Primary cardiac tumours are rare entities and angiosarcoma is the most frequent primary cardiac malignant tumour. Mean
survival is six months and the tumour responds poorly to chemotherapy. We present the case of a 50 year-old patient with
localised pericardial angiosarcoma who survived 23 months after diagnosis with a combined approach of chemotherapy and
surgery

Branco, LM

Bravio, I

Cruz Ferreira, R

Matos, P

Pinto, E

Timóteo, AT

Repository of the Hospital Center of Central Lisbon

Pierwotny Mięsak Naczyniowy Osierdzia. Opis Przypadku i Przegląd Piśmiennictwa

Repositório do Centro Hospitalar de Lisboa Central, EPE

www.kardiologiapolska.viamedica.plChorzy trudni nietypowi/Case report Kardiologia Polska2010; 68, 7: 802–805Copyright © Via MedicaISSN 0022–9032Address for correspondence:Ana Teresa Timóteo, Cardiology Department, Santa Marta Hospital, Rua Santa Marta 1169-024 Lisboa, Portugal, tel: + 351 21 3594000,e-mail: ana_timoteo@yahoo.comReceived: 16.11.2009 Accepted: 18.11.2009Primary angiosarcoma of the pericardium:case report and review of the literaturePierwotny mięsak naczyniowy osierdzia: opis przypadku i przegląd piśmiennictwaAna Teresa Timóteo, Luísa Moura Branco, Ivan Bravio, Eugénia Pinto, Teresa Timoteo,Pedro Matos, Rui Cruz FerreiraSanta Marta Hospital, Lisbon, PortugalA b s t r a c tPrimary cardiac tumours are rare entities and angiosarcoma is the most frequent primary cardiac malignant tumour. Meansurvival is six months and the tumour responds poorly to chemotherapy. We present the case of a 50 year-old patient withlocalised pericardial angiosarcoma who survived 23 months after diagnosis with a combined approach of chemotherapy andsurgery.Key words: pericardial angiosarcoma, primary cardiac tumours, chemotherapyKardiol Pol 2010; 68, 7: 802–805INTRODUCTIONPrimary cardiac tumours are rare entities and angiosarcomais the most frequent primary malignant cardiac tumour [1, 2].The mean survival is six to eleven months and the tumourresponds poorly to chemotherapy [3, 4].CASE REPORTA 50 year-old female was referred to our institution for evalu-ation of pericardial effusion. She complained of persistent pre-cordial pain with onset five months before admission with pro-gressive development of dyspnea, fatigue, ankle oedema andincrease in abdominal volume in subsequent months. A chest-Xray, electrocardiogram and routine laboratory studies werenormal. A transthoracic echocardiogram (TTE) showed a mo-derate-sized pericardial effusion. Later on, the patient develo-ped tamponade and pericardiocentesis yield 700 mL of he-morrhagic fluid. Cytological and bacteriological tests of the flu-id were negative. The patient was treated for presumed viralpericarditis. Follow-up TTE showed residual small pericardialeffusion next to the right atrium, with an echodense image thatwas considered a residual organized hematoma. Two monthsafter drainage, symptoms recurred. At that time, TTE alsoshowed a bright echodensity in the right atrium posterior wall(35 ¥ 33 mm) and adjacent pericardial space, with another extrin-sic mass outside left atrium (24 ¥ 31 mm), confirmed by transe-sophageal echocardiography. The first mass was in the pericar-dial sac between the right atrium and left atrium, extendinginto the right atrium (Fig. 1). A thoracic computed tomography(CT)-scan done in the meantime showed diffuse thickening ofthe pericardial membrane and pathological lymph nodes in thesuperior and anterior mediastinum. The patient was again ad-mitted for evaluation. Laboratory studies showed an increasein erythrocyte sedimentation rate (ESR), as well as slight nor-mocytic normocromic anaemia. The electrocardiogram sho-wed sinus tachycardia and negative T waves in the anteriorand lateral leads. Abdominal and kidney ultrasound was nor-mal. Cardiac magnetic resonance imaging (MRI) confirmeddiffuse thickening of the pericardium with moderate to largepericardial effusion that caused constriction, with a haematicand solid component. There was a mass in the top of rightatrium, which invaded the wall as well as the superior media-stinum (namely superior vena cava) (Fig. 2, 3). The left atriumextrinsic mass was interpreted as a blood collection that com-pressed right lower pulmonary