Cardiac involvement in disseminated diffuse large B-cell lymphoma, successful management with chemotherapy dose reduction guided by cardiac imaging: A case report and review of literature

Table 1 Reported cases of cardiac lymphoma treated with reduced chemotherapy doses

Ref.	Cardiac involvement	Pathology	Chemotherapy dose	Additional therapy	Cardiac imaging	Follow up
Beckwith et al[29], 2000	PCL; Right atrial mass.	DLBCL	Chemotherapy initiated 2 wk after surgery with 50% CHOP chemotherapy with continuous cardiac monitoring in the intensive care unit; After 2 cycles of CHOP, TEE showed tumor resolution. Two additional cycles of CHOP were given (patient received 90% of planned doses due to cytopenias and gastrointestinal toxicity)	Prophylactic placement of bovine pericardial patch over the involved atrial free wall; cranial irradiation and intrathecal methotrexate	TEE	After 2 mo, intracerebral tumor was detected, treated by cranial irradiation then with intrathecal methotrexate for lymphomatous meningitis. The patient died from the CNS disease
Dawson et al[3], 2006	PCL; Extensive intramyocardial mass involving the right atrium, right ventricular free wall and encircling the pulmonary artery trunk	DLBCL CD20- and CD79a-positive	Dose-dense schedule of R-CHOP every 14 d. 50% reduction of cyclophosphamide and Adriamycin doses in the initial course; Chemotherapy completed with a median of 16 d between cycles	Growth factor support with pegylated granulocyte colony-stimulating factor was administered 24 h following the completion of chemotherapy	TEE, CT, FDG-PET, MRI. MRI tagged short axis slices used to assess the potential for cardiac rupture with chemotherapy	Survived and in remission at 11 mo
Shah et al[31], 2014	PCL; Large right atrial mass resulting in severe tricuspid stenosis and invading the interatrial septum and encasing the aortic root	DLBCL, CD19 and CD20 positive, CD5 and CD10 negative	Rituximab 375 mg/m2 and prednisone 40 mg daily for 10 d; Low dose R-CHOP (cyclophosphamide 400 mg/m2, doxorubicin 25 mg/m2, vincristine 1 mg, prednisone 60 mg and rituximab 375 mg/m2) 2 cycles, administered every 3 wk; Full dose R-CHOP, every 3 wk for 4 cycles		TTE, TEE, PET, follow up TTE at 3 wk demonstrated no change in tumor size however there was no evidence of ventricular septal perforation	Well and in remission at 12 mo
Cereda et al[32], 2017 (report of 3 patients)	PCL; Lymphoma localized to the right chambers in all patients	DLBCL in all PCL	Pretreatment with steroids and vincristine; 6 cycles of R-COMP (Myocet not pegylated liposomal doxorubicin, rituximab, vincristine, and prednisone)		TTE, FDG-PET/CT scan TTE with tissue Doppler-derived strain and 2D-strain imaging of the RV showed progressive improvement of RV function Following complete resolution by TTE, a second FDG-PET/CT scan confirmed remission	2 patients were in complete remission at 25 mo. 1patient had extra-cardiac relapse and underwent a salvage therapy and autologous transplantation; 2 patients developed late cardiac toxicity during post-remission surveillance
Almehisen et al. 2019 (present case)	SCL; Large mass invading the left atrium, interatrial septum, right atrium Infiltration of lateral LV wall. Partial occlusion of the left upper pulmonary vein and occlusion of the remaining pulmonary veins. Compression of right pulmonary artery and SVC	DLBCL. Positive for CD20, CD45, CD19, CD79A, CD10, and BCL-6, and negative for CD5	The first cycle of CHOP was divided into 3 parts administered 4-7 d apart. The first part: 50% doxorubicin, 25% cyclophosphamide, and full-dose prednisolone. The second part: 50% vincristine, 25% cyclophosphamide, and full-dose prednisolone. The third part: 50% doxorubicin, 25% vincristine, 50% cyclophosphamide, and full-dose prednisolone; Chemotherapy consisted of etoposide augmentation in the R-CHOP protocol for the remaining cycles	Six doses of intrathecal chemotherapy; Bone marrow transplant	TTE, Strain, CT, and MRI; MRI demonstrated tumor infiltration of the atrial wall, the infiltrative left ventricular lesion and lymphoma resolution by fibrosis, after which chemotherapy was escalated to full dose	Complete remission with normal LV function at 3 yr

CT: Computed tomography; MRI: Magnetic resonance imaging; TEE: Transesophageal echocardiogram; TTE: Transthoracic echocardiogram; SVC: Superior vena cava; PCL: Primary cardiac lymphoma; DLBCL: Diffuse large B-cell lymphoma; CHOP: Cyclophosphamide/doxorubicin/vincristine/prednisolone; PET: Positron-emission tomography; FDG: [18F]-fluorodeoxyglucose.