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Excisional lymph node biopsy with immunohistochemistry in a patient with B cell lymphoma. A. CD20 (+++). B. Bcl-2 (+, >80%). C. Bcl-6 (+). D. Mum-1 (+). E. Ki-67 (+, >80%). F. CD10 (slightly +).
Source publication
Objective
To summarize the clinical and pathological features of patients with cardiac lymphoma.
Methods
The general conditions, clinical features, pathological types, and prognostic indices of 37 patients with cardiac lymphoma treated in our hospital were analyzed.
Results
Among the 37 patients, only one had primary cardiac lymphoma, and the oth...
Citations
... They may manifest as pericardial effusion [6]. Nonetheless, patients usually present with extracardiac symptoms instead making the diagnosis cardiac involvement in systemic lymphomas more challenging and more likely to be missed [2,3]. ...
... When it occurs, it is more likely to be a secondary cardiac tumor, which may originate from melanoma, lymphoma, lung, breast or renal cancers [1]. Pericardium is the commonest site of involvement in secondary malignancies [1,2]. Systemic lymphoma may involve the heart in 20% of the cases [3,4]. ...
... In an early study by Peterson et al. in 1976, median onset of cardiac involvement is 20 months after an initial diagnosis of lymphoma [7]. As a result, symptoms and swelling outside the heart presents earlier, for an example, painless, superficial lymph node enlargement with systemic symptoms such as fever, night sweats and weight loss, whereas, heart related symptoms are delayed Fig. 1 Electrocardiogram showing right bundle branch block [2,3]. Thus, symptomatic, massive pericardial effusion as the presenting manifestation of high-grade systemic lymphoma, as seen in our case, is very rare [3]. ...
Background
Cardiac tamponade as the presenting manifestation of systemic lymphoma is relatively uncommon. Pericardium is the commonest site of involvement in secondary malignancies with systemic lymphoma involving the heart in 20% of the cases.
Case presentation
We describe a case of a 78-year-old gentleman, who presented with symptoms of new onset cardiac failure, and hemodynamic compromise. An echocardiography revealed cardiac tamponade, necessitating an emergency pericardiocentesis. With the aid of multimodality imaging, he was found to have a right atrioventricular groove mass, widespread lymph node enlargement with bone and peritoneal involvement. Ultimately, a histopathological evaluation revealed a diagnosis of Diffuse Large B Cell Lymphoma (DLBCL).
Conclusions
Our case illustrates that a patient with DLBCL may present with cardiac tamponade as a result of metastasis. This diagnosis, although rare, is likely to be missed, which can cause fatal complications, such as cardiac tamponade, fatal arrhythmias or sudden cardiac death.
... Le manifestazioni cliniche dipendono dal grado di infiltrazione e dal tessuto colpito e includono versamento pericardico, disfunzione ventricolare, insufficienza cardiaca, blocchi di conduzione, aritmie, ostruzione all'efflusso o morte improvvisa [9][10][11] . ...
Lymphoma patients are at high risk of cardiovascular events due to anthracycline cardiotoxicity and, in rare cases, related to heart infiltration. The presence of cardiac masses adds further complexity to the management of lymphoma patients beyond myocardial chemotherapy-related toxicity, given possible unpredictable acute complications such as arrhythmias, atrioventricular block, myocardial ischemia, pericardial ef-fusion and cardiac tamponade. Here we describe the clinical presentation and successful multidisciplinary management of diffuse large B-cell lymphoma with multifocal cardiac involvement identified by total body 18 FDG positron emission tomography performed at disease staging.
... Rosenberg et al. analyzed 1,269 patients with lymphoma when the imaging findings were not sufficient to diagnose cardiac involvement before the autopsy. Only 13 patients (1.1%) out of 1,269 had strong evidence of cardiac involvement with lymphoma during the clinical (19). Among these 37 patients, 36 had secondary cardiac lymphoma. ...
Objectives Although malignant lymphoma (ML) can occur in every organ, diagnosing cardiac involvement without cardiac manifestations is difficult. We therefore investigated the incidence of cardiac involvement in ML in our hospital and clarified the transthoracic echocardiography (TTE) findings of cardiac involvement.
Methods Patients with ML referred to our hospital between January 2013 and December 2019 were retrospectively reviewed.
Patients During the study period, 453 patients were identified. The mean age was 64.9 years old, and 54% of the patients were men.
