACT
El hemangioendotelioma epiteloide hepático (HEHE) es un tumor vascular raro de menor malignidad que el hemangiosarcoma. En los poco frecuentes casos unilobulares, puede indicarse hepatectomía parcial con riesgo de recurrencia agresiva; en enfermedad hepática extensa, incluso con compromiso extrahepático, el trasplante hepático ha resultado efectivo. Las metástasis son más frecuentes en pulmón,peritoneo, ganglios linfáticos, bazo y sistema nervioso. Se presenta el caso de un adolescente asintomático con HEHE con metástasis pulmonares y compromiso ganglionar abdominal que recibió trasplante hepático con evolución favorable.
Hepatic epithelioid hemangioendothelioma (HEHE) is a rare vascular tumor of less malignancy than hemangiosarcoma. In the rare unilobar cases, partial hepatectomy may be indicated with risk of aggressive recurrence; in extensive liver disease, even with extrahepatic involvement, liver transplantation has been performed successfully. Metastases are more common in the lung, peritoneum, lymph nodes, spleen, and nervous system. We present the case of an asymptomatic adolescent with HEHE with lung metastases and abdominal lymph node involvement who received a liver transplant with a favorable outcome.
Subject(s)
Humans , Male , Adolescent , Liver Transplantation , Hemangioendothelioma, Epithelioid/surgery , Hemangioendothelioma, Epithelioid/diagnosis , Hemangioendothelioma, Epithelioid/pathology , Liver Neoplasms/surgery , Liver Neoplasms/pathology , Lung Neoplasms/surgery , Treatment OutcomeABSTRACT
Hepatic epithelioid hemangioendothelioma (HEHE) is a rare vascular tumor of less malignancy than hemangiosarcoma. In the rare unilobar cases, partial hepatectomy may be indicated with risk of aggressive recurrence; in extensive liver disease, even with extrahepatic involvement, liver transplantation has been performed successfully. Metastases are more common in the lung, peritoneum, lymph nodes, spleen, and nervous system. We present the case of an asymptomatic adolescent with HEHE with lung metastases and abdominal lymph node involvement who received a liver transplant with a favorable outcome.
El hemangioendotelioma epiteloide hepático (HEHE) es un tumor vascular raro de menor malignidad que el hemangiosarcoma. En los poco frecuentes casos unilobulares, puede indicarse hepatectomía parcial con riesgo de recurrencia agresiva; en enfermedad hepática extensa, incluso con compromiso extrahepático, el trasplante hepático ha resultado efectivo. Las metástasis son más frecuentes en pulmón, peritoneo, ganglios linfáticos, bazo y sistema nervioso. Se presenta el caso de un adolescente asintomático con HEHE con metástasis pulmonares y compromiso ganglionar abdominal que recibió trasplante hepático con evolución favorable.
Subject(s)
Hemangioendothelioma, Epithelioid , Liver Neoplasms , Liver Transplantation , Lung Neoplasms , Adolescent , Hemangioendothelioma, Epithelioid/diagnosis , Hemangioendothelioma, Epithelioid/pathology , Hemangioendothelioma, Epithelioid/surgery , Humans , Liver Neoplasms/pathology , Liver Neoplasms/surgery , Lung Neoplasms/surgery , Treatment OutcomeABSTRACT
El tumor fibroso solitario de la pleura (TFSP) representa una neoplasia benigna de evolución habitualmente silente, hallazgo incidental y heterogeneidad en su presentación. Raramente se malignizan y la sintomatología se relaciona fundamentalmente con el compromiso por compresión de las estructuras vecinas. Su tratamiento es la cirugía. Se describe el caso de un paciente masculino de 49 años con antecedentes de hipertensión arterial, diabetes mellitus y obesidad que consultó al Servicio de urgencias por disnea. Se solicitó tomografía de tórax que evidenció voluminosa masa de densidad de partes blandas en hemitórax izquierdo con desplazamiento del mediastino y descenso del diafragma del mismo lado. La fibrobroncoscopia no evidenció lesión endoluminal. Fue evaluado por Cirugía del Tórax. Se realizó abordaje de la masa mediante toracotomía postero lateral izquierda. El estudio anatomopatológico informó formación tumoral de 25 × 16 × 13 cm, peso de 1905 gr y en la microscopía se describió proliferación celular fibroblástica, células fusiformes de núcleos pequeños ovoides y escaso citoplasma dispuestas en haces desordenados con colágeno interpuesto. La inmunohistoquímica informó vimentina y CD34 positivos. Se realizó el diagnóstico de tumor fibroso solitario de la pleura. La evolución posterior fue buena.
