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An unusual tumour involving heart and liver
An Unusual Tbmour Involving Heart And Liver Anthony S. Walton, FRACP, Aubrey Pitt, FRACP, Bruce B. Davis,** FRACS, Julian A. Smith,** FRACS, Brian W. Essex,* FRCPA, Michael Nicholson,# FRACP Department of Cardiology, Alfred Hospital Department of Anatomical Pathology*, Alfred Hospital C J Officer-Brown Cardiothoracic Surgery Unit**, Alfred Hospital Commercial Road, Prahran, Victoria, Australia The Royal Hobart Hospital#, Hobart, Tasmania, Australia
W
e report the successful removal of a large right atriai haemangioma associated with multiple liver haemangiomas. Preoperative echocardiography showed a large right atria1 mass, and computerised tomography revealed lesions in the liver. A preoperative diagnosis of malignant cardiac tumour and liver secondaries was made. However, the tumour was benign. This case emphasises the need to obtain a definitive histologic diagnosis preoperativeiy, if possible, before rejecting a patient for surgery, and to be aware that benign lesions can be multifocai. (AustraiAs J Cardiac Thorac Surg 1993;2(1):36-38)
Introduction Primary cardiac tumours are rare. The reported incidence at autopsy varies from 0.0017% to 0.028% 1. About 75% are benign, with myxoma being the most common type*. Cardiac haemangiomas have previously been reported, 3.4-j although they are rare, being 3.5% of benign cardiac tumours 1. There have been no previous reports of this condition associated with lesions in the liver, although liver haemangiomas have been linked with cardiac angiosarcomas 6.
Transthoracic doppler echocardiography revealed a 9 cm nonmobile mass occupying most of the right atrium. It had a mottled appearance with several large echo-free spaces and appeared to be attached to the septum and posterior right atria1 wall without a pedicle.
Case Report A previously well, 47-year-old man presented with increasing shortness of breath for 2 weeks. This had been associated with atypical central chest pain, palpitations and dizziness. At the time of presentation, he was found to be in atria1 flutter which was treated with intravenous digoxin and spontaneously reverted to sinus rhythm. The physical findings were unremarkable. On repeated examination, there was no abnormality of the jugular venous pressure, nor were any murmurs were present. The resting electrocardiogram was normal. Chest xray examination revealed cardiomegaly with an enlarged right atrium. Full blood examination, erythrocyte sedimentation rate, urea and electrolyte examination, and liver function tests were normal.
At the time of surgery, transoesophageal echocardiography was performed and confirmed the preoperative findings. The chest was opened by midline sternotomy, and cardiopulmonary bypass was instituted. Venous drainage was achieved by direct cannulation of the superior vena cava and the femoral vein.
Computerised tomographic (CT) scanning of the chest and abdomen confirmed the extensive right atria1 mass (Fig. 1) and revealed multiple liver lesions (Fig. 2). Two attempts were made prior to surgery to biopsy the liver lesions to exclude metastatic malignancy, but no definitive abnormality was detected.
The right atrium was found to be grossly enlarged. The tumour was broadly adherent to the whole of the upper portion of the right atrium including the roof. In resecting the tumour, it was necessary to remove part of the atria1 roof. The atrium was repaired and closed by direct suture. The liver was exposed, and multiple lesions were found, one of which was excised. The patient made an uneventful postoperative recovery. After a follow-up of 12 months he remained well.
36
AustralAs
J Cardiac
Thorac
Surg 1993:2(1):36
- 38 Heart
Fig. 1. CT scan of the chest showing
Fig. 2. CT scan of the abdomen
the right atria1 mass (arrow).
Pathology
showed
multiple
Walton and liver turnour
liver lesions
(arrows).
rare, representing only 3.5% of benign tumours of the heart 1. They have been found at autopsy in persons from birth to 78 years of age, who had been asymptomatic.
The atria1 mass was 100 mm in maximum diameter (Fig.3) and consisted of a sponge-like network of bloodcontaining spaces lined by an attenuated endothelium with fragments of adherent thrombus. The external wall was made up of a relatively acellular fibrous layer about 3 mm thick.
The diagnosis is often made difficult by the wide range of symptoms and signs that cardiac tumours can manifest, masquerading as other more common cardiovascular and systemic diseases.
The hepatic nodule was 12 mm in diameter and of the same histology as the atria1 tumour. In both situations there were no histological features of malignancy. The diagnosis was of vascular malformations involving the atria1 wall and liver (Fig.4).
Haemangiomas are most often found in the right heart chambers and are generally sessile or polypoid subendocardial nodules ranging from 2-3.5 cm in diameter, although they may be larger. Clinically they are often silent but may result in disturbances of conduction or rhythm, and may cause syncope or sudden death 5. Intramural tumours may produce symptoms by virtue of their size and location.
Discussion Primary cardiac tumours are much less common than cardiac metastases 9. Primary cardiac haemangiomas are
Features of tricuspid stenosis or regurgitation may be present. Right ventricular haemangiomas have been reported to cause right ventricular outflow-tract obstruction 4. Histologically they consist of endotheliallined spaces which may contain blood, lymph or thrombi. They are classified according to the predominant type of proliferating vascular channel 1. The natural history of these tumours is uncertain. Grenadier and colleagues reported an unresectable lesion in a 12-year-old girl which showed no significant change over 4 years 7. Most benign cardiac tumours are curable at operation. Therefore, diagnosis is critical. Advances in noninvasive cardiovascular diagnosis (in particular, echocardiography) has facilitated early diagnosis.
Fig. 3. Macroscopic maximum
diameter
appearance of the excised was 100 mm.
right atria1 turnour.
If the tumour is resectable, the prognosis is usually good, though recurrence is possible. Therefore, diligent follow-up is required. Cardiac transplantation has been used successfully to treat primary cardiac tumours 8.
The
37
AustralAs
.I Cardiac
Thorac
Surg 1993:2(1):36
- 38 Heart
Fig. 4. Microscopic
appearance
of the right atria1 tumour.
Haematoxylin
Walton and liver tumour
and eosin stain at x200 magnification
In our case, there was some reluctance to proceed to surgery in a man with potentially metastatic malignancy. This situation emphasises the need to obtain a definitive histologic diagnosis preoperatively, if possible, before rejecting a patient for surgery, and to be aware that benign lesions can be multifocal.
Deep x-ray therapy has also been used successfully for an inoperable haemangioma 4. Reports of multifocal haemangiomas involving the heart and other organs are rare. Burke et al 5 reported a series of 10 cardiac haemangiomas where one patient had concomitant lesions of the heart, oral mucosa and bowel.
References 1.
Braunwald E. Heart Disease. A textbook medicine. 3rd ed. Philadelphia: WB Saunders,
2.
Heath
D. Pathology
of cardiac
turnours.
of cardiovascular 1988:1470.
Am J Cardiol
5.
1968;21:315.
3.
Tabry IF, Nassar VH, Rizk G, et al. Cavernous haemangioma the heart. J Thorac Cardiovasc Surg 1975; 69:415-20.
4.
Soberman MS, Plauth, WH, Winn right ventricle causing outflow Cardiovasc Surg 1988;96:307-9.
of
KJ, et al. Haemangioma on the tract obstruction. J Thorac
Burke A, Johns J, Virmani R. Haemangiomas Cardiovasc Path01 1990;3:283-90.
Am J
6.
Smith C. Tumours
7.
Grenadier E, Margulis T, Palant A, et al. Huge cavernous haemangioma of the heart: a completely evaluated case report and review of the literature. Am Heart J 1989;117:479-81.
8.
Aravot DJ, Banner NR, Madden B et al. Primary cardiac tumours is there a place for cardiac transplantation. Eur J Cardiothorac Surg 1989;3:521-4. -
38
of the heart.
of the heart. Arch Path01 Lab Med 1986; 110: 1.
W
e report the successful removal of a large right atriai haemangioma associated with multiple liver haemangiomas. Preoperative echocardiography showed a large right atria1 mass, and computerised tomography revealed lesions in the liver. A preoperative diagnosis of malignant cardiac tumour and liver secondaries was made. However, the tumour was benign. This case emphasises the need to obtain a definitive histologic diagnosis preoperativeiy, if possible, before rejecting a patient for surgery, and to be aware that benign lesions can be multifocai. (AustraiAs J Cardiac Thorac Surg 1993;2(1):36-38)
Introduction Primary cardiac tumours are rare. The reported incidence at autopsy varies from 0.0017% to 0.028% 1. About 75% are benign, with myxoma being the most common type*. Cardiac haemangiomas have previously been reported, 3.4-j although they are rare, being 3.5% of benign cardiac tumours 1. There have been no previous reports of this condition associated with lesions in the liver, although liver haemangiomas have been linked with cardiac angiosarcomas 6.
Transthoracic doppler echocardiography revealed a 9 cm nonmobile mass occupying most of the right atrium. It had a mottled appearance with several large echo-free spaces and appeared to be attached to the septum and posterior right atria1 wall without a pedicle.
Case Report A previously well, 47-year-old man presented with increasing shortness of breath for 2 weeks. This had been associated with atypical central chest pain, palpitations and dizziness. At the time of presentation, he was found to be in atria1 flutter which was treated with intravenous digoxin and spontaneously reverted to sinus rhythm. The physical findings were unremarkable. On repeated examination, there was no abnormality of the jugular venous pressure, nor were any murmurs were present. The resting electrocardiogram was normal. Chest xray examination revealed cardiomegaly with an enlarged right atrium. Full blood examination, erythrocyte sedimentation rate, urea and electrolyte examination, and liver function tests were normal.
At the time of surgery, transoesophageal echocardiography was performed and confirmed the preoperative findings. The chest was opened by midline sternotomy, and cardiopulmonary bypass was instituted. Venous drainage was achieved by direct cannulation of the superior vena cava and the femoral vein.
Computerised tomographic (CT) scanning of the chest and abdomen confirmed the extensive right atria1 mass (Fig. 1) and revealed multiple liver lesions (Fig. 2). Two attempts were made prior to surgery to biopsy the liver lesions to exclude metastatic malignancy, but no definitive abnormality was detected.
The right atrium was found to be grossly enlarged. The tumour was broadly adherent to the whole of the upper portion of the right atrium including the roof. In resecting the tumour, it was necessary to remove part of the atria1 roof. The atrium was repaired and closed by direct suture. The liver was exposed, and multiple lesions were found, one of which was excised. The patient made an uneventful postoperative recovery. After a follow-up of 12 months he remained well.
36
AustralAs
J Cardiac
Thorac
Surg 1993:2(1):36
- 38 Heart
Fig. 1. CT scan of the chest showing
Fig. 2. CT scan of the abdomen
the right atria1 mass (arrow).
Pathology
showed
multiple
Walton and liver turnour
liver lesions
(arrows).
rare, representing only 3.5% of benign tumours of the heart 1. They have been found at autopsy in persons from birth to 78 years of age, who had been asymptomatic.
The atria1 mass was 100 mm in maximum diameter (Fig.3) and consisted of a sponge-like network of bloodcontaining spaces lined by an attenuated endothelium with fragments of adherent thrombus. The external wall was made up of a relatively acellular fibrous layer about 3 mm thick.
The diagnosis is often made difficult by the wide range of symptoms and signs that cardiac tumours can manifest, masquerading as other more common cardiovascular and systemic diseases.
The hepatic nodule was 12 mm in diameter and of the same histology as the atria1 tumour. In both situations there were no histological features of malignancy. The diagnosis was of vascular malformations involving the atria1 wall and liver (Fig.4).
Haemangiomas are most often found in the right heart chambers and are generally sessile or polypoid subendocardial nodules ranging from 2-3.5 cm in diameter, although they may be larger. Clinically they are often silent but may result in disturbances of conduction or rhythm, and may cause syncope or sudden death 5. Intramural tumours may produce symptoms by virtue of their size and location.
Discussion Primary cardiac tumours are much less common than cardiac metastases 9. Primary cardiac haemangiomas are
Features of tricuspid stenosis or regurgitation may be present. Right ventricular haemangiomas have been reported to cause right ventricular outflow-tract obstruction 4. Histologically they consist of endotheliallined spaces which may contain blood, lymph or thrombi. They are classified according to the predominant type of proliferating vascular channel 1. The natural history of these tumours is uncertain. Grenadier and colleagues reported an unresectable lesion in a 12-year-old girl which showed no significant change over 4 years 7. Most benign cardiac tumours are curable at operation. Therefore, diagnosis is critical. Advances in noninvasive cardiovascular diagnosis (in particular, echocardiography) has facilitated early diagnosis.
Fig. 3. Macroscopic maximum
diameter
appearance of the excised was 100 mm.
right atria1 turnour.
If the tumour is resectable, the prognosis is usually good, though recurrence is possible. Therefore, diligent follow-up is required. Cardiac transplantation has been used successfully to treat primary cardiac tumours 8.
The
37
AustralAs
.I Cardiac
Thorac
Surg 1993:2(1):36
- 38 Heart
Fig. 4. Microscopic
appearance
of the right atria1 tumour.
Haematoxylin
Walton and liver tumour
and eosin stain at x200 magnification
In our case, there was some reluctance to proceed to surgery in a man with potentially metastatic malignancy. This situation emphasises the need to obtain a definitive histologic diagnosis preoperatively, if possible, before rejecting a patient for surgery, and to be aware that benign lesions can be multifocal.
Deep x-ray therapy has also been used successfully for an inoperable haemangioma 4. Reports of multifocal haemangiomas involving the heart and other organs are rare. Burke et al 5 reported a series of 10 cardiac haemangiomas where one patient had concomitant lesions of the heart, oral mucosa and bowel.
References 1.
Braunwald E. Heart Disease. A textbook medicine. 3rd ed. Philadelphia: WB Saunders,
2.
Heath
D. Pathology
of cardiac
turnours.
of cardiovascular 1988:1470.
Am J Cardiol
5.
1968;21:315.
3.
Tabry IF, Nassar VH, Rizk G, et al. Cavernous haemangioma the heart. J Thorac Cardiovasc Surg 1975; 69:415-20.
4.
Soberman MS, Plauth, WH, Winn right ventricle causing outflow Cardiovasc Surg 1988;96:307-9.
of
KJ, et al. Haemangioma on the tract obstruction. J Thorac
Burke A, Johns J, Virmani R. Haemangiomas Cardiovasc Path01 1990;3:283-90.
Am J
6.
Smith C. Tumours
7.
Grenadier E, Margulis T, Palant A, et al. Huge cavernous haemangioma of the heart: a completely evaluated case report and review of the literature. Am Heart J 1989;117:479-81.
8.
Aravot DJ, Banner NR, Madden B et al. Primary cardiac tumours is there a place for cardiac transplantation. Eur J Cardiothorac Surg 1989;3:521-4. -
38
of the heart.
of the heart. Arch Path01 Lab Med 1986; 110: 1.