A rare presentation of left atrial tumor as acute stroke

Abstract

This case report deals with a case of stroke in young female patient who was later diagnosed to have left atrial tumor. This female patient in her late 20s presented with a history of 1 month of progressive postural giddiness (in upright position), which was followed by sudden onset right monoparesis. The patient arrived to the hospital with above mentioned complaints. Her National Institutes of Health Stroke Scale was 5, and on examination of cardiovascular system, she had a middiastolic murmur in upright position, which resolved in lying position. Her brain imaging noncontrast computerized tomography (NCCT), magnetic resonance imaging (MRI) revealed lacunar infarcts in multiple vascular territories. Her transthoracic echocardiography revealed a pedunculated mass of around 4 × 2 cm from inter atrial septum in left atrium. She was started on anticoagulation and was referred to a cardio thoracic, and vascular center for tumor excision and biopsy. She underwent successful tumor excision and histopathological examination of the resected mass revealed cardiac myxoma. Incidence of primary cardiac tumor is very less in realtime world data. An atrial myxoma presenting as acute stroke secondary to tumor embolization is extremely rare.

Introduction

Primary cardiac tumors are extremely rare, and their incidence is estimated to be about 0.02% of total cases on autopsy as per European Society of Cardiology. Cardiac tumors have the potential to cause obstruction of intracardiac flow, arrhythmias, and embolization of tumor fragments.¹ The most feared complication of cardiac tumors is systemic embolization, and the risk is as high as 30%–40% in patients with myxomas; however, the risk of embolization is not limited to myxomas. Papillary fibroelastomas and other tumor types have been reported to have high rates of embolization as well.^{2, 3} Among all cardiac tumors, atrial myxoma is the most common benign cardiac tumor that is detected often in younger adults with stroke or transient ischemic attack (1 in 250) than in older patients (1 in 750).⁴ The etiology of many of cardiac tumors is unknown and can be part of carney complex, which has an autosomal dominant inheritance.⁵ The treatment of these cardiac tumors is primarily tumor excision and other symptomatic treatment and anticoagulation in case of tumor embolization. Cardiac myxomas are the most common primary tumors and are largely associated with embolic events. Cardiac myxomas typically arise from the interatrial septum at the border of the fossa ovalis in the left atrium, and other location is considered atypical. Embolism, one of the complications of myxoma, is associated with high morbidity and mortality.⁷ More than 80% of cardiac myxomas arising from the left atrium embolize (cerebral and/or systemic). Fifty percentage of cardiac myxoma patients present with symptoms related to mitral valve obstruction (dyspnea, dizziness, palpitations, and congestive heart failure) and can occur in up to 70% of them during the natural course of the illness. Neurological symptoms have been documented in about 26%–45% of cardiac myxomas. Cerebral embolization may present commonly as ischemic infarcts and rarely with myxomatous intracranial aneurysms, brain parenchymal myxomatous metastases, as well as intracerebral hemorrhage. Surgical resection is the definitive treatment option for cardiac myxoma, as medical management even with anticoagulant therapy has been proven ineffective to prevent recurrent embolic events.⁸ In this case report, we present a case of left atrial myxoma presented as a case of acute ischemic stroke secondary to embolization.

Case report

A young female patient in her late 20s with no prior comorbidities presented to emergency with complaints of sudden onset weakness in her right upper limb, and she was apparently asymptomatic 1 month prior to onset of sudden weakness of right upper limb where she developed giddiness on sudden change of posture (from sitting/supine to standing upright). The symptom of giddiness slowly progressed in terms of duration, initially lasted for around 5 min which progressed to around 15 min, and these symptoms were associated with unquantified weight loss and intermittent low-grade fever. Following these symptoms, she decided to review herself in hospital; on the day of onset of weakness, she woke up at around 6 a.m. and noticed that she was not able to hold a glass of water with right hand and had difficulty in reaching overhead objects with right upper limb; however, she was able to feel sensations and differentiate hot and cold objects on affected limb. She denied any history of involuntary movements, dyspnea, palpitations, and chest pain. Her obstetric history was uneventful with two children of ages 6 years and 4 years, respectively, who were delivered at hospital by normal vaginal delivery. Her menstrual history included normal menstrual cycles and denied history of usage of oral contraceptive pills. She was immediately rushed to the hospital; on clinical evaluation, she had decreased power (3/5) and exaggerated deep tendon reflexes in right upper limb along with right side extensor plantar response. Her cardiovascular system examination revealed middiastolic murmur in mitral area in upright position, which disappeared in supine position. Her imaging studies (noncontrast computerized tomography (NCCT) and magnetic resonance imaging [MRI] brain) revealed multifocal acute infarcts (Fig. 1). In view of suspicion of cardioembolic stroke, transthoracic echocardiography was performed, which revealed a pedunculated mass of size 4 × 2 cm moving freely attached to inter atrial septum in left atrium (Fig. 2); however, her left ventricular function was normal. The mass visualized on echocardiography was resembling atrial myxoma, which is the most common benign primary cardiac tumor, but histopathological examination is required to confirm the diagnosis.⁶ The presence of stalked mass hanging from interatrial septum in left atrium rules out the possibility of left atrial thrombus. She was started on low molecular weight heparin and was referred to cardio thoracic center for resection of tumor as definitive management. Patient consent for inclusion in study was also obtained.