Results Diffuse large B-cell lymphoma (DLBCL) was the most common lymphoma, followed by follicular lymphoma. Of the 453 patients, 394 (87.0%) underwent TTE at the initial diagnosis or during the clinical course. The performance rates of TTE in DLBCL, Hodgkin lymphoma, and mantle cell lymphoma were above 90%. Cardiac involvement was detected in 6 (five with DLBCL and one with B-cell lymphoma) (1.5%) of the 394 patients who underwent TTE. The involved lesions of the heart varied, and five patients had pericardial effusion. Five patients had a preserved left ventricular ejection fraction. All patients were treated with chemotherapy, and some were treated with radiation and surgery.
Conclusion Cardiac involvement was observed in six (1.5%) of the patients with ML who underwent TTE. B-cell lymphoma, especially DLBCL, is a common ML with cardiac involvement. Although five patients had pericardial effusion, the involved lesions of the heart were not uniform. TTE is a useful imaging modality to noninvasively and repeatedly evaluate the tumor characteristics, response to ML treatment, and cardiac function.
... 6 T-cell lymphomas are more likely to spread widely, with pericardial effusion being more closely linked to the expansion of the disease. 7 Ventricular thrombosis is generally due to malignancy-related hypercoagulability. The common presentation is a mass in one or multiple chambers of the heart, whereas an isolated lymphomatous infiltration of the myocardium is more rarely observed. ...
Background
Myocardial infiltration by primary cardiac neoplasm is a rare entity, providing diagnostic and therapeutic challenges. The pathological spectrum includes more frequently benign forms. Refractory heart failure, pericardial effusion and arrhythmias due to infiltrative mass are the most common clinical manifestations.
Case summary
We describe the case of a 35-year-old man complaining of shortness of breath and weight loss in the last two months. A previous acute myeloid leukemia treated with allogenic bone marrow transplant was reported. Transthoracic echocardiography revealed an apical thrombus in the left ventricle, with inferior and septal hypokinesia conditioning a mildly reduced ejection fraction, circumferential pericardial effusion and abnormal right ventricular thickening. Cardiac magnetic resonance confirmed diffuse thickening of the right ventricular free wall due to myocardial infiltration. Positron emission tomography showed the presence of neoplastic tissue with increased metabolic activity. A pericardiectomy was performed showing a widespread cardiac neoplastic infiltration. Histopathological analysis done on right ventricular pathological samples obtained during cardiac surgery revealed the presence of a rare and aggressive cardiac anaplastic T-cell non-Hodgkin lymphoma. Few days after the operation the patient developed refractory cardiogenic shock and unluckily died before initiating an adequate antineoplastic therapy.
Discussion
Primary cardiac lymphoma is not frequent and the lack of specific symptoms makes the diagnosis extremely challenging and often limited to autopsy findings. Our case highlights the importance of an appropriate diagnostic algorithm, requiring non-invasive multimodality assessment imaging and then invasive cardiac biopsy. This approach may allow an early diagnosis and an adequate therapy for this otherwise fatal pathology.
... This is an especially pertinent technique for differentiating lymphomas specifically. 1,4 It is important to investigate all the differential diagnoses that occur in pericardial effusions, especially cardiac neoplasms. Literature cites an important number of tumors that can affect the heart or a part of it: hemangiosarcoma, aortic body tumors (chemodectoma and paraganglioma), ectopic thyroid carcinoma, thyroid adenoma, melanoma, mast cell tumor, blastoma, granular cell tumor, mesothelioma, myxoma, myxosarcoma, mesenchymoma, undifferentiated sarcoma of presumptive myofibroblastic origin, fibroma, fibrosarcoma, rhabdomyoma, rhabdomyosarcoma, leiomyoma, leiomyosarcoma, chondrosarcoma, osteosarcoma, paraganglioma, peripheral nerve sheath tumor, hamartoma, lipoma, valvular osteosarcoma, valvular myxosarcoma and valvular metastasis of disseminated tumors, and histiocytic sarcoma. 2 However, some clinical presentations may be more common than others; for example, hemangiosarcoma, the most common type of cardiac tumor, is usually mass forming, and aortic base tumors, such as chemodectoma, are generally considered nonfunctional, benign, and of low metastatic power. 2 Thus, the prognosis, treatment, and clinical presentation may vary, and the investigation of clinical, laboratory, or imaging alterations in other organs, although not always present, should be performed with parsimony. ...
... This is relevant because of the frequent lack of evident mass formation or identifiable masses. 4 Although cytological analysis must be performed when there is pericardial effusion formation, its effectiveness is questionable. Studies 2,3 have demonstrated that false negatives can represent up to 74% of cardiac tumor identification, being successful mainly when the fluid hct is below 10%. ...
