RESUMEN
Infantile fibrosarcoma (IFS) is a rare pediatric tumor which often presents the ETV6-NTRK3 gene fusion. NTRK3 encodes the neurotrophin-3 growth factor receptor tyrosine kinase, a druggable therapeutic target. Selective tropomyosin receptor kinase (TRK) inhibitors, such as larotrectinib, have shown efficacy and safety in the treatment of IFS. We report a case of an abdominal IFS diagnosed in a newborn associated with an aortic aneurysm that was successfully treated with larotrectinib without relevant adverse effects.
Asunto(s)
Neoplasias Abdominales/tratamiento farmacológico , Aneurisma de la Aorta Abdominal/complicaciones , Fibrosarcoma/tratamiento farmacológico , Inhibidores de Proteínas Quinasas/uso terapéutico , Pirazoles/uso terapéutico , Pirimidinas/uso terapéutico , Neoplasias Abdominales/complicaciones , Neoplasias Abdominales/diagnóstico , Femenino , Fibrosarcoma/complicaciones , Fibrosarcoma/diagnóstico , Humanos , Lactante , Recién NacidoRESUMEN
Congenital extrahepatic portosystemic shunt (CEPS) is a rare condition in which the portomesenteric blood drains into a systemic vein, bypassing the liver through a complete or partial shunt. Most often, the diagnosis is made primarily with Doppler ultrasonography. Computed tomographic angiography and magnetic resonance angiography are used for further classification of the shunt and assessment of accompanying anomalies. Conventional angiography is necessary when results of the other tests disagree or are inconclusive. CEPS is classified into two types according to the pattern of anastomoses between the portal vein and systemic vein. In type 1, intrahepatic portal venous supply is absent; in type 2, intrahepatic portal venous supply is preserved. Type 1 usually occurs in girls with associated malformations, such as situs ambiguous with polysplenia and congenital heart defects. Associated anomalies are less frequent in type 2, and symptoms usually develop later without a gender preference. Hepatic encephalopathy and liver dysfunction are possible complications of both types and usually develop during adulthood. Both types are also associated with regenerative hepatic nodules. The clinical setting and imaging appearance of these nodules can help one avoid misdiagnosis. Definitive treatment of CEPS is determined by the type of shunt. Liver transplantation is the only effective treatment for symptomatic type 1 CEPS; surgical closure or embolization of the shunt is the therapeutic approach for type 2.
Asunto(s)
Malformaciones Arteriovenosas/diagnóstico , Sistema Porta/anomalías , Anomalías Múltiples , Angiografía , Malformaciones Arteriovenosas/terapia , Diagnóstico Diferencial , Humanos , Recién Nacido , Imagen por Resonancia Magnética , Tomografía Computarizada por Rayos X , Ultrasonografía DopplerRESUMEN
The "whirl sign" is an uncommon finding on emergency CT. However, it is easy to overlook if not kept in mind. Its recognition is of capital importance, being most of its causes potentially lethal. Surgical treatment is also mandatory when signs of complication are found. The whirl sign is usually found associated to midgut, cecal and sigmoid volvulus, small-bowel volvulus and closed-loop obstructions, and post-surgical mesenteric windows (including retroanastomotic hernias). CT is an optimal imaging technique to depict the so-called sign and associated CT features suggesting complication (circumferential wall thickening, pneumatosis intestinalis, pneumoperitoneum, mesenteric fat stranding, free intraperitoneal fluid, mesenteric haziness). Radiologists must be able to recognize the whirl sign and seek associated findings that strongly support the diagnosis of a spectrum of entities, some of them lethal if no treatment is established.
Asunto(s)
Interpretación de Imagen Radiográfica Asistida por Computador , Radiografía Abdominal , Tomografía Computarizada por Rayos X , Bases de Datos Factuales , Diagnóstico Diferencial , Medicina de Emergencia , Hernia/diagnóstico , Humanos , Vólvulo Intestinal/diagnóstico , Vólvulo Intestinal/diagnóstico por imagen , Intestino Delgado/diagnóstico por imagen , Mesenterio/diagnóstico por imagen , Estudios RetrospectivosRESUMEN
Surgical resection is the first-line curative treatment of hepatocellular carcinoma (HCC). However most patients are unable to undergo surgical resection because of advanced tumoral stage, severe liver dysfunction or poor clinical status. Therefore, image-guided tumor ablation techniques have been introduced for the treatment of unresectable HCC. Among them, radiofrequency ablation (RFA) has been demonstrated to be an effective alternative curative therapy. However, local ablative therapy for tumors located close to structures such as the diaphragm or gastrointestinal tract is technically challenging because of the risk of collateral thermal damage to nearby structures or incomplete treatment of the HCC resulting from poor visibility on sonography. The introduction of artificial ascites can separate adjacent organs from the tumor and improve the sonic window. The aim of this study was to evaluate the feasibility, safety and efficacy of RFA with artificial ascites for HCC adjacent to the diaphragm.
Asunto(s)
Carcinoma Hepatocelular/cirugía , Ablación por Catéter , Hepatectomía/métodos , Neoplasias Hepáticas/cirugía , Adulto , Anciano , Carcinoma Hepatocelular/etiología , Carcinoma Hepatocelular/terapia , Diafragma , Femenino , Hepatitis C Crónica/complicaciones , Humanos , Infusiones Parenterales , Cirrosis Hepática/complicaciones , Cirrosis Hepática Alcohólica/complicaciones , Neoplasias Hepáticas/etiología , Neoplasias Hepáticas/terapia , Masculino , Persona de Mediana Edad , Cirugía Asistida por Computador , Adherencias Tisulares/complicacionesAsunto(s)
Carcinoma de Células Escamosas/complicaciones , Infecciones por VIH/complicaciones , Neoplasias del Recto/complicaciones , Adulto , Carcinoma de Células Escamosas/diagnóstico , Carcinoma de Células Escamosas/cirugía , Humanos , Masculino , Neoplasias del Recto/diagnóstico , Neoplasias del Recto/cirugíaRESUMEN
La cirugía constituye la primera línea de tratamiento curativo en el hepatocarcinoma (CHC). Sin embargo, una gran mayoría no se puede beneficiar de la misma debido al avanzado estadio tumoral, disfunción hepática severa o mala condición clínica. Por ello, se han introducido varias técnicas de ablación guiadas por imagen. Entre ellas, la radiofrecuencia percutánea (RFp) constituye una alternativa eficaz como tratamiento curativo. Sin embargo, la localización de la lesión cerca de estructuras como el diafragma o el tracto gastrointestinal, limita esta técnica debido al riego de daño térmico colateral y a un tratamiento incompleto por mala visualización. La introducción de ascitis artificial previa puede separar el CHC de los órganos adyacentes y mejorar la ventana acústica. El objetivo de este estudio fue evaluar la viabilidad, seguridad y eficacia de la RFp con ascitis artificial para el tratamiento del CHC adyacente al diafragma (AU)
Surgical resection is the first-line curative treatment of hepatocellular carcinoma (HCC). However most patients are unable to undergo surgical resection because of advanced tumoral stage, severe liver dysfunction or poor clinical status. Therefore, image-guided tumor ablation techniques have been introduced for the treatment of unresectable HCC. Among them, radiofrequency ablation (RFA) has been demonstrated to be an effective alternative curative therapy. However, local ablative therapy for tumors located close to structures such as the diaphragm or gastrointestinal tract is technically challenging because of the risk of collateral thermal damage to nearby structures or incomplete treatment of the HCC resulting from poor visibility on sonography. The introduction of artificial ascites can separate adjacent organs from the tumor and improve the sonic window. The aim of this study was to evaluate the feasibility, safety and efficacy of RFA with artificial ascites for HCC adjacent to the diaphragm (AU)
Asunto(s)
Humanos , Masculino , Femenino , Persona de Mediana Edad , Anciano , /métodos , Carcinoma Hepatocelular/cirugía , Neoplasias Hepáticas/cirugía , Cavidad Peritoneal/cirugíaRESUMEN
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