Figure 1
Teaching Case
SectionCardiovascular
Case TypeClinical Cases
AuthorsMarie Gavrel1, Imen Ganzoui1, Julie Mayer1, Julia Chalaye2, Vania Tacher1, Emmanuel Itti2, Alain Rahmouni1, Jean-François Deux1
Connected authors
55 years, male
A 55-year-old man was recently diagnosed with a DLBCL on pathological examination of a cervical lymph node surgical biopsy. The patient had no cardiac symptom and no ECG abnormality. Baseline contrast-enhanced CT and 18F-FDG PET/CT demonstrated an inter-atrial septum mass. Cardiac MRI was performed to better delineate the lesion.
Cardiac involvement of a DLBCL revealed on initial 18F-FDG PET/CT.
Myxoma
Thrombus
CT showed cervical and axillary lymph nodes, renal involvement and a mass of the inter-atrial septum. SUV-max (Standardized Uptake Value) of this mass was 7.2 (Fig. 1a) in the range of the SUV of the other lesions (2.9 to 11.3).
Cardiac MRI delineates a 33x16 mm mass with well-defined contours, slightly bulging into the right atrium. Its signal intensity was identical to the myocardium on T1-weighted images (WI) and was hyper-intense on STIR T2-WI (Fig. 2a). On first-pass myocardial perfusion, its enhancement occurred at the same time but was less intense than the normal myocardium. On late gadolinium enhancement sequences, the signal of the mass was slightly higher than the signal of the adjacent myocardium (Fig. 2b, c).
After 2 cycles of chemotherapy (G-CHOP), the patient was considered in complete metabolic response according to Deauville Criteria [1] (Fig. 1b). MRI showed a residual thickening of the inter-atrial septum (Fig. 2d, e) with no detectable enhancement (Fig. 2f).
Cardiac MRI delineates a 33x16 mm mass with well-defined contours, slightly bulging into the right atrium. Its signal intensity was identical to the myocardium on T1-weighted images (WI) and was hyper-intense on STIR T2-WI (Fig. 2a). On first-pass myocardial perfusion, its enhancement occurred at the same time but was less intense than the normal myocardium. On late gadolinium enhancement sequences, the signal of the mass was slightly higher than the signal of the adjacent myocardium (Fig. 2b, c).
After 2 cycles of chemotherapy (G-CHOP), the patient was considered in complete metabolic response according to Deauville Criteria [1] (Fig. 1b). MRI showed a residual thickening of the inter-atrial septum (Fig. 2d, e) with no detectable enhancement (Fig. 2f).
According to autopsies, cardiac involvement is observed in 20-30% of patients with advanced stage lymphoma [2-3]. By contrast, primary cardiac lymphoma account for 0.5% of extranodal lymphomas at autopsy [3]. Nearly all primary cardiac lymphomas are aggressive B-cell lymphomas [2].
Cardiac lymphoma most commonly manifests as an ill-defined infiltrative mass of the myocardium [3] or as unique or multiple solid nodular masses [3-4]. The diffuse infiltrative form seems to be most commonly described in autopsy cases. We were able to find only 24 cases reported in the literature of cardiac lymphoma involvement demonstrated by imaging. Pericardial thickening or effusion associated with unique or multiple cardiac masses were the most commonly described presentations. The right ventricle and the right atrium are the most commonly involved sites. Compared to CT and US, cardiac MRI offers higher temporal resolution and additional tissue characterization [3-5]. Cardiac involvement appears with low signal on T1-WI and high signal on T2-WI compared to the myocardium. To our knowledge, dynamic enhancement has been described in only one case [6]. While myocardial FDG uptake is variable, 18F-FDG PET/CT imaging can reveal previously unsuspected cardiac involvement [7]. We found three previous cases of early fortuitous diagnosis of heart lymphoma [8-9] in which PET-CT was also useful in the monitoring of response to chemotherapy.
Primary cardiac tumours are rare, with an autopsy frequency of 0.001-0.3% [10]. The main differential diagnosis is myxoma, which accounts for approximately half of all primary cardiac neoplasms. Most myxomas are attached to the fossa ovalis of the inter-atrial septum. MRI shows an ovoid mass, with high signal intensity on T2-weighed sequences and heterogeneous late gadolinium enhancement. However, this tumour demonstrates generally low-grade or no significant FDG avidity [11-12]. At baseline, in our patient, the diagnosis of lymphoma versus myxoma was possible due to high FDG uptake. This diagnosis was confirmed on imaging after treatment (Fig. 1b).