... In most cases, the presenting symptoms are heart failure, arrhythmia and cardiac arrest [14]. The most common types of pathological primary cardiac lymphoma are DLBCL, followed by Burkitt's lymphoma, T cell lymphoma, small lymphocyte lymphoma and plasmablastic lymphoma [8,14], while the most common secondary cardiac lymphomas are DLBCL, T-lymphoblastic lymphoma and Hodgkin's lymphoma (HL) [12,15,16]. Cardiac involvement of CLL/SLL is observed extremely rarely [13][14][15][16]. ...
... The most common types of pathological primary cardiac lymphoma are DLBCL, followed by Burkitt's lymphoma, T cell lymphoma, small lymphocyte lymphoma and plasmablastic lymphoma [8,14], while the most common secondary cardiac lymphomas are DLBCL, T-lymphoblastic lymphoma and Hodgkin's lymphoma (HL) [12,15,16]. Cardiac involvement of CLL/SLL is observed extremely rarely [13][14][15][16]. In a retrospective analysis of 94 patients with heart infiltration of non-Hodgkin's lymphoma (NHL), Gordon et al. found CLL/SLL involvement in only six cases (7%) [13]. ...
... Zhao et al. identified 37 cases in a Chinese population with cardiac lymphoma [16]. The cardiac manifestations included chest tightness, shortness of breath, increased heart rates and electrocardiographic abnormality caused by pericardial effusion. ...
Cardiac involvement of chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) is recognized extremely rarely. In addition, most CLL/SLL patients with heart infiltration are asymptomatic. In this review, we present the results of a literature search for English language articles concerning CLL/SLL or Richter transformation with symptomatic cardiac involvement. In total, 18 well-described cases with CLL/SLL and heart infiltration were identified. Only three patients were not diagnosed with CLL/SLL before the cardiac manifestation. In other patients, cardiac CLL/SLL was diagnosed between 5 months and 20 years from CLL/SLL diagnosis. All patients in these series had a diagnosis of secondary cardiac CLL/SLL. In addition, we identified four reported cases with Richter transformation in the heart. The treatment of patients with CLL/SLL and cardiac infiltration is variable and depends on the previous history and clinical characteristics of heart infiltration. In addition, no recommendations exist on how to treat patients with CLL/SLL and cardiac involvement.
... It can show the location and size of a lesion and indicate whether hemodynamics are affected. In addition, TTE also exhibits high sensitivity and specificity in evaluating the extent of lesions, which is extremely valuable for a surgeon's preoperative preparation (5). Most cardiac tumors need to be surgically removed to halt disease progression and prevent serious complications. ...
... Primary cardiac lymphomas are rare lymphomas that usually occur in the right atrium but can also invade the right ventricle, pericardium, left atrium, or left ventricle (5). Some of these tumors have a pedicle and intact capsule, which grow within the heart cavity ( Figure 5K1,5K2). ...
Background:
Space-occupying cardiac lesions are uncommon but fatal. Echocardiography can identify diseases quickly in the clinic. This study reviews the clinical data of patients with space-occupying cardiac lesions in the past 10 years and analyzes their echocardiographic features, pathological diagnosis, and prognosis.
Methods:
We performed a retrospective analysis of 412 patients admitted to Affiliated Drum Tower Hospital of Nanjing University Medical School, Nanjing from 2011 to 2020. All patients were diagnosed with cardiac masses based on transthoracic echocardiography (TTE) and transesophageal echocardiography (TEE). We compared the diagnostic results of echocardiography and the postoperative pathological diagnosis and analyzed the characteristics of different types of space-occupying cardiac lesions. We also compared the mortality of patients with different types of space-occupying cardiac lesions through follow-up results of postoperative patients.
Results:
The 412 patients included 189 males and 223 females. Among them, 214 patients had benign tumors (including 176 patients with myxomas), 29 had primary malignant tumors, 32 had metastatic tumors, 41 had thrombi, 92 had infectious neoplasms, and 4 patients had special types of space-occupying lesions. A total of 376 lesions were correctly characterized by TTE, with an accuracy of 91.3%. Patients with benign tumors (9/214), thrombi (4/41), infectious neoplasms (5/92), or special types of space-occupying lesions (0/4) exhibited low rates of mortality or recurrence. In contrast, patients with primary malignant tumors (16/29) or metastatic tumors (16/32) exhibited high mortality rates.
Conclusions:
Echocardiography is a valuable tool for characterizing space-occupying cardiac lesions. It can provide important preoperative diagnostic information for cardiothoracic surgeons.
Cardiac lymphoma is rare in children. Treatment typically includes chemotherapy, combination of radiotherapy, or surgery. We report a case of stage IV precursor B lymphoblastic lymphoma with secondary involvement of the heart in an 11-year-old girl who was treated with acute lymphoblastic leukemia-based chemotherapy. Also, we review the literature on this uncommon malignancy.
