Childs Nerv Syst (2012) 28:475–479 DOI 10.1007/s00381-011-1631-6
CASE REPORT
Atrial fibrillation as an uncommon presentation in a large pleomorphic xanthoastrocytoma Sadettin Sezer & Ali Baykan & Ebru Yilmaz & Ekrem Unal & Sertaç Hanedan Onan & Ali Yikilmaz & Kazım Uzum & Mehmet Akif Ozdemir & Nazmi Narin
Received: 3 May 2011 / Accepted: 2 November 2011 / Published online: 16 November 2011 # Springer-Verlag 2011
Introduction Atrial fibrillation (AF) is a rare rhythm that presents in children. It is defined as a disorganized, rapid atrial activity, with atrial rates ranging from 350 to 600 bpm. Various cardiac diseases, including ischemic heart disease, valvular diseases, atrial septal defect closure, and cardiomyopathy, are associated with AF [1–3]. Other systemic diseases, such as pulmonary embolism, hyperthyroidism, and many infections and inflammatory diseases, have been related to AF [1–5]. Atrial fibrillation was reported to be associated with arachnoid cyst in a 79-year-old woman [5]; Kneissl et al.
S. Sezer : A. Baykan : S. Hanedan Onan : K. Uzum : N. Narin Division of Pediatric Cardiology, Department of Pediatrics, Faculty of Medicine, Erciyes University, Kayseri, Turkey E. Yilmaz Department of Pediatrics, Faculty of Medicine, Erciyes University, Kayseri, Turkey E. Unal (*) : M. A. Ozdemir Division of Pediatric Hematology and Oncology, Department of Pediatrics, Faculty of Medicine, Erciyes University, 38039, Talas, Kayseri, Turkey e-mail:
[email protected] E. Unal e-mail:
[email protected] A. Yikilmaz Division of Pediatric Radiology, Department of Radiology, Faculty of Medicine, Erciyes University, Kayseri, Turkey
[6] reported AF after the start of radiochemotherapy for a glioblastoma multiforme located at the right temporal lobe in a 49-year-old patient. Despite of these case reports, we did not encounter such a presentation at childhood in the English medical literature. In order to draw attention to this interesting association, we aimed to report a child with pleomorphic xanthoastrocytoma (PXA) who presented with AF.
Case report A 13-month-old boy was referred to our center with complaints of palpitation during the past 12 h. He had no history of cardiovascular, pulmonary, or respiratory disease. At the initial physical examination, his body weight was 11 kg (90 percentile), height 76 cm (90 percentile), head circumference 49 cm (>97percentile), body temperature 36°C, respiratory rate 30/min, and arterial blood pressure 110/ 60 mmHg, while the pulse examination revealed arrhythmia with 220 bpm, and the rest of the physical examination was unremarkable. ECG revealed AF, frequent ventricular extrasystoles, and nonsustained ventricular tachycardia (Fig. 1a). The patient was hospitalized, and verapamil, digoxin, and amiodarone were used, respectively. After a complete cardiac evaluation and being certain that left atrial thrombi was absent, cardioversion was applied, but the patient did not respond. A flutter pattern was defined as a specific P wave configuration on the standard ECG (Fig. 1b). Echocardiographic examination was normal in etiology (EF 73%, FS 35%). Ammonia, lactate, pyruvate, levels of amino acids in the urine and blood, thyroid function tests, anti-dsDNA, ANA, and echovirus, coxsackievirus, and enterovirus serology were found to be negative. The patient was thought to have idiopathic atrial fibrillation at infancy.
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Fig. 1 ECG shows atrial fibrillation, frequent ventricular extrasystoles, and nonsustained ventricular tachycardia (a). ECG shows atrial flutter (b)
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For ventricular rate control, digoxin and propranolol were started and acetylsalicylic acid was also administered; he was discharged from the hospital at the 15th day of hospitalization. The patient experienced a generalized tonic clonic seizure when he was 21 months old. On admission to the hospital, he complained of nausea and vomiting. Electroencephalography (EEG) showed paroxysmal discharges in the right frontotemporal region, supporting the presence of the organic lesion. A cranial MRI revealed a very large supratentorial tumor mass in the right hemisphere measuring 10×7×8 cm (Fig. 2a). The mass filled the parietal and occipital lobes and extended into the frontal and temporal lobes. The internal structure of the mass was striking because it contained numerous cysts throughout. There was mass effect and peritumoral edema with partial invasion of the ipsilateral lateral ventricle. There was a tiny nodular mass in the temporal horn of the right lateral ventricle with a diameter of 5 mm, which was probably a drop metastasis (Fig. 2b). The tumor was subtotally resected with a parietal approach. On histopathological review, the tumor was diagnosed as PXA. Six months after the operation, follow-up MRI showed partial resolution of the main mass while the nodule in the temporal horn enlarged (Fig. 3). Atrial fibrillation disappeared and antiarrhythmic drugs were withdrawn. Twelve months after the diagnosis, the patient presented with another attack of refractory atrial fibrillation with findings of progression on cranial MRI (Fig. 4). The tumor was subtotally resected, and the histopathologic examination revealed anaplastic features showing malignant degeneration. As in the previous clinical course, atrial fibrillation did not respond to medications but the surgical
Fig. 2 Initial cranial MRI shows a massive supratentorial mass measuring 10×7×8 cm in the right hemisphere on axial T2-weighted (a) and gadolinium-enhanced T1-weighted (b) images at the level of lateral ventricles. The mass is prominently enhancing and containing diffuse cystic areas. There is peritumoral edema and mass effect with
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Fig. 3 On the follow-up MRI 6 months after surgery, axial T1weighted gadolinium-enhanced images at the level of lateral ventricles (a) and temporal horns (b) demonstrate that the main tumor mass still persists with a slight decrease in size; however, the metastatic nodule in the temporal horn has markedly enlarged (arrow)
control of the mass had diminished the arrhythmia. After 3 months, the patient suffered from palpitation and seizure. ECG showed AF at the time of recurrence. Tumor was evaluated as unresectable and the patient received 180 cGy for 25 days for a total of 4,500 cGy cranial radiotherapy. Radiotherapy also diminished AF. After radiotherapy, his family denied any medical or surgical treatment.
Discussion Brain tumors are the most common group of solid malignant tumors in childhood, accounting for 20% of all
the invasion of the lateral ventricle. There is a small nodule with a diameter of 5 mm in the right temporal horn, which is most likely a drop metastasis (arrow) on gadolinium-enhanced T1-weighted axial image
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Fig. 4 On the 18th-month follow-up MRI, axial T1-weighted gadolinium-enhanced images at the level of lateral ventricles (a) and temporal horns (b) demonstrate that the main mass is diminished in size with a large postoperative cavity in the right hemisphere; however, there are residual masses around the cavity (asterisk). The nodular mass in the temporal horn has enlarged (arrow)
childhood malignancies [6]. Symptoms and signs are related to the location, size, and growth rate of the tumor, and the symptoms of brain tumor are headache, vomiting, disturbances of gait and balance, impaired vision, mental disturbances, seizures, endocrine abnormalities, and diencephalic syndrome [6]. PXA is a rare astrocytic tumor occurring primarily in children and young adults. The superficial location of the tumor facilitates gross total resection, thus conferring a relatively favorable outcome [7]. As demonstrated by other glial tumors [8], malignant transformation of PXA has been described in the literature [7, 9, 10]. AF is a supraventricular tachyarrhythmia characterized by disorganized atrial electrical activity and progressive deterioration of atrial electromechanical function. Electrocardiographic manifestations of atrial fibrillation include absence of sinus P waves; rapid oscillations (or fibrillatory [f] waves) that vary in amplitude, frequency, and shape; and an irregular ventricular response. The prevalence of atrial fibrillation increases dramatically with age [1, 2]. Atrial fibrillation is rare in children. While patients can be asymptomatic, many experience a wide variety of symptoms, including palpitations, dyspnea, fatigue, dizziness, angina, and decompensated heart failure. In addition, AF can be associated with hemodynamic dysfunction, tachycardiainduced cardiomyopathy, and systemic thromboembolism. The causes of AF can be divided into cardiovascular versus noncardiovascular causes [1, 2]. In the central nervous system, signals from the cerebral cortex and hypothalamus can affect the activity of almost all of the autonomic centers of the lower brain stem, which, among other functions, control the heart rhythm. The
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presence of a specific stimulus or lesion would secondarily cause a direct, reciprocal, increased sympathetic and decreased parasympathetic relation in the ipsilateral hemisphere [5]. Chronotropic cardiac organization has been demonstrated in the posterior insular cortex of the rat. In humans, stimulation of the right insula would increase sympathetic activity, and stimulation of the left insula would decrease it [11]. Stimulation of the posterior and lateral hypothalamus increases blood pressure and heart rate, whereas in the anterior preoptic area of the hypothalamus, in which parasympathetic tone predominates, the opposite occurs [5, 11]. Although EEG did not show any stimulation of the insular cortex in the presented patient, it can be underestimated or affected by the effect of the organic lesion. The authors speculated that more sensitive EEG with subdural grid electrodes may be useful for the identification of the insular cortex for the workup of similar cases. Furthermore, the compression of the pressor center at the rostral ventrolateral medulla results in stimulation of the sympathetic nervous system, leading to hypertension and tachycardia. There are several reports on the association between posterior fossa tumors and hypertension and/or tachycardia [12]. In the presented case, the tumor did not originate from the posterior fossa, but the increased intracranial pressure may cause compression at the level of the dorsocaudal medulla. Similarly, Kneissl et al. [6] reported AF after the start of radiochemotherapy for a glioblastoma multiforme located at the right temporal lobe in a 49-year-old patient. On the other hand, the authors encountered some studies reporting the different effects of the left and right hemispheric located seizures and primary brain tumors on heart rate, including somatization symptoms [13, 14]. Right hemispheric seizure onset has been found to be associated with a higher incidence of tachycardia compared to seizures arising from the left hemisphere [15]. Human stimulation studies have shown that perioperative left insular stimulation is associated with bradycardia, and right insular stimulation is associated with tachycardias [16]. Mainio et al. [14] reported that the specific symptoms were dizziness and cardiopulmonary symptoms among patients with a right hemispheric tumor. In support of this hypothesis, the reported brain tumors associated with AF were also located on the right hemisphere [5, 6]. Even though the definite mechanism in the presented patient is unknown, it can be speculated that brain tumor such a PXA should be considered as a triggering or modulating factor of AF. Although the AF association of intracranial tumors has not been fully understood, this case highlights that physicians should be aware of this possibility.
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