title: Right cardiac intracavitary metastases from a primary intracranial myxofibrosarcoma.
abstract: Primary intracranial myxofibrosarcoma is exceedingly rare, with less than 10 cases published. We present a case of a 23-year-old man with previous history of a primary low grade myxofibrosarcoma of the left parietal-occipital convexity resected in March 1999. He subsequently underwent several interventions for multiple local recurrent disease until March 2004. At that time, complete remission was documented. About 8 years later, in February 2012, the patient was admitted to the emergency room with refractory acute pulmonary oedema. On work up, sustained monomorphic ventricular tachycardia and hyperechoic myocardial mass with invasion of the right ventricular cavity were detected. Electrical cardioversion was unsuccessful and irreversible cardiac arrest followed. The autopsy confirmed multiple bilateral lung metastases, malignant pulmonary embolism and myocardial invasion by the primary tumour, with intracavitary cardiac thrombosis and absence of intracranial disease. To the best of our knowledge, this is the first report of extracranial metastases of this neoplasm.
authors.affiliation: Department of Medical Oncology, Instituto Português de Oncologia de Lisboa Francisco Gentil, EPE, Lisbon, Portugal Underwater and Hyperbaric Medicine Centre, Portuguese Navy, Lisbon, Portugal Nova Medical School, Lisbon, Portugal.
authors.forename: Diogo Alpuim
authors.initials: DA
authors.lastname: Costa
authors.affiliation: Nova Medical School, Lisbon, Portugal Department of Medical Oncology, Centro Hospitalar de Lisboa Central, Lisbon, Portugal.
authors.forename: Pedro
authors.initials: P
authors.lastname: Barata
authors.affiliation: Department of Medical Oncology, Instituto Português de Oncologia de Lisboa Francisco Gentil, EPE, Lisbon, Portugal.
authors.forename: Emanuel
authors.initials: E
authors.lastname: Gouveia
authors.affiliation: Pathology Department, Centro Hospitalar de Lisboa Central, Lisbon, Portugal Instituto Português de Oncologia de Lisboa Francisco Gentil, EPE, Lisbon, Portugal.
authors.forename: Manuela
authors.initials: M
authors.lastname: Mafra
mesh_terms: D001932:Brain Neoplasms / Q000473:pathology / Q000601:surgery; D017809:Fatal Outcome; D005354:Fibrosarcoma / Q000473:pathology / Q000601:surgery*; D006338:Heart Neoplasms / Q000000981:diagnostic imaging* / Q000473:pathology; D006801:Humans; D008175:Lung Neoplasms / Q000000981:diagnostic imaging / Q000556:secondary*; D008297:Male; D009236:Myxosarcoma / Q000473:pathology / Q000601:surgery; D016609:Neoplasms, Second Primary / Q000000981:diagnostic imaging* / Q000473:pathology; D011654:Pulmonary Edema / Q000209:etiology* / Q000503:physiopathology; D055815:Young Adult