Primary cardiac sarcoma complicated with cerebral infarction and brain metastasis: A case report and literature review

Abstract

In large autopsy series, the estimated frequency of primary tumors of the heart ranges from 0.0017% to 0.33%. Approximately 25% of primary cardiac tumors are malignant, and nearly 20% of these are sarcomas. To date, a completely feasible surgical resection remains the major treatment measure of cardiac sarcoma, especially for recurrent focal cardiac sarcoma and the recurrence of a restrictive metastasis. Although characteristically medical treatments are recommended, there is no consistent opinion for adjuvant radiotherapy and chemotherapy following an operation. Since these tumors usually undergo extensive spread by the time that the diagnosis is established, the prognosis of cardiac sarcoma remains poor. In this report, we described a case who underwent initial cardiac tumor resection, and was confirmed to be a pleomorphic undifferentiated sarcoma based on pathological findings. However, the patient complicated with cerebral infarction and subsequent brain metastasis sarcoma after the initial surgery, which was confirmed by brain tissue pathology. During the course of therapy, the patient underwent three surgical operations and refused to accept any chemotherapy and radiotherapy intervention. To the best of our knowledge, this is the first case report describing a primary cardiac sarcoma complicated with cerebral infarction and brain metastasis. The management of primary cardiac sarcoma is also discussed.

1. Introduction

Primary cardiac malignancies account for one fourth of all cardiac tumors. Primary cardiac sarcomas may arise from mesenchymal stem cells with the ability to generate tumors with multilineage differentiation [1]. These can give rise to brain metastasis and cerebral vascular complications such as infarcts, hemorrhages and intracranial aneurysms [2]. Primary cardiac sarcomas represent rare malignant neoplasms that follow varying clinical courses. The site of the metastases show predilection for the lung, liver and mediastinum, but the brain seems to be an uncommon site of metastasis [3]. Due to the low incidence, there is no standard treatment method.

2. Case report

A 33-year-old male was admitted to the hospital in January 2016 with a three month history of chest distress and shortness of breath. The patient’s physical examination on admission was notable for a heart rate of 80 bpm, a blood pressure of 110/67 mmHg, a body weight of 72 kg, a body mass index of 24, and the absence of cardiac murmurs on auscultation. Electrocardiogram revealed a normal sinus rhythm. The patient underwent transthoracic echocardiogram, which demonstrated normal cardiac chambers and an ejection fraction (EF) of 61%. However, a mass of approximately 40 $\times$ 69 mm was evident in the left atrium, and attached to the central region of the atrial septum in the echocardiographic four-chamber view (Fig. 1A) and left ventricle long axis view (Fig. 1B). The patient was diagnosed with the left atrium mass.

Figure 1.

Echocardiogram Imaging. (A) Four-chamber view and (B) Left ventricle long axis view showing the cardiac mass in the basal region of the left atrium, and attached to the atrial septum.

The patient underwent an uncomplicated surgical excision of the cardiac left atrial mass two days after admission. The operation went smoothly except for the incomplete tumor resection, and then the resected mass was sent to pathology for further evaluation. On the first day after the cardiac mass excision, the patient was weaned off mechanical ventilation smoothly. However, the patient had drowsiness, somnolence, and barylalia, and left-side defective limb with the muscle strength of grade 2. These features alerted us to anticipate a high possibility of neurological complication. A craniocerebral multi-row computed tomography (CT) examination was immediately performed on the patient. In this process, the patient’s symptoms continued to deteriorate, which presented as lethargic conditions and with incomplete motor aphasia. Craniocerebral CT revealed that a large low density shadow located in the right hemisphere (approximately 16–23 HU) and the right brain sulcus and gyrus were not clear (post-operative [PO] day 1, Fig. 2). Therefore, based on imaging findings, the patient was diagnosed with right cerebral hemisphere infarction.

Figure 2.

Craniocerebral multi-row CT Imaging. (A) The preoperative cerebral anatomy was normal. (B) One day after the cardiac mass section, CT showing a big flake low density shadow in the right hemisphere and the sulci and gyri of right hemiencephalon were not clear. (C) Fourteen days after the cardiac mass section, CT showing the improvement of low density shadow. (D) Four months after the cardiac mass section and the decompressive craniectomy operation, CT showed a large area of low density shadow by the right lateral ventricle with compression, midline shift and apparent cephalocele. The right side of the brain sulcus and gyrus were not evident. (E) Six months after the cardiac mass section (two months after the right temporoparietal occipital lesion section), CT showed a small area of low density shadowing near the right lateral ventricle without obvious compression and midline shift. CT, computed tomography; PO, postoperation.

On the second day after the cardiac mass excision, the patient underwent an uncomplicated decompressive craniectomy, which was performed by neurosurgical team, in order to prevent cerebral hernia. Following a series treatment of dehydration and nerve nutrition, the patient recovered gradually, but had a sequela that the left-side limb was defective and the muscle strength was grade 3. Furthermore, craniocerebral CT results slightly improved (PO day 14, Fig. 2). The final cardiac pathological diagnosis was undifferentiated pleomorphic sarcoma (Fig. 3A and B). However, the patient refused to accept any postoperative radiotherapy or chemotherapy, and was discharged from hospital in February 2016.

Figure 3.

Cardiac and cerebral mass pathology. (A) The cardiac mass in the cardiac left atrium with a volume of 60 $\times$ 50 $\times$ 40 mm and a slim papilla appearance on the surface. (B) HE staining of the cardiac mass slice showing necrosis, pleomorphic and undifferentiated sarcoma cells with scant myocardial cells ($\times$ 10). (C) Three hazel cerebral mass with visible necrosis and mucus. (D) The cerebral mass slice HE staining showed that a high grade, pleomorphic, and undifferentiated soft tissue sarcoma accompanying with necrosis cells ($\times$ 10). HE; Hematoxylin and eosin.

Four months after the cardiac mass section and the decompressive craniectomy operation, the patient presented with encephalocele of the right temporo-parieto-occipital region and was re-admitted in May 2016. Physical examination revealed a skull defect of the right temporoparietal lobe and left-side defective limb with the muscle strength of grade 3. Craniocerebral CT confirmed a large area of low-density lesions by the right lateral ventricle, and separated regions existed in the lesions. The brain tissue was also compressed and the midline shifted to the left, which led to an unclear brain sulcus and gyrus of the right side (PO month4, Fig. 2). The right temporo-parietal occupying lesions raised intracranial pressure, which forced us to perform a resection of the right temporo-parietal occupying lesion. The specimen obtained from the incisional biopsy was a brown, bilobed tumor, which was identified as malignant high differentiation soft tissue sarcoma with metastatic origin (Figs 3C and D).

Two months after resection of the right temporo-parietal occupying lesions, the patient presented with metastasis in his body. First, in July 2016, a mass measuring approximately 60 $\times$ 40 mm was identified on the right hip bone. Eight days later, a mass size of approximately 50 $\times$ 60 mm manifested at the back of the patient’s left leg. At the same time, it was noted that the patient’s right external auditory canal was filled with dried blood. After blood clearing, the otorhinolaryngologist found ongoing errhysis from the right external auditory canal with an intact tympanic membrane. The patient was treated with expansive sponges containing adrenaline and ofloxacin, which were changed daily until the errhysis decreasing and scar occurring (Fig. 4). Re-examination by CT imaging revealed a slight recurrence of right-sided cerebral sarcoma, as we have suspected (PO month 6, Fig. 2).

Figure 4.

Six months after the cardiac mass section and two months after the right temporoparietal occipital lesion resection, the blood crusts were found occupying the patient’s right external auditory canal.

From beginning to end, the patient refused to accept any palliative radiotherapy or chemotherapy, except for the necessary surgery. The patient was still alive approximately one year after definite diagnosis of cardiac sarcoma, but he was suffering from global aphasia and left-side limb paralysis.

3. Discussion

Primary cardiac tumors are rare diseases. In large autopsy series, the estimated frequency of primary tumors of the heart range from 0.0017% to 0.33% [4]. Approximately 25% of primary cardiac tumors are malignant, and nearly 20% of these are sarcomas [5]. In our case, a 33-year-old male was confirmed with undifferentiated pleomorphic sarcoma based on the cardiac mass section and histopathology, which was a rare case of primary cardiac sarcoma. Cardiac sarcomas are more likely to metastasize hematogenously to the lung or the liver, and cerebral metastasis from malignant cardiac tumor is an extremely rare phenomenon [6]. Our case was a primary cardiac atrial pleomorphic undifferentiated sarcoma with a confirmed pathological diagnosis. The patient’s clinical course was complicated in succession with right hemisphere cerebral infarction and right temporo-parietal metastatic sarcoma. Therefore, this was a case of primary cardiac pleomorphic undifferentiated sarcoma complicated with intricate neurological complications. The patient also probably suffered from metastasis to the right hip bone and back of the left leg seven months after the cardiac mass resection. We also found active hemorrhage of the right external auditory canal two months after cerebral metastasis of the sarcoma section, which might have been caused by the recurrence of cerebral metastases.

At present, feasible, completely surgical resection remains the main measure of cardiac sarcoma therapy, especially for recurrent local cardiac sarcoma and the recurrence of limited metastatic lesions, which may lead to good survival outcomes [7]. Unfortunately, only a small proportion of the tumor could be carried out. Partial resection of primary malignant cardiac tumors is advantageous, because the surgery can contribute to alleviate obstructive symptoms and improve the living condition of patients, but often has a low survival rate [8]. A handful of evidences have proven that patients can benefit from cardiac transplantation, since even low-grade sarcomas are likely to have early systemic metastasis [9].

The role of chemotherapy and radiation therapy for sarcoma management remains controversial. Postoperative radiotherapy following complete resection has the potential to decrease the local recurrence rate, but there is no consensus opinion for the fatal radiation-induced cardiomyopathy and pericarditis [10]. Unlike the outcomes for radiotherapy, chemotherapy shows no significant beneficial effect on survival for cardiac sarcomas, and can be used only for cytoreduction before surgery or the palliation of unresectable diseases. Our patient underwent three surgical interventions including cardiac mass section, decompressive craniectomy, and right temporoparietal occupying lesion excision. The patient was alive at the time this report was authored, which was approximately one year after his initial cardiac mass section surgery. Since the patient refused further radiotherapy and chemotherapy treatment, he developed a recurrent disease, and is currently suffering from global aphasia and left-side limb paralysis. This case enlightened us that if radiotherapy and/or chemotherapy was actively and timely performed, the patient might postpone the tumor recurrence, prolong overall survival, and even improve the quality of life.

4. Conclusions

In this report, we described the first case of rare primary sarcoma complicated with cerebral infarction and brain metastases after initial cardiac mass section surgery. From the review of literature, we conclude that although surgical intervention was necessary for resection of the tumor, it is important to identify malignant tumors early, in order for to enable patients to undergo radiotherapy or chemotherapy as soon as possible.

Conflict of interest

The authors declare that they have no competing interests.