Cardiac Myxoma with Cerebral Metastases and Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma: A Case Report and Review

Abstract

Background Cardiac myxomas, the most common primary cardiac tumors, are generally benign neoplasms. Primary cardiac lymphoma is a rare cardiac malignancy with a very poor prognosis. Here we present a case of a cardiac myxoma with cerebral metastases and chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) arising within the cerebral metastases. Case description A 62-year-old man, who presented with symptoms of multiple transient ischemic attacks, was found to have a left atrial myxoma. Twelve months after excision of the myxoma, the patient experienced a recurrence of neurologic symptoms. Brain magnetic resonance imaging revealed multiple hemorrhagic masses. Craniotomy was performed to resect the lesions. Histopathologic examination confirmed cardiac myxoma metastases and a small lymphocytic infiltrate within the tumor consistent with CLL/SLL. Conclusion Including the present case, there are 27 cases of cardiac myxoma cerebral metastases and 22 cases of lymphomas arising within myxomas. The present case is the first known instance of both entities in the same patient. There is no standard management for either cardiac myxoma metastases or lymphoma within a myxoma. For both diseases, surgical excision is the primary treatment modality, but postoperative chemotherapy and/or radiation have been attempted. Myxomas may create a chronic inflammatory state that could lead to the development of CLL/SLL.

Cerebral embolization associated with parenchymal seeding of the left atrial myxoma: Potential role of interleukin‐6 and matrix metalloproteinases

“…Although the mechanisms of embolization of a benign tumor and metastasis of an invasive malignancy are fundamentally different, the appearance of multiple lesions at a distant site in the setting of a cardiac myxoma has occasionally been referred to (erroneously) in the literature as “metastasis.” 17,18 Since the first description made by Rankin et al ., in 1978, 19 our comprehensive review revealed 36 histologically established cases of intracerebral embolization associated with parenchymal brain invasion from a benign left atrial myxoma (Table 1). 10,14,18–51 A careful review of the cases confirmed that three patients have been reported twice, 14,19,20,23,24,28 six patients had unavailable histological proof, 37,52–56 and four apparent primary cardiac sarcomas were erroneously reported as a cardiac myxoma 57–60 . In one case, the original report could not be located, 61 and another report described embolic myxoma cells in the leptomeninges without evidence of parenchymal seeding 62 …”

“…63 Thus, parietal and occipital lobes are the most affected regions (91.6%). Moreover, the bone 19,35,46 and the skin 23,50 can be simultaneously involved. The most common clinical presentation of these patients was seizures (37.1%), followed by headache (31.4%), and hemiparesis (20%).…”

“…The most common clinical presentation of these patients was seizures (37.1%), followed by headache (31.4%), and hemiparesis (20%). However, unusual presentations such as gait disorders 37,42,50 and visual disturbances 25,27,43 have also been reported. In addition, the high frequency of intracranial aneurysms (75%) in these patients suggests that invasion and proliferation of myxoma cells into the vessel wall might represent a previous step to brain parenchymal seeding.…”

Relapsed chronic lymphocytic leukemia with a cardiac mass: A case report

“…A review of case reports from the last 20 years conducted by Maas et al. [9] revealed that there have been 22 cases of cardiac lymphomas arising within cardiac myxomas. Another case reported by Park et al.…”

Multiple craniotomies for the resection of symptomatic multifocal intracranial metastatic cardiac myxoma: A case report

“…[ 8 ] Radiation therapy has also been used in patients with multiple metastases, usually whole-brain radiation at 25–30 Gy. [ 5 ] Postoperative chemotherapy with either ifosfamide and doxorubicin or doxorubicin alone has also been attempted in patients with multifocal disease, though with mixed results. [ 7 ] One case report even documents the use of Gamma Knife radiosurgery to treat cerebral metastases, without recurrence of disease 2 years postoperatively.…”