Primary cardiac lymphoma in immunocompetent patients: a report of three cases and review of the literature

Abstract

Primary cardiac lymphoma is an extranodal non-Hodgkin's lymphoma exclusively located in the heart and/or pericardium, extremely rare in immunocompetent patients, and more frequent in immunodepressed patients. We present 3 retrospectives cases of primary cardiac lymphoma in immunocompetent patients and review 35 cases reported in the literature. Two patients were adults and one was a child. Primary cardiac lymphoma presented with constitutional symptoms in two cases and superior vena caval syndrome in one case. Diagnosis of a tumor mass was made in all cases by transthoracic echocardiography. Primary cardiac lymphoma arose in the heart right chambers in two cases. Histological diagnoses, obtained after thoracotomy, were diffuse large B-cell lymphoma in two cases, and Burkitt's lymphoma in one case. All three cases received chemotherapy, combined with radiotherapy in one patient. Of our patients, 2 are alive and asymptomatic 12 months and 33 months after diagnosis. In conclusion, diagnosis of primary cardiac lymphoma is difficult due to non-specific clinical manifestations and should be considered in patients with a cardiac mass sometimes with pericardial effusion. It is confirmed using transthoracic echocardiography and magnetic resonance imaging and certified using cytology or open biopsy. The only effective treatment is chemotherapy, but prognosis remains poor.

Primary cardiac lymphoma: Utility of multimodality imaging in diagnosis and management

“…Chemotherapy, immuno-chemotherapy, stem cell transplant, radiation therapy and combined modality approaches have been used to treat PCL. 7,8,[11][12][13][14] Recent reviews have attempted to assess CR rates using these various approaches in PCL. 7,8 Traditionally, PCL is thought to have an extremely poor prognosis due to life-threatening complications, such as cardiac rupture, which can occur at diagnosis or during treatment due to the anatomical location of the tumor.…”

“…9,10 Left-sided PCLs have however been reported. 7 Several strategies for treating PCL are described in the medical literature. A significant fraction of patients are unfortunately too clinically unstable at presentation to undergo treatment of any kind.…”

Primary Cardiac Lymphoma in an Immunocompetent 71-Year-Old Man

“…To our knowledge, only 23 cases of PCL in immunocompetent patients have been reported to date (Table I). [13][14][15][16][17][18][19][20][21][22][23][24][25][26][27][28][29][30] According to these reports, no patient presented with noncardiac lymphomatous localization, but, as in our patient, it was impossible to absolutely exclude noncardiac involvement in the absence of autopsic investiga- Nine of these patients were treated with surgical excision followed by chemotherapy, and 5 of them survived at least 6 months after treatment. No evidence exists concerning the necessity of surgery as an adjunct to chemotherapy in PCL; moreover, in those 9 cases surgery was the final diagnostic step, along with excision as treatment.…”

Efficacy of Rituximab Monotherapy for an Elderly Hemodialysis Patient with Primary Cardiac Lymphoma

“…PCL is classified mainly as DLBCL (60%-81% of cases) and less frequently as immunoblastic, Burkitt type, or lymphoblastic lymphoma. In more than 90% of cases, lymphoma cells belong to the B-cell lineage (11). In the present case, since cardiac tumor biopsy via thoracotomy seemed risky in this elderly patient with concomitant morbidity, biopsy was not performed, and the diagnosis of B-cell lymphoma was made by examination of morphology, cell surface markers, and monoclonality of the cytologic specimen (12).…”