Two cases of central nervous system (CNS) relapse in patients with primary cardiac lymphoma underline the importance of CNS evaluation in these patients, according to researchers.
Cardiac lymphoma is a rare condition, and doctors often overlook the potential of metastasis to the CNS, said study author Niccolò Frungillo, MD, of the European Institute of Oncology in Milan, Italy.
“In my opinion, it is very important to identify prognostic factors that predict the brain relapse of lymphoma,” he said. “It’s a rare—but often fatal—complication.”
In ecancermedicalscience, Dr Frungillo and his colleagues described the diagnosis and treatment of two women with cardiac lymphoma who achieved remission and later presented with isolated CNS relapse.
Doctors diagnosed the patients via endomyocardial biopsy, and results were consistent with diffuse large B-cell lymphoma in both cases. Immunochemotherapy produced complete remissions (CRs) in both women.
The patients then experienced isolated CNS relapses, one 8 weeks after achieving a CR, and one 5 weeks after CR.
In the first patient, MRI and cerebrospinal fluid confirmed her relapse, after she presented with a 3-day history of headache and vomiting.
The patient then received high-dose methotrexate and high-dose cytarabine, which prompted a second CR. She went on to receive an autologous stem cell transplant but died before engraftment due to a pulmonary infection.
The second patient received systemic CNS prophylaxis with high-dose methotrexate prior to her relapse. Nevertheless, one week after she was declared lymphoma-free, she presented with headache and a deterioration of motor skills.
MRI and lumbar puncture confirmed her relapse, and she received induction chemotherapy with high-dose methotrexate. Her disease progressed after two courses of treatment, so she was placed on salvage therapy with cytarabine and high-dose methotrexate.
Whole-brain radiotherapy prompted a CR, and the patient went on to receive an autologous stem cell transplant.
The researchers noted that isolated CNS relapse is very uncommon in cardiac lymphoma, but CNS evaluation should be considered. Additional studies are needed to determine the appropriate management of CNS relapse in cardiac lymphoma.
