Cavernous hemangioma of the heart Case report and review of the literature

Cavernous hemangioma of the heart Case report and review of the literature

Cavernous hemangioma of the heart Case report and review of the literature A case of diffuse cavernous hemangioma of the heart discovered incidentally...

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Cavernous hemangioma of the heart Case report and review of the literature A case of diffuse cavernous hemangioma of the heart discovered incidentally during life is reported. Resection of the tumor was impossible at operation. Two similar cases were found in the literature. Radiation therapy is suggested, and the importance of a simple blood pool scan in the investigation of paracardiac masses is stressed.

Imad F. Tabry, M.D., Victor H. Nassar, M.D., Ghassan Rizk, M.D., Amin Touma, M.D., and Ibrahim K. Dagher, M.D., Beirut, Lebanon

Xrimary tumors of the heart are uncommon and, among them, hemangiomas are exceptionally unusual.1010 Until recently, hemangiomas of the heart were considered to be autopsy curiosities. With the advent of angiocardiography and open-heart surgery, a few such lesions have been recognized during life and managed surgically. The purpose of this paper is to present one such case and to review the pertinent literature. Case report N. G. (No. 378745), a 57-year-old Lebanese housewife, was admitted on Feb. 8, 1974, to the American University Medical Center for the investigation of a mass along the right border of the heart. The lesion had been discovered for the first time on a chest roentgenogram done 2 months earlier. She gave a history of recurrent palpitations for the previous 20 years. Three months prior to admission, she had an episode of paroxysmal atrial fibrillation documented on an electrocardiographic tracing. A small left thyroid nodule was present but no evidence of hyperthyroidism was found. On admission, the neck veins were flat, and there was a mobile, nontender nodule measuring 2 cm. in diameter in the lower pole of the left lobe of the thyroid. The From the Departments of Surgery, Cardiology, Pathology, and Radiology, School of Medicine, American University Hospital, Beirut, Lebanon. Received for publication July 3, 1974. Address for reprints: Dr. Victor H. Nassar, Department of Pathology, American University Hospital, Beirut, Lebanon.

patient looked otherwise healthy, and results of her examination were essentially normal except for frequent ectopic beats. Blood pressure was 140/80 mm. Hg, and laboratory data were within normal limits. The electrocardiogram revealed atrial ectopic beats but was otherwise normal. A cardiac series showed a soft tissue, nonpulsatile mass contiguous with the right heart border (Fig. 1). A forward angiogram disclosed compression of the right atrial border with elevation of the right main pulmonary artery. The right ventricle and other cardiac chambers were not involved (Fig. 2). The general impression was that of a pericardial cyst causing the recurrent episodes of paroxysmal atrial fibrillation and atrial ectopic activity. Exploratory thoracotomy was performed. On opening the pericardium, we found a large mass adherent to the right lateral pericardium with vascular adhesions. The spongy mass bulged anteriorly to compress the right atrium and the superior vena cava. It involved the right side of the left atrial wall and extended posteriorly to involve the right pulmonary veins, medially the origin of the aorta, and inferiorly the left atrioventricular groove. The ventricles were pushed forward and to the left. Because of its extension, the mass was considered unresectable and a biopsy was taken. Bleeding was controlled by sutures and pressure. A left thyroid lobectomy was performed during the same operation. Histologically, the heart tumor was composed of cavernous channels embedded in a loose fibrous stroma. The appearance was distinctive for cavernous hemangioma (Fig. 3). The left thyroid lobe contained a simple colloid adenoma. The postoperative course was smooth and uneventful. A postoperative blood pool scan

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Fig. 1. Posteroanterior (A) and lateral (B) chest films. There is a water density mass contiguous to the right cardiac border and located anteriorly.

Fig. 2. A, Forward angiogram. The tissue mass is seen compressing the right atrial border and elevating the right main pulmonary artery. The right ventricle is normal. B, Levophase of the angiogram. The left cardiac chambers are normal. No abnormal vascularity is noted in the region of the mass. showed a considerable amount of radioactivity within the cardiac tumor (Fig. 4). The patient was discharged after a week on digoxin 25 mg. daily, Eraldin 100 mg. twice daily, and Proloid 2 grains daily for life. She was started on a course of radiation therapy to the heart. Comment Hemangiomas of the heart are a rarity. In 1951 there were six verified hemangiomas

of the heart 11 ; at present, only 35 case reports can be found in the world literature. Until recently, these tumors were usually autopsy findings, with an incidence of 0.0017 per cent in 480.331 autopsies.10 Anatomically, the location of hemangiomas of the heart is ubiquitous; the lesions may occur in the myocardium, endocardium, or pericardium. In the pericardium