vein.www.kardiologiapolska.pl803Primary angiosarcoma of the pericardium: case report and review of the literatureThe clinical condition of the patient deteriorated quicklyand mediastinoscopy was performed with pericardiocente-sis, without significant removal of pericardial fluid, but withpericardial and lymph node biopsy. Pathological examina-tion revealed a papillary neoplasia composed of pleomorphicspindle cells arranged as interconnecting vascular spaces. Im-munohistochemical staining was positive for Vimentin andCD31, confirming vascular differentiation and supporting thediagnosis of poorly differentiated angiosarcoma (Fig. 4). Lymphnode biopsy showed only reactive inflammation. No otherlocalization was found for angiosarcoma. The patient wastransferred to the Oncology Department, where chemothe-rapy was started with Ifosfamide 5 g/m2 day 1, Epirrubicine50 mg/m2 day 1, Mesna 5 g/m2 day 1 and 2.5 mg/m2 day 2,every 3 weeks (nine cycles: cumulative dose of epirrubicine540 mg/m2). At six-month follow up, the patient was symp-tom-free with complete disappearance of pericardial effusionand no residual mass in the right atrium. A few months later,a small pericardial effusion and mass recurred, confirmed bycardiac-MRI, although the patient remained with few symp-toms (chest pain). Chemotherapy was restarted for more sixcycles (lipossomic doxorrubicine 30 mg/m2 day 1, instead ofepirrubicine). Positron emission tomography (PET)-scan exc-luded disseminated disease and the patient decided to un-Figure 1. A. Sub-costal view showing a mass inside the pericardium (with pericardial effusion) and extending to the right atriumfree wall and inside; B. Apical 4-chamber view showing the pericardial mass extending to the right atriumFigure 2. MRI Proton Density (Double IR) showing an isointensemass in right atrium wall, invading surrounding structures andalso pericardial effusionFigure 3. MRI T2 FATSAT sequence, which shows the samemass, now hyperintense, in the same location as described inFigure 2804www.kardiologiapolska.plAna Teresa Timóteo et al.derwent cardiac surgery at another institution. Intra-operati-vely, there was a localized mass in the pericardium that didnot seem to invade the right atrial wall, so the tumour wassurgically removed “en bloc” as well as right atrial free wallwhich was reconstructed using a pericardial patch. There washowever a new recurrent pericardial effusion detected at sur-gery, that was positive for malignant cells in post-operativepathological examination. In the first post-operative TTE (onemonth later), moderate heterogeneous pericardial effusion waspresent, mainly septated next to the right ventricular apex.No mass was detected in the right atrium. In subsequent TTE,pericardial effusion increased, without causing tamponade.A new PET-scan showed images of neoplastic masses around theheart. Chemotherapy was changed to Docetaxel 100 mg/m2due to a decrease in cardiac function, with poor toleranceand only slight improvement in pericardial effusion dimen-sions. The patient died with multiorgan failure 30 months afterthe first symptoms, 23 months after diagnosis and 13 monthsafter surgery.DISCUSSIONPrimary cardiac tumours are rare entities (0.0017–0.003% ofroutine autopsy studies, 25% malignant) [1]. Angiosarcoma isthe most frequent primary malignant cardiac tumour (31% ofall the malignancies) [2]. They have a tendency to occur inthe 3rd – 5th decade and are more common in males [5].Metastatic disease is very frequent at the time of diagnosis(66–89%), mainly locally (mediastinal lymph nodes, lungs andvertebra) [6]. In a retrospective analysis of 149 primary car-diac tumours, those arising from the pericardium (80%) andarising on the right side of the heart (43.5%) were usuallymalignant [7]. Those originating from the posterior wall of theright atrium increase the likelihood of a malignant process.These tumours have a silent evolution for a long time, withnon-specific symptoms [8].Primary cardiac tumours are often first detected by TTEwith pericardial effusion as in our case. Transesophageal echo-cardiography delineates the tumour more accurately, namelyits location and characteristics [7]. Computed tomography andMRI have excellent diagnostic advantages with regard totumour delineation, characterisation and spread [5]. In MRI,T1 weighted or proton density (Double IR) images are iso orhyperintense related to myocardium and T2 weighted are hy-perintense. These characteristics were present in our case. MRIalso allowed a more precise definition of localisation, contour,local spread and involvement of adjacent structures. When dra-ined, the fluid is usually hemorrhagic and cytological analysis isoften negative for malignant cells as in our patient [9]. The mainlocation is in the right atrium (90%) and pericardial involve-ment can cause pericardial constriction [9]. These tumours growrapidly with local invasion and distant metastasis. It is usuallya fungating pink-tan mass with vascular channels by endothe-lial malignant differentiation [9]. It stains immunohistochemi-cally positive for CD31, CD34 and Factor VIII, confirming va-scular differentiation (CD31 in our patient) [9].Mean survival is 6–11 months with some anecdotal ca-ses up to two years and one case up to four years [3, 10].Angiosarcoma responds poorly to chemotherapy [4]. Che-motherapeutic drugs used to treat cardiac angiosarcoma inc-lude adriamicin, ifosfamide, cyclophosfamide, vincristine ordacarbazine [5]. Most oncologists use adriamicine-based re-gimens. Results of surgical resection alone have been disco-uraging because most patients have advanced disease witha mean survival of nine months, depending on whether adju-vant treatment with radiation and/or chemotherapy was gi-ven [6]. Mean survival of patients with more localised masses,with tumour resection (more than half received adjuvant che-motherapy), is only ten months [6]. Orthotopic cardiac trans-plantation has been carried out in some cases, but survivaldid not differ from those without transplantation [11].Our patient had a very impressive initial response to che-motherapy during the first 6 months. However, the tumoursoon recurred. Although the lack of myocardial adhesionsand distant metastisation allowed surgical exeresis, and de-spite prolonged intensive chemotherapy, the patient diedbecause of pericardial recurrence. This is one of the longestsurvivals of a patient with cardiac angiosarcoma, in a patientwith a relatively localised tumour.Figure 4. A, B. Papillary neoplasia composed of atypical endothelial cells (pleomorphic spindle cells) arranged as interconnectingvascular spaces (HE); C. Immunohistochemistry with positive staining for CD31, confirming vascular differentiationwww.kardiologiapolska.pl805Primary angiosarcoma of the pericardium: case report and review of the literatureReferences1. Silverman NA. Primary cardia tumor. Ann Surg, 1980; 191: 127–138.2. Blondeau PH. Primary cardiac tumours. French studies of533 cases. Thorac Cardiovasc Surg, 1990; 38: 192–196.3. Dichek DA. Holmvang G, Fallon JT. Angiosarcoma of the heart:three-year survival and follow-up by nuclear resonance imag-ing. Am Heart J, 1988; 115: 1323–1324.4. Putnam JB Jr, Sweeney MS, Colon R et al. Primary cardiacsarcomas. Ann Thorac Surg, 1991; 51: 906–910.5. Kurian KK, Weisshaar D, Parekh H et al. Primary angiosarco-ma: case report and review of the literature. CardiovascularPathology, 2006; 15: 110–112.6. Herrmann MA, Shankerman RA, Edwards WD et al. Primarycardiac angiosarcoma: a clinico-pathological study of six cas-es. J Thorac Cardiovasc Surg, 1992; 103: 655–654.7. Qingyi M, Hong L, Lima J et al. Echocardiographic and patho-logical characteristics of primary cardiac tumors: a study of149 patients. Int J Card, 2002; 84: 69–75.8. Grande AM, Ragni T, Vigano M. Primary cardiac tumors.A clinical experience of 12 years. Tex Heart Inst J, 1993; 20:223–230.9. Tremaine LA, Gaertner EM. Persistent hemopericardial effu-sion in a 54-year-old-man. Arch Pathol Lab Med, 2005; 129:117–118.10. Cantella T, Oliva E, Andrade IG et al. A patient with cardiacangiosarcoma who survived for four years. Case report andliterature review. Rev Esp Cardiol, 2005; 58: 310–312.11. Kakizaki S, Takagi H, Hosaka Y. Cardiac angiosarcoma re-sponding to multidisciplinary treatment. Int J Cardiol, 1997;62: 273–275.

Pierwotny Mięsak Naczyniowy Osierdzia. Opis Przypadku i Przegląd Piśmiennictwa

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