Cardiac Granulocytic Sarcoma (Chloroma): InVivo Diagnosis with Transesophageal

EchocardiographyPedro Marcos-Alberca, MD, Borja Ibanez, MD, Manuel Rey, MD, Alejandro Roman, MD,

Rosa Rabago, MD, Miguel Orejas, MD, Jose F. Tomas, MD, PhD, and Jeronimo Farre, MD, PhD,Madrid, Spain

Granulocytic sarcoma, or chloroma, is an uncom-mon presentation of acute leukemia. Cardiac in-volvement is very rare and usually diagnosed atautopsy. We present a case that discloses the essen-

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tial role of transesophageal echocardiography for itsin vivo diagnosis. The principal features with thisimaging technique are finely described. (J Am SocEchocardiogr 2004;17:1000-2.)

Granulocytic sarcoma is a local infiltrative massformed by immature granulocytic cells that cancomplicate acute or chronic myeloid leukemia.1

Cardiac granulocytic sarcoma is very rare,2-6 andeven more exceptional is its in vivo diagnosis on thebasis of imaging techniques, mainly echocardiogra-phy.7,8 The transesophageal echocardiographic(TEE) features of granulocytic sarcoma have beenexceptionally described.

CASE REPORT

A 39-year-old man was admitted into our hospital withprogressive fatigue, dyspnea, and edema in both legsduring the last 2 weeks. Five years before this admissionhe had been given a diagnosis of M1 acute myeloidleukemia and treated with standard chemotherapy. Arelapse of his hematologic disorder developed 1 year afterthe initial treatment, requiring an allogenic bone-marrowtransplantation. He remained thereafter in complete clin-ical remission.

On physical examination, he was tachypneic, his bloodpressure was 85/60 mm Hg, and he had pulmonaryauscultatory findings consistent with a right pleural effu-sion, hepatomegaly, and severe bilateral edema in thelower extremities. Moreover, neck veins were evidentlydistended to the angle of the jaw, with a tall A wave anda diastolic murmur heard along the lower parasternal

From the Departments of Cardiology and Hematology (A.R.,J.F.T.), Fundacion Jimenez Diaz, Universidad Autonoma.Reprint requests: Pedro Marcos-Alberca, MD, Laboratorio deEcocardiografia, Servicio de Cardiologia, Fundacion JimenezDiaz, Avenida Reyes Catolicos 2, 28040 Madrid, Spain (E-mail:marcosal@arrakis.es).0894-7317/$30.00Copyright 2004 by the American Society of Echocardiography.doi:10.1016/j.echo.2004.04.030

border, increasing with inspiration. A chest radiographconfirmed the presence of severe right pleural effusionand cardiomegaly. A transthoracic echocardiogram re-vealed mild pericardial effusion, severe thickening of theright ventricular free wall (suggesting infiltration of themyocardium), and a well-defined right atrial (RA) massextending through the tricuspid valve toward the rightventricle and producing a severe obstruction of the tricus-pid diastolic flow. In addition, the substernal view showedan enlarged liver with evident dilation of suprahepaticveins, together with a dilated inferior cava vein, notcollapsing with inspiration. TEE confirmed the presenceof a huge RA mass (29 � 35 mm) occluding almostcompletely the tricuspid orifice (Figure 1). Multiplaneexamination allowed us to show that the primary attach-ment of the mass was located at the junction between thesuperior cava vein and the RA (Figure 2). From this site themass extended toward the tricuspid ring across the an-terolateral part of the RA.

The peripheral blood count and the bone-marrowbiopsy specimen were unremarkable, but the analysis ofthe pleural effusion revealed the presence of large quan-tities of granulocytic cells with an immunophenotypeequal to that of the primary myeloid leukemic process.With the clinical suggestion of cardiac granulocytic sar-coma, the patient underwent intensive chemotherapy andthoracic radiotherapy for 8 consecutive weeks. At the endof this treatment, signs of right-sided heart failure haddisappeared. TEE was not carried out because of thesevere mucositis, but a transthoracic echocardiogramrevealed no pericardial effusion, and the absence of anyintracavitary mass or infiltration of the right ventricularmyocardium. Doppler examination exhibited the dramaticimprovement of the diastolic tricuspid flow after theresolution of the mass, according to the alleviation of thesymptoms and signs of right-sided heart failure (Figure 3).The latter evolution supports the diagnosis of cardiacgranulocytic sarcoma.6 A second allogenic bone-marrowtransplantation was subsequently performed.

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DISCUSSION

Granulocytic sarcoma, or chloroma, is an uncom-mon presentation of acute leukemia and, as in ourcase, can exist in the absence of leukemic cells inperipheral blood or bone-marrow biopsy speci-mens.9 Chloromas are most frequently found in theskin at the sites of venopunctures.1,9 Cardiac in-volvement has been reported, generally consistingof a massive myocardial infiltration.4,5,8 The presen-tation as an isolated and locally invasive intracavitarymass has been rarely reported.3,6,7 Most cardiacchloromas so far reported have been diagnosed atautopsy.2 Our case is rare in that it was diagnosedwhile the patient was still alive and the TEE featuresof this extremely unusual tumoral cardiac pathology

Figure 1 Transesophageal long-axis horizontal view at 0degrees. A, Huge mass (29 � 35 mm) is at tricuspid valvelevel (arrow). There is unusual increase in thickness of rightventricular (RV) free wall and mild pericardial effusion(arrowheads) suggesting presence of infiltrative process. B,Intravenous contrast (Optison, 0.5 mL) was administeredto better delineate mass and establish relationships withsurrounding cardiac structures. Mass had well-definedboundaries and polylobulated morphology. Mild contrastreplenishment denotes some degree of perfusion. LA, Leftatrium; LV, left ventricle; RA, right atrium.

are precisely described.7,8 It is noteworthy to sup-port our clinical suggestion in the result of thecytometric analysis of the samples obtained from thepleural effusion, considering the negativity of theresults in peripheral blood sample and bone-marrowbiopsy specimen, thus, avoiding the risks of a trans-jugular biopsy of the mass. The early diagnosispermitted in our case an aggressive treatment of thislife-threatening condition that resulted in a completeechocardiographic resolution of the cardiac involve-

Figure 2 Transesophageal long-axis vertical view at 90degrees showing left atrium (LA), right atrium (RA), andopening of superior cava vein (SCV). Mass, in anterolateralRA, attaches just at joint of SCV (arrow). There are alsopericardial and pleural effusions (arrowheads).

Figure 3 Comparison of 2-dimensional transthoracic echo-cardiogram (top) and Doppler analysis of diastolic tricuspidflow (bottom) at admission (A) and after 8 consecutiveweeks of chemotherapy and radiotherapy (B). Note disap-pearance of huge right atrial (RA) mass (arrow) and dra-matic improvement of diastolic flow: at inspiration, maxi-mal diastolic velocity decreased from 2.2 m/s at admissionto 1.1 m/s after end of treatment. Chart velocity in A is100 mm/s and in B is 25 mm/s. LA, Left atrium; LV, leftventricle; RV, right ventricle.

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ment and the disappearance of the clinical manifes-tations of tricuspid obstruction.

REFERENCES

1. Hutchinson RE, Kurec AS, Davey FR. Granulocytic sarcoma.Clin Lab Med 1990;10:889-91.

2. Foucar K, Foucar E, Willman C, Horvath A, Gerety RL. Non-leukemic granulocytic sarcoma of the heart: a report of a fatalcase. Am J Hematol 1987;25:325-32.

3. Jankovic M, Bonacina E, Masera G, Uderzo C, Galli MA,Ottaviani V, et al. Cardiac relapses in myeloid leukemia: casereport and review of the literature. Pediatr Hematol Oncol1987;4:237-45.

4. Tillawi IS, Variakojis D. Refractory right ventricular failure dueto granulocytic sarcoma. Arch Pathol Lab Med 1990;114:

983-5.

5. Orts M, Ribera JM, Calatrava A, Larrouse E, Catalan R, Na-varro JT, et al. Pseudotumorous cardiac infiltration in a patientwith acute monoblastic leukemia. Med Clin (Barc) 1996;106:545-7.

6. Ichikawa K, Ishizuka N, Shimamura K, Tanimoto K, Aoka Y,Nagashima H, et al. Mitral valve stenosis due to primary cardiacgranulocytic sarcoma relapsing 8 years after complete remis-sion: a case report. J Cardiol 2001;38:219-24.

7. Erdol C, Ovali E, Baykan M. Granulocytic sarcoma presentingas a right atrial mass. Acta Cardiol 2003;58:155-8.

8. Makaryus AN, Tung F, Liu W, Mangion J, Kort S. Extensiveneoplastic cardiac infiltration in a patient with acute myeloge-nous leukemia: role of echocardiography. Echocardiography2003;20:539-44.

9. Baer MR. Management of unusual presentations of acuteleukemia. Hematol Oncol Clin North Am 1993;7:275-

92.