Primary cardiac tumours are rare. Cardiac fibroma is a benign tumour of the heart. It is fairly common among children and adolescents and is rarely encountered in adults. We present the case of a thirty-eight year old lady who presented with shortness of breath and was found to have a very large intra-cardiac mass that had cardiac magnetic resonance (CMR) features consistent with cardiac fibroma. The patient was referred for tumour resection, however could not survive the surgery

Hasan, Khwaja Yousuf

Sultan, Fateh Ali Tipoo

English

eCommons@AKU

eCommons@AKUSection of Cardiology Department of MedicineJuly 2018Large intra-cardiac fibroma identified on cardiacMRI - a case report and review of literatureKhwaja Yousuf HasanDarulSehat Hospital.Fateh Ali Tipoo SultanAga Khan University, fatehali.tipoo@aku.eduFollow this and additional works at: https://ecommons.aku.edu/pakistan_fhs_mc_med_cardiolPart of the Cardiology CommonsRecommended CitationHasan, K. Y., Sultan, F. (2018). Large intra-cardiac fibroma identified on cardiac MRI - a case report and review of literature. JPMA.The Journal of the Pakistan Medical Association., 68(7), 1105-1107.Available at: https://ecommons.aku.edu/pakistan_fhs_mc_med_cardiol/106AbstractPrimary cardiac tumours are rare. Cardiac fibroma is abenign tumour of the heart. It is fairly common amongchildren and adolescents and is rarely encountered inadults. We present the case of a thirty-eight year old ladywho presented with shortness of breath and was found tohave a very large intra-cardiac mass that had cardiacmagnetic resonance (CMR) features consistent withcardiac fibroma. The patient was referred for tumourresection, however could not survive the surgery.Keywords: Fibroma, Cardiac MRI, Cardiac tumours,Cardiac fibroma, CMR.IntroductionCardiac fibromas are rare benign tumours of fibroblasts.They are the second most common primary cardiactumours among children; however they are rarely seen inadults.1 Their clinical presentation depends upon theirlocation and size. Rarely they may be asymptomatic andfound on routine imaging. Treatment is with complete orpartial resection of the tumour. We present here the casereport of a patient who was found to have a cardiacfibroma on Cardiac Magnetic Resonance Imaging (CMR).To the best of our knowledge this is the first case reportfrom Pakistan that describes the CMR features of a cardiacfibroma.Case ReportA thirty-eight year old lady with no known comorbidconditions presented with three months history ofprogressively worsening shortness of breath on exertion.She had an echocardiogram done at a local hospital thatrevealed a cardiac mass. The patient was referred to theAga Khan University Hospital for Cardiac MagneticResonance Imaging (CMR) in December 2014 for furtherevaluation of the mass.After taking informed consent from the patient forcarrying out the cardiac MRI and telling her that the studymay be used for academic writing or educationalpurposes, CMR was done on Siemens MAGNETOMAVANTO 1.5 Tesla system. The CMR revealed a large intra-cardiac mass (128x75 mm) embedded in theVol. 68, No. 7, July 20181105CASE REPORTLarge intra-cardiac fibroma identified on cardiac MRI — a case report andreview of literatureKhwaja Yousuf Hasan, Fateh Ali TipooAga Khan University Hospital, Karachi.Correspondence: Khwaja Yousuf Hasan. Email: drkyhasan82@gmail.comFigure-1: SSFP 4-chamber view showing a hypointense mass in the interventricularseptum, occupying most of left ventricular cavity.Figure-2: Delayed enhanced imaging (4-chamber view) showing marked late hyper-enhancement of the mass with gadolinium, which is a hallmark of cardiac fibroma.interventricular septum. It was hypo-intense on SteadyState Free Precision (SSFP) images, occupying major partof the left ventricular cavity causing left ventricularoutflow tract (LVOT) and left ventricular inflow tractobstruction. Figure-1 shows the SSFP 4-chamber viewwith the tumour in the interventricular septum andoccupying most of the left ventricular cavity. The leftventricular systolic function was also mildly reduced withan ejection fraction of 45%. On Turbo Spin Echo (TSE) T1weighted images the mass appeared iso-intense toslightly hyper-intense, while on TSE T2 weighted images itappeared hypo-intense. Delayed enhanced imaging withgadolinium showed marked hyper-enhancement of thewhole mass as seen in the 4-chamber view in Figure-2.These findings were consistent with cardiac fibroma. Thepatient was referred for surgical resection of the tumourhowever; she could not survive the operation.DiscussionPrimary cardiac tumours are rare. They have an autopsyfrequency of between 0.001-0.030%, with three-fourth ofthem being benign.2 Almost half of the benign cardiactumours are myxomas and the rest are papillaryfibroelastomas, rhabdomyomas, fibromas and lipomas.2Cardiac fibroma is the second most common paediatrictumour of the heart.1 The usual age at presentation is 13years3 with almost one-third patients presenting at lessthan one year of age.4 Only 15% patients present inadulthood.4 Our patient presented quite late at the age ofthirty-eight years which is uncommon for cardiacfibromas.Clinical presentation of patients with cardiac fibromadepends on the location and size of the tumour. Ourpatient presented with symptoms of progressivelyworsening shortness of breath. Patients with cardiacfibroma can present with symptoms of heart failure,arrhythmias, or sudden cardiac death. Quite a few casesare discovered incidentally on imaging, whereas a fewpatients may present with vague symptoms of chestpain. Tumour embolization is rarely seen in cardiacfibroma. The most common presenting symptom ofcardiac fibroma is progressively