Cardiac lymphoma most commonly manifests as an ill-defined infiltrative mass of the myocardium [3] or as unique or multiple solid nodular masses [3-4]. The diffuse infiltrative form seems to be most commonly described in autopsy cases. We were able to find only 24 cases reported in the literature of cardiac lymphoma involvement demonstrated by imaging. Pericardial thickening or effusion associated with unique or multiple cardiac masses were the most commonly described presentations. The right ventricle and the right atrium are the most commonly involved sites. Compared to CT and US, cardiac MRI offers higher temporal resolution and additional tissue characterization [3-5]. Cardiac involvement appears with low signal on T1-WI and high signal on T2-WI compared to the myocardium. To our knowledge, dynamic enhancement has been described in only one case [6]. While myocardial FDG uptake is variable, 18F-FDG PET/CT imaging can reveal previously unsuspected cardiac involvement [7]. We found three previous cases of early fortuitous diagnosis of heart lymphoma [8-9] in which PET-CT was also useful in the monitoring of response to chemotherapy.
Primary cardiac tumours are rare, with an autopsy frequency of 0.001-0.3% [10]. The main differential diagnosis is myxoma, which accounts for approximately half of all primary cardiac neoplasms. Most myxomas are attached to the fossa ovalis of the inter-atrial septum. MRI shows an ovoid mass, with high signal intensity on T2-weighed sequences and heterogeneous late gadolinium enhancement. However, this tumour demonstrates generally low-grade or no significant FDG avidity [11-12]. At baseline, in our patient, the diagnosis of lymphoma versus myxoma was possible due to high FDG uptake. This diagnosis was confirmed on imaging after treatment (Fig. 1b).
References
[1] Sally F. Barrington et al. (2014) Role of Imaging in the Staging and Response Assessment of Lymphoma: Consensus of the International Conference on Malignant Lymphomas Imaging Working Group. J Clin Oncol 32: 3048-3058 (PMID: 25113771)
[2] Manish Motwani et al. (2013) MR Imaging of Cardiac Tumors and Masses: A Review of Methods and Clinical Applications. Radiology 268(1):26-43 (PMID: 23793590)
[3] J Jeudy et al. (2012) Cardiac lymphoma: Radiologic-pathologic correlation. Radiographics 32:1369-1380 (PMID: 22977025)
[4] K. Reynen et al. (2004) Metastases to the heart. Annals of Oncology 15:375–381 (PMID: 14998838)
[5] O’ Donnell et al. (2009) Cardiac tumors: optimal cardiac MR sequences and spectrum of imaging appearances. AJR 193:377-387 (PMID: 19620434)
[6] Seok Jong Ryu et al. (2001) CT and MR Findings of Primary Cardiac Lymphoma: Report upon 2 Cases and Review. Yonsei Med J 42:451-456 (PMID: 11519090)
[7] Deirdre O\'Mahony et al. (2008) Cardiac Involvement with Lymphoma: A Review of the Literature. Clin Lymphoma Myeloma 8(4):249–252 (PMID: 18765314)
[8] Kanhaiyalal Agrawal et al. (2012) FDG PET/CT in Detection of Metastatic Involvement of Heart and Treatment Monitoring in Non-Hodgkin\'s Lymphoma. World J Nucl Med 11(1): 33–34 (PMID: 22942783)
[9] Julian A et al. (2011) FDG PET/CT leads to the detection of metastatic involvement of the heart in non-Hodgkin’s lymphoma. Eur J Nucl Med Mol Imaging 38:1174 (PMID: 21431399)
[10] Jagdish Butany et al. (2005) Cardiac tumours : diagnosis and management. Lancet Oncol 6: 219-28 (PMID: 15811617)
[11] Alan H. Maurer et al. (2011) How to Differenciate Benign versus Malignant Cardiac and Paracardiac 18F-FDG Uptake at Oncologic PET/CT. Radiographics 31:1287-1305 (PMID: 21918045)
[12] Kambiz Rahbar et al. (2012) Differentiation of Malignant and Benign Cardiac Tumors Using 18F-FDG PET/CT. J Nucl Med 53:1-8 (PMID: 22577239)
Case information
| URL: | https://www.eurorad.org/case/12620 |
| DOI: | 10.1594/EURORAD/CASE.12620 |
| ISSN: | 1563-4086 |
Figure 2
Department of Radiology, Henri Mondor Hospital and Paris-Est University, Créteil, France
Department of Radiology, Henri Mondor Hospital and Paris-Est University, Créteil, France
Department of Radiology, Henri Mondor Hospital and Paris-Est University, Créteil, France
Department of Radiology, Henri Mondor Hospital and Paris-Est University, Créteil, France
Department of Radiology, Henri Mondor Hospital and Paris-Est University, Créteil, France
Department of Radiology, Henri Mondor Hospital and Paris-Est University, Créteil, France