Cardiac angiosarcoma: A paradigmatical case?

Abstract

Primary cardiac angiosarcoma (AS) is an extremely rare malignanttumor associated with a dismal prognosis due to a rapid infiltratinggrowthandasystemicspread.Symptomsaregenerallylinkedtocardiactamponadeorcontractileimpairment,butaspecificmanifestationsmayalsocharacterizetheclinicalscenario.Thediagnosisisusuallysuggestedby transthoracic echocardiography and confirmed at computer tomog-raphy (CT) or at magnetic resonance imaging (MRI), although specifichistopathologicalandimmunohistochemicalassessmentsmaybeneed-ed at time [1].WedescribeacaseofafemalepatientadmittedtoourIntensiveCareUnit for severe respiratory insufficiency requiring mechanical ventila-tion and initially attributed to non cardiac-related disease because ofgenericsymptoms,withafinaldiagnosisofASresponsibleforacompli-cated and dismal clinical course.A 29-year-old woman was referred to our hospital for progressiveshortnessofbreathandrespiratorydistresswhichrequiredtrachealintu-bation immediately after hospitaliz ation and admittance to the IntensiveCareUnit.Thepatienthadbeenrecentlyhospitalizedtwiceinanotherpe-ripheralhospital forspontaneouspn eumothorax,enter orrhagiaandperi-cardialtamponade.Insuchpreviousadmittancesshehadbeensubmittedtocomplete,althoughnotconclusive,workup(includingpercutaneouspericardial fluid drainage with fluid assessment for tuberculosis orneoplastic cells, echocardiography, bronchoscopy and colonoscopy).Evidence of blood in the rectal ampulla had been found during thoseassessments,withembolizationofaninferiormesentericarterybranchthought to beresponsible for the intestinalbleeding. Noother findingshad been reported.Chest X-ray carried out in the ICU showed a right pneumothorax(Fig. 1A) that was immediately drained. ECG showed sinus rhythmand low voltage. The transesophageal echocardiography showed amarked pericardial effusion, percutaneously drained, an aneurismaticinteratrial septum, a large patent foramen ovale (8–10 cm), a giantright appendage, and a highly echogenic mass at the posterior wall.The atrial mass presented a mobile component with irregular profile,and crossed the tricuspid orifice, without apparent infiltration of thevalvular apparatus (Fig. 1B).The thoracic MRI confirmed the presence of the cardiac mass andshowedunspecifiednodulesspreadinbothlungs( Fig.1C).Atransvenousmyocardial biopsy was performed unsuccessfully, since the sample wasnot representative. Surgical resection of the cardiac mass was considerednot indicated because of the uncertain nature of the cardiac mass and thepoor patient clinical conditions.A sudden blindness and coma subsequently occurred. The brain CTscan documented several hemorrhagic lesions around the tentoriumterritory (Fig. 1D). Blood tests were within normal levels except fromhighPCR, procalcitonin,increasedspecific neuronal enolase, and severeanemia and thrombocytopenia (30,000).After this neurological event, patient general conditions further dete-riorated until the exitus at one week after the ICU admission.The autopsy revealed a cardiac mass consistent with spindle sarco-ma cells, secondary tumor localizations as well as several hemorrhagicareas in the brain and in both lungs. Immunohistochemical stainsshowed a positivity for the endothelial marker CD31, CD34, Fly1 andnegativity for the ORF 73 cytokeratin 7 and 20 and actin.The diagnosis of primary cardiac AS was finally confirmed (Fig. 2).Primary cardiac tumors are extremely rare with an incidence rang-ing between 0.001% and 0.03% in autopsy series [1]. Malignant cardiacneoplasmsoccurmoreoftenasmetastasisoflymphoma,leukemia,mel-anoma, and lung cancer. In adults, malignant primary tumors of theheart are quite rare (25% of all cardiactumors) and includeAS, rhabdo-myosarcoma and fibrosarcoma [1].While the most common benign primary tumors of the heart areusually single and affect the left cardiac side, malignant tumors distrib-ute equally between both sides of the heart. Angiosarcoma is the most