worsening heart failurethat occurs due to outflow or inflow tract obstruction,and obliteration of the left ventricular cavity as was seenin our patient.5In a recent systemic review and literature search, the leftventricle was found to be the most common site ofcardiac fibroma (57.3%), followed by the right ventricle(27.5%), inter-ventricular septum (17%), right atrium(5.3%) and left atrium (1.8%).6 In our case although thetumour was embedded in the inter-ventricular septum, itwas bulging into the left ventricular cavity occupyingmajor part of the left ventricle. Younger age at the time ofdiagnosis and involvement of the inter-ventricularseptum was associated with a greater incidence ofarrhythmias, conduction abnormalities and generally apoor prognosis.6A transthoracic echocardiogram is usually the firstinvestigation, whenever a cardiac tumour is suspected. Inour patient, the intra-cardiac mass was first identified on atransthoracic echocardiogram. Echocardiography offersexcellent information with regards to the location andfunctional impact of a cardiac tumour; however it has itslimitations in terms of tissue characterization. CMR,although time consuming and has limited availability,offers excellent tissue characterization. Unlike computedtomography (CT) it does not expose the patient toionizing radiation. Hoffmann et al7 compared histology ofcardiac tumours with CMR using a multi-parametric MRprotocol to evaluate signal properties, morphologiccharacteristics and contrast enhancement of cardiactumours in 55 patients. MR imaging had a diagnosticaccuracy of 0.92 (area under the curve) in determiningwhether the mass is malignant.Features on CMR that allow us to differentiate betweenbenign and malignant cardiac tumours include borders,size, location, and pericardial effusion. Benign tumoursare well defined with no irregularities or infiltration.8Malignant tumours are more likely to be ill-defined,lobular with invasive borders and may already beinvading the pericardium at the time of diagnosis.9Several studies have been done on CMR features ofcardiac tumours. The features that have consistentlybeen shown to favour a diagnosis of cardiac fibromainclude the following:10 an intra-myocardial location,especially the interventricular septum, well-definedborders with a thin rim of myocardium, hypo-intense onSSFP images, iso-intense to hyper-intense on T1-weighted images and intense homogenousenhancement on late gadolinium images. Similarfindings were observed in our patient.A number of case reports have now been published thatdescribe the CMR features of cardiac fibroma; however, tothe best of our knowledge, this is the first case report of itskind from Pakistan. Availability of histopathology wouldhave provided us more insight into this case butunfortunately it could not be acquired. Our patient wasappropriately sent for surgery, which is the only availabletreatment option for symptomatic patients with cardiacfibroma. Unfortunately the patient did not survive thesurgery and died on the table.J Pak Med Assoc1106 K. Y. Hasan, F. A. TipooConclusionPrimary cardiac tumours are rare and require a high levelof suspicion for their diagnosis. They are lethal if leftuntreated. An echocardiogram should always be done forpatients who present with signs and symptoms of heartfailure to identify possible causes of heart failure. CardiacMRI is an excellent tool for evaluation of patients withheart failure and suspected cardiac masses as theyprovide excellent tissue characterization and may giveinformation about the possible etiology of cardiac massthus foregoing the need for myocardial biopsy.Disclaimer: None to declare.Conflict of Interest: None to declare.Funding Disclosure: None to declare.References1. Burke A, Virmani R. Pediatric heart tumours. Cardiovasc Pathol.2008; 17:193-8.2. Centofanti P, Di Rosa E, Deorsola L, Dato GM, Patanè F, La Torre M,et al. Primary cardiac tumours: early and late results of surgicaltreatment in 91 patients. Ann Thorac Surg. 1999; 68:1236-41.3. Burke AP, Rosado-de-Christenson M, Templeton PA, Virmani R.Cardiac fibroma: clinicopathologic correlates and surgicaltreatment. J Thorac Cardiovasc Surg. 1994; 108:862-70.4. Grebenc ML, Rosado de Christenson ML, Burke AP, Green CE,Galvin JR. Primary cardiac and pericardial neoplasms: radiologic-pathologic correlation. Radiographics. 2000; 20:1073-103.5. Reynen K. Cardiac myxomas. N Engl J Med. 1995; 333: 1610-7.6. Torimitsu S, Nemoto T, Wakayama M, Okubo Y, Yokose T, KitaharaK, et al. Literature survey on epidemiology and pathology ofcardiac fibroma. Eur J Med Res. 2012; 17: 5.7. Hoffmann U, Globits S, Schima W, Loewe C, Puig S, Oberhuber G,et al. Usefulness of magnetic resonance imaging of cardiac andparacardiac masses. Am J Cardiol. 2003; 92: 890-5.8. Araoz PA, Mulvagh SL, Tazelaar HD, Julsrud PR, Breen JF. CT andMR imaging of benign primary cardiac neoplasms withechocardiographic correlation. Radiographics. 2000; 20: 1303-19.9. Araoz PA, Eklund HE, Welch TJ, Breen JF. CT and MRimaging of primary cardiac malignancies. Radiographics.1999; 19:1421-34.10. O Donnell DH, Abbara S, Chaithiraphan V, Yared K, Killeen RP, CuryRC, et al. Cardiac tumours: optimal cardiac MR sequences andspectrum of imaging appearances. AJR Am J Roentgenol. 2009;193:377-87.Vol. 68, No. 7, July 2018Large intra-cardiac fibroma identified on cardiac MRI — a case report and review of literature 1107

Large intra-cardiac fibroma identified on cardiac MRI - a case report and review of literature

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Large intra-cardiac fibroma identified on cardiac MRI - a case report and review of literature

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