Fig. 1.

Magnetic resonance imaging (MRI) brain indicating multiple cerebral infarcts.

Fig. 2.

Left atrial mass of around 4 × 2 cm attached to inter atrial septum.

Discussion

We present this case to increase awareness and to stress at early evaluation of secondary causes of ischemic cerebrovascular incident (CVA), outside the realm of hypercoagulability. This patient was admitted as a case of stroke in young on basis of history, clinical examination, and preliminary investigations. Her basic investigations such as complete blood counts, renal function, and liver function tests were essentially normal except for increase in erythrocyte sedimentation rate (ESR) indicating inflammatory response that is a known fact in cardiac tumors such as atrial myxoma due to increase in interleukin-6 activity (IL-6).⁶ However, to rule out other causes of stroke in young, she underwent workup for vasculitis, atherosclerosis, 24-h Holter, arterial blood gas analysis for obstructive sleep apnea, and whole-body positron emission tomography CT scan to rule out any secondary localization, either metastases or any neuroendocrine tumors. We hereby stress on the importance of history and clinical examination in this case; on initial evaluation, the patient had cardiac murmur (mid-diastolic murmur in mitral area) in upright position, which disappeared in supine position. However, characteristic finding of tumor plop was not appreciated, with postural intensified middiastolic murmur in mitral area, there was a strong suspicion of left atrial mass immediately after primary examination of cardiovascular system. The diagnosis of left atrial tumor was confirmed by transthoracic echocardiography, which revealed a pedunculated mass hanging from interatrial septum, which changes its position in left atrial chamber subjective to postural changes. However, MRI brain was performed in view of right upper limb weakness, which showed multiple lacunar infarcts in different vascular territories, signifying likely multiple embolic showers. Tumor embolization of primary cardiac tumors is a well-known fact; following clinical examination and emergency investigations, the diagnosis of primary cardiac tumor leading to acute stroke was made and needed to be confirmed after ruling out differentials of vasculitis, systemic lupus erythematosus, and malignancy. After ruling out other causes, it was confirmed that acute stroke was a result of tumor embolization secondary to primary cardiac tumor.

Conclusion

The patient successfully underwent tumor excision surgery (Fig. 3), and specimen of the same was sent for histopathological evaluation. Histopathological evaluation showed sheets of myxomatous cells encircling tumor vessels (Fig. 4), which was consistent with diagnosis of myxoma. She was discharged and was advised 1 month of rehabilitation/convalescence period. We hereby conclude by advising that in evaluation of secondary causes of multiple cerebral infarcts in a young individual, left atrial tumors should be considered in the differential diagnosis.

Learning points:

• 1.This case report is unique, with cardiac tumor presenting as acute stroke.
• 2.Clinical examination and history continue to be vital part in diagnosis even after development of sophisticated investigations.
• 3.Stroke in young female, apart from coagulopathy cardiac tumors and septal defects of heart, should be included in part of evaluation work up.

Fig. 3.

Gross morphology of resected specimen.

Fig. 4.

Histopathological examination of resected mass showing sheets of myxomatous cells surrounding tumor vessels.

Patients/ Guardians/ Participants consent

Patients informed consent was obtained.

Ethical clearance

Not Applicable.

Source of support

Nil.

Disclosure of competing interest

The authors have none to declare.