The solitary fibrous tumor of the pleura (SFTP) represents a benign neoplasm of commonly silent evolution, incidental finding and heterogeneous presentation. It rarely becomes malignant and symptoms mainly result from the involvement of neighboring structures due to compression. It is treated with surgery. We describe the case of a 49-year-old male patient with history of arterial hypertension, diabetes mellitus and obesity who consulted the Emergency Services because he was experiencing dyspnea. We requested chest tomography that showed a voluminous soft tissue density mass in the left hemithorax with mediastinal shift and decreased diaphragm on the same side. The fibrobronchoscopy didn't show endoluminal lesion. The patient was evaluated by the Thorax Surgery staff. The mass was treated by means of left posterolateral thorachotomy. The anatomopathological study reported the formation of a tumor of 25 × 16 ×13 cm and 1905 gr, and the microscopy described fibroblast cell proliferation, spindle cells of small ovoid nuclei and very little cytoplasm placed in disorganized bundles with collagen interposition. The immunohistochemistry disclosed positive vimentin and CD34. The patient was diagnosed with solitary fibrous tumor of the pleura, with good subsequent evolution.
Subject(s)
Solitary Fibrous Tumor, Pleural , Immunohistochemistry , NeoplasmsABSTRACT
The A.A.E.E.H has developed this guideline for the best care of patients with hepatocellular carcinoma (HCC) from Argentina. It was done from May 2018 to March 2020. Specific clinical research questions were systematically searched. The quality of evidence and level of recommendations were organized according to GRADE. HCC surveillance is strongly recommended with abdominal ultrasound (US) every six months in the population at risk for HCC (cirrhosis, hepatitis B or hepatitis C); it is suggested to add alpha-feto protein (AFP) levels in case of inexeperienced sonographers. Imaging diagnosis in patients at risk for HCC has high specificity and tumor biopsy is not mandatory. The Barcelona Clinic Liver Cancer algorithm is strongly recommended for HCC staging and treatment-decision processes. Liver resection is strongly recommended for patients without portal hypertension and preserved liver function. Composite models are suggested for liver transplant selection criteria. Therapies for HCC with robust clinical evidence include transarterial chemoembolization (TACE) and first to second line systemic treatment options (sorafenib, lenvatinib, regorafenib, cabozantinib and ramucirumab). Immunotherapy with nivolumab and pembrolizumab has failed to show statistical benefit but the novel combination of atezolizumab plus bevacizumab has recently shown survival benefit over sorafenib in frontline.
Subject(s)
Carcinoma, Hepatocellular/therapy , Decision Support Techniques , Liver Neoplasms/therapy , Medical Oncology/standards , Neoplasm Staging/standards , Algorithms , Argentina , Biopsy/standards , Carcinoma, Hepatocellular/diagnostic imaging , Carcinoma, Hepatocellular/mortality , Carcinoma, Hepatocellular/pathology , Clinical Decision-Making , Consensus , Evidence-Based Medicine/standards , Humans , Liver Neoplasms/diagnostic imaging , Liver Neoplasms/mortality , Liver Neoplasms/pathology , Predictive Value of Tests , Risk Assessment , Risk Factors , Treatment Outcome , Ultrasonography/standardsABSTRACT
La tolerancia operacional (ausencia de rechazo del injerto y buena evolución sin inmunosupresión) ha sido objeto de intensa investigación en trasplante hepático pediátrico en los últimos años. La morbimortalidad relacionada con la exposición a drogas inmunosupresoras a largo plazo en estos pacientes es bien conocida. Reportamos un caso de tolerancia operacional de nuestro centro en un receptor pediátrico de trasplante hepático libre de inmunosupresión desde los 16 meses postrasplante luego de una progresiva disminución a partir de su primoinfección asintomática por virus de Epstein-Barr, con buena evolución histológica y clínico-humoral en 22 meses de seguimiento. De acuerdo con nuestro conocimiento, este es el primer caso de tolerancia operacional en un receptor pediátrico de trasplante hepático reportado en nuestro país y creemos que debería profundizarse el estudio de estos pacientes para detectar características que permitan identificar una población potencialmente tolerante en la cual es posible disminuir y suspender la inmunosupresión.(AU)
Operational tolerance (absence of allograft rejection and good outcome without immunosuppression) has been object of intense research in pediatric liver transplant in the last years. The morbidity and mortality related to long-term immunosuppressive treatment of these patients are well known. We report a case of operational tolerance of our unit in a pediatric liver transplant recipient who is immunosuppressant-free since 16 month after transplant after progressive withdrawal related to asymptomatic Epstein-Barr virus first infection. He has good histological, clinical and serological outcome after 22 month of follow-up. To our knowledge, this is the first operational tolerance reported case in our country after liver transplant in a pediatric recipient and we believe that the study of these patients is important in order to detect characteristics that allow to identify a potentially tolerant group in which it is possible to withdraw immunosuppressive drugs.(AU)
ABSTRACT
La tolerancia operacional (ausencia de rechazo del injerto y buena evolución sin inmunosupresión) ha sido objeto de intensa investigación en trasplante hepático pediátrico en los últimos años. La morbimortalidad relacionada con la exposición a drogas inmunosupresoras a largo plazo en estos pacientes es bien conocida. Reportamos un caso de tolerancia operacional de nuestro centro en un receptor pediátrico de trasplante hepático libre de inmunosupresión desde los 16 meses postrasplante luego de una progresiva disminución a partir de su primoinfección asintomática por virus de Epstein-Barr, con buena evolución histológica y clínico-humoral en 22 meses de seguimiento. De acuerdo con nuestro conocimiento, este es el primer caso de tolerancia operacional en un receptor pediátrico de trasplante hepático reportado en nuestro país y creemos que debería profundizarse el estudio de estos pacientes para detectar características que permitan identificar una población potencialmente tolerante en la cual es posible disminuir y suspender la inmunosupresión.
Operational tolerance (absence of allograft rejection and good outcome without immunosuppression) has been object of intense research in pediatric liver transplant in the last years. The morbidity and mortality related to long-term immunosuppressive treatment of these patients are well known. We report a case of operational tolerance of our unit in a pediatric liver transplant recipient who is immunosuppressant-free since 16 month after transplant after progressive withdrawal related to asymptomatic Epstein-Barr virus first infection. He has good histological, clinical and serological outcome after 22 month of follow-up. To our knowledge, this is the first operational tolerance reported case in our country after liver transplant in a pediatric recipient and we believe that the study of these patients is important in order to detect characteristics that allow to identify a potentially tolerant group in which it is possible to withdraw immunosuppressive drugs.
Subject(s)
Humans , Male , Child, Preschool , Pediatrics , Immunosuppression Therapy , Liver Transplantation , Transplantation Tolerance , Immune ToleranceABSTRACT
Operational tolerance (absence of allograft rejection and good outcome without immunosuppression) has been object of intense research in pediatric liver transplant in the last years. The morbidity and mortality related to long-term immunosuppressive treatment of these patients are well known. We report a case of operational tolerance of our unit in a pediatric liver transplant recipient who is immunosuppressant-free since 16 month after transplant after progressive withdrawal related to asymptomatic Epstein-Barr virus first infection. He has good histological, clinical and serological outcome after 22 month of follow-up. To our knowledge, this is the first operational tolerance reported case in our country after liver transplant in a pediatric recipient and we believe that the study of these patients is important in order to detect characteristics that allow to identify a potentially tolerant group in which it is possible to withdraw immunosuppressive drugs.
Subject(s)
Liver Transplantation , Transplantation Tolerance , Child, Preschool , Humans , Immunosuppressive Agents , MaleABSTRACT
Operational tolerance (absence of allograft rejection and good outcome without immunosuppression) has been object of intense research in pediatric liver transplant in the last years. The morbidity and mortality related to long-term immunosuppressive treatment of these patients are well known. We report a case of operational tolerance of our unit in a pediatric liver transplant recipient who is immunosuppressant-free since 16 month after transplant after progressive withdrawal related to asymptomatic Epstein-Barr virus first infection. He has good histological, clinical and serological outcome after 22 month of follow-up. To our knowledge, this is the first operational tolerance reported case in our country after liver transplant in a pediatric recipient and we believe that the study of these patients is important in order to detect characteristics that allow to identify a potentially tolerant group in which it is possible to withdraw immunosuppressive drugs.