Cardiac pheochromocytoma

Journal o/ Internal Medicine 1994; 236: 93-96

CASE R E P O R T

Cardiac pheochromocytoma A. JONSSON, B. HALLENGREN, P. MANHEM, B. LILJA,* L. STAVENOW,t E. STAH],$ & c. T O R N Q U I S T ~ Frorti the Department of Endocrinology. *Clinical Physiology. and §Radiology. University of Lund. General Hospital. Malrno: the jGustav Adolj Medical Centre. MalrnB: and the $Department of Thoracic Surgery, University of Lund, Lund Hospital. Lund; Sweden

Abstract. Jonsson A, Hallengren B, Manhem P, Lilja B, Stavenow L, StHhl E, Tornquist C (Departments of Endocrinology, Clinical Physiology, and Radiology, University of Lund, General Hospital, Malmo ; the Gustav Adolf Medical Centre, Malmo; and the Department of Thoracic Surgery, University of Lund, Lund Hospital, Lund: Sweden). Cardiac pheochromocytoma (Case report). J Intern Med 1994: 236: 93-96.

may be difficult to localize. We present a 32-year-old male with a cardiac pheochromocytoma that was successfully resected. An initial unenhanced CT did not reveal the tumour. MIBG-scintigraphy indicated the location, but to get full information, a dynamic contrast-enhanced CT of the chest during adequate alpha and beta blockade was essential. ECG-gated MRI gave further information about the anatomical details.

Cardiac pheochromocytoma is a rare tumour and

Keywords : cardiac pheochromocytoma, localization.

Introduction Pheochromocytorna is a relatively rare tumour. Most of the tumours are located in the abdomen, predominantly in the adrenal glands, whereas less than 2% are located in the chest [ 11. Localization of these lesions can be difficult [2, 31, but newer diagnostic techniques have facilitated this. We present a patient with an intracardiac pheochromocytoma that was successfully resected.

Case report The patient, a male born in 1960, was admitted to hospital in the autumn of 1990 because of serous meningitis. He was found to be hypertensive and the blood pressure was difficult to control despite varying combinations of antihypertensive drugs. He complained of sweating attacks, especially in the supine position, and severe headache, but he had no dyspnoea or palpitations. These symptoms grew worse in autumn 1 9 9 1 and in January 1992 markedly elevated total 24-h urinary noradrenalin levels [4] of 10 14 0 nmol (reference range 3 0 4 4 0 ) , were found. The patient was admitted to the

endocrinology department of Malmo General Hospital for further evaluation with a strong suspicion of pheochromocytoma. The patient was still hypertensive but physical examination including ophthalmoscopy was otherwise normal and no heart murmurs were heard. Twenty-four-hour blood pressure monitoring demonstrated a mean of 183/ 126 mmHg without diurnal rhythm. Total 24-h urinary catecholamine levels [4] showed very high noradrenalin values of 8630-24 720 nmol, whereas adrenalin values were within the reference range. The plasma noradrenalin concentration [ 51 was constantly markedly elevated [36.9-78.0 nmol L-' (reference range 0.7-4.0)], and plasma adrenalin concentration [5] was elevated [1.2-2.4 nmol L-' (reference range 0.1-0.5)]. The plasma chromogranin A + B concentration [ G ] was elevated [1500 mg L-l ( < 350)j. These laboratory findings confirmed the diagnosis of pheochromocytoma. In order to localize the tumour, a CT of the abdomen and the chest was performed but no tumour was found. These studies were performed without intravenous contrast medium enhancement as the patient had just started alpha adrenergic blockade with phenoxybenzamine and beta adrenergic block93

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Fig. 1 Computed tomography (CT) scan of the heart during intravenous contrast medium infusion demonstrating a mass lesion

(arrow) measuring 4 x 3 cm in the right atrium.

ade with propranolol and was still insufficiently blocked. MIBG-scintigraphy revealed a small area of increased uptake in the right part of mediastinum. A new CT of the chest during intravenous contrast medium infusion revealed a mass located in the right atrium (Fig. 1).Magnetic resonance imaging of the heart confirmed a high signal tumour in the right atrium (Fig. 2a & b). Two-dimensional echocardiography demonstrated that the tumour did not affect the bloodflow or the valves. The patient underwent midline sternotomy and a tumour measuring 4 x 5 cm was found in the right atrium. The tumour was radically resected during cardiopulmonary bypass. Histopathological examination demonstrated a pheochromocytoma without signs of malignancy. Selective blood sampling for catecholamine concentration were performed intraoperatively before and 10 min after resection of the tumour. The highest value of noradrenalin was noted in the right atrium, followed by the right ventricle and superior vena cava. After the resection the values were considerably lower (Table 1). Postoperatively, total 24-h urinary noradrenalin levels and plasma chromogranin A + B concentration were normalized. The patient’s blood pressure returned to normal levels without medication and all symptoms disappeared.

Discussion The described patient had a pheochromocytoma located in the right atrium of the heart. A cardiac

location of this type of tumour has previously been reported in about 2 0 cases [7]. The diagnosis of a pheochromocytoma was suspected from the clinical picture and was evident from the biochemical findings. However, it was difficult to localize the tumour and clinical signs or symptoms gave no hint of the cardiac location. It is well known that intravascular contrast media may elevate plasma catecholamines [8] and angiography [9] ,venography [ l o ] as well as contrast-enhanced CT [ l l ] can bring about hypertensive crisis in patients with pheochromocytoma. Thus, in our patient (before being sufficiently alpha and beta blocked) a n unenhanced CT was performed as the initial localizing procedure [12]. This did not demonstrate the tumour, probably because of lack of contrast difference between the tumour and the heart on CT and motion artifacts in the heart during scanning. MIBG-scintigraphy, having the advantage of including the whole body, although with low sensitivity [13-151, indicated the location in the chest but did not provide more precise information. Following adequate alpha and beta blockade a dynamic CT of the chest with intravenous contrast medium infusion demonstrated a mass lesion in the right atrium of the heart (Fig. 1).An ECG-gated MRI of the heart (no contrast medium) also demonstrated the turnour and provided anatomical details because of the high contrast between the high-signal tumour and the loss of signal from rapidly flowing blood adjacent to the tumour (Fig. 2a & b). Twodimensional echocardiography, usually regarded as noncontributional in patients with cardiac pheo-

CARDIAC PHEOCHROMOCYTOMA

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Fig. 2 Magnetic resonance imaging of the heart (ECG-gated axial and coronal T1-weighted images) demonstrating a high signal tumour (arrows) in the dorsolateral part of the right atrium.

Table 1 Plasma catecholamine concentrations intraoperatively before and 10 min after resection of a cardiac pheochromocytoma in a 32-year-old man Noradrenalin (nmol L-')

Adrenalin (nmol K 1 )

Location

Before

After

Before

After

Superior vena cava Right atrium Right ventricle

108.2 375.5 76.5

7 9.5

8.63 9.05 4.32

4.95 5.83 5.82

75.2 16.8

chromocytoma [16], in this case provided information about the haemodynamic conditions, apart from the cardiac location. Intraoperative blood sampling revealed the highest 7

value of plasma noradrenalin in the right atrium, much higher than in patients undergoing cardiac surgery (arterial samples) [ 171 and in healthy volunteers during exercise (samples from coronary sinus) [18], probably because of the tumour drainage into the coronary sinus. The plasma catecholamine levels decreased rapidly after resection [19] but were not normalized, probably because of slow release of stored catecholamines from sympathetic nerve granulae. Computed tomography without intravenous contrast medium enhancement has been recommended as the initial localizing procedure in patients with suspected pheochromocytoma [ 121. Following this policy, in this case a cardiac pheochromocytoma was not demonstrated, but MIBG-scintigraphy indicated the location. We suggest that CT performed with I M B 236

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optimal techniques, including intravenous contrast medium enhancement, is used in a diagnostic search for extra-adrenal pheochromocytomas and the studies should be performed after adequate alpha and beta blockade. Magnetic resonance imaging may give some additional information regarding anatomic details in these patients.

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Received 4 October 1993, accepted 22 December 1993. Correspondence: Anders Jonsson MD. Department of Endocrinology, University of Lund. Malmo General Hospital, S-214 01 Malmo. Sweden.