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Fig. 3. The tumor is composed of cavernous channels embedded in a loose fibrous stroma. The appearance is diagnostic of cavernous hemangioma.

they usually involve the epicardial layer 8 ' 1R ; very seldom do they occur in the parietal layer.17 Intramural hemangiomas were found in the interventricular septum1'1 and the anterior wall of the ventricles11 and atria (as in our case). Subendocardial hemangiomas have been described in all the cardiac chambers, but most occur on the right side of the heart: the right ventricle," right atrium,- and left atrium.11 Most reported hemangiomas have been small, subendocardial, bluish nodules varying between 2 mm. and 3.5 cm. in diameter. They are sessile or polypoid, usually single but occasionally multiple, and lack infiltrative features. They are seldom extensive and poorly demarcated, as in our case and those presented by Contamin/' Franciosi,7 and their associates. Although usually solitary, cardiac hemangiomas at times have been associated with other angiomas, mainly cutaneous or visceral, a condition which constitutes diffuse angiomatosis.4 Histologically, vascular tumors of the heart are classified according to the predominant type of the proliferating vascular channel. The benign hemangiomas, excluding hemangiomatosis, are therefore of the following subtypes: benign hemangioendotheliomatous, capillary or juvenile, cavernous, venous, and racemose (cirsoid) heman-

Fig. 4. A blood pool scan (with technetiumalbumin) shows radioactivity in the mass, indicating the presence of blood within it.

giomas. Racemose hemangiomas resemble a malformation composed of tortuous, thickwalled blood vessels of venous and/or arterial types. The term hamartoma is best preserved for those lesions that, in addition, feature a conspicuous component of other mesenchymal tissues inherent to the locale, such as cardiac musculature, pericardial serosa, and so on, in the case of the heart. The symptoms depend on the location of the tumor. When situated at the right side of the interventricular septum, the hemangioma may simulate infundibular pulmonary stenosis.°' " A right atrial location on the rim of the foramen ovale15 or interatrial septum usually produces no symptoms, but the lesion may mimic atrial myxoma with

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Table I. Patients operated upon for hemangiomas of the heart Age of Author (date)

patient (yr.)

Hochberg and Robinson8 (1950)

Sex

Clinical

presentation

Cineangiography

F

Dyspnea on exertion; bulge along right side of heart on chest x-ray

Not done

Nicks" (1964)

20

F

Dyspnea on exertion; findings of infundibular pulmonary stenosis

Done (right)

May et al." (1965)

10

M

"Chest cold"; bulge along right side of heart on chest x-ray

Done (right)

F

Dyspnea on exertion; right heart failure

Done (right and left)

Franciosi et al.7 (1970) Warembourg et al.18 (1971)

44

F

Asymptomatic; left mediastinal mass on chest x-ray; one year later, dyspnea on exertion and increase in size of the mass

Done (right)

Delaye et al." (1972)

23

M

Right heart failure; findings of infundibular pulmonary stenosis

Done (right)

Contamin et al/' (1973)

20

F

Done (right)

Present case (1974)

57

F

Acute pericarditis plus cardiomegaly at age 10; pericardiectomy at age 18; large tumor of LV at operation; right heart failure at age 20 Palpitations for 20 yr.; paroxysmal atrial fibrillation for 2 yr.; cold thyroid nodule; bulge along right side of heart on chest x-ray

Done (right)

Legend: LV, Left ventricle. RV, Right ventricle. RA, Right atrium. MPA, Main pulmonary artery. LA, Left atrium. RPV, Right

associated tumor emboli or may obstruct the superior vena cava. Intramural growths have been responsible for atrioventricular block15 and compression of the coronary vessels simulating a myocardial infarct.14 In some instances, tumors developing in the pericardium have produced pericardial effusion,''' hemopericardium,3 and even lethal tamponade. 9 Extrinsic compression of the main pulmonary artery or its branches (as in our case) has also been described.18 In case of extensive involvement, the patient may present with congestive heart failure, usually on the right,5- ~ due to restricted filling of the right cardiac chambers.

Prior to the last two decades, it was difficult to demonstrate conclusively the presence of a neoplasm of the heart by the available diagnostic methods, chest roentgenography in particular. Recent advances in cardiac catheterization and angiography made