RESUMEN
PURPOSE: To determine if rare primary malignancies of the liver may have consistent features on magnetic resonance imaging (MRI). MATERIALS AND METHODS: This IRB-compliant retrospective study reviewed the records from the pathology departments of four university centres over an 11-year period from 2005-2016 to identify rare primary malignant tumours, which were cross-referenced with MRI records. MRI studies of these patients were reviewed to determine if these tumours exhibited consistent and distinctive features. RESULTS: Sixty patients were identified with rare primary liver tumours. The following distinctive features and frequency of occurrence were observed: mixed hepatocellular carcinoma-cholangiocarcinoma showed regions of wash-out in 7/19 of patients; 6/6 of fibrolamellar carcinomas demonstrated large heterogeneous lesions with large heterogeneous central scars; epithelioid haemangioendothelioma larger than 2 cm showed target-like enhancement in late-phase enhancement in 9/13; sarcomas excluding angiosarcoma had central necrosis in 3/9 and haemorrhage in 5/9; angiosarcomas showed centripedal progressive nodular enhancement in 3/6 and showed regions of haemorrhage in 3/6; and 7/7 of primary hepatic lymphomas showed encasement of vessels. CONCLUSION: Although helpful features for the differentiation of rare primary malignancies of the liver are identified, no MRI features appear to be specific and therefore histopathological confirmation is usually required for definitive diagnosis. KEY POINTS: ⢠No MRI features appear to be specific for rare primary liver malignancies. ⢠Haemorrhage is a helpful sign in diagnosis of primary hepatic sarcomas. ⢠Angiosarcomas may show progressive nodular enhancement towards the centre mimicking haemangioma. ⢠Vessel encasement is a helpful sign in diagnosis of primary hepatic lymphoma.
Asunto(s)
Colangiocarcinoma/diagnóstico por imagen , Hemangioendotelioma Epitelioide/diagnóstico por imagen , Neoplasias Hepáticas/diagnóstico , Linfoma/diagnóstico por imagen , Imagen por Resonancia Magnética/métodos , Sarcoma/diagnóstico por imagen , Centros Médicos Académicos , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Carcinoma Hepatocelular/diagnóstico por imagen , Carcinoma Hepatocelular/patología , Colangiocarcinoma/patología , Diagnóstico Diferencial , Femenino , Hemangioendotelioma Epitelioide/patología , Humanos , Hígado/diagnóstico por imagen , Hígado/patología , Neoplasias Hepáticas/patología , Linfoma/patología , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Sarcoma/patología , Adulto JovenRESUMEN
A 39-year-old G3P3 female presented with abrupt onset dyspnoea of one month duration. She was markedly symptomatic when lying supine and resorted to prone sleeping. Chest X-ray reported as cardiomegaly. Transthoracic echocardiography was unremarkable twice. Computed tomography chest showed a dilated pulmonary artery. Transesophageal echocardiography identified a 12-mm ostium secundum atrial septal defect with mild pulmonary hypertension. The defect was closed with a cocoon device and rendered her symptom free. This case highlights the importance of timely organization of transesophageal echocardiography when transthoracic echocardiography is negative. It also illustrates marked dyspnoea could be a presentation of undiagnosed atrial septal defect with mild pulmonary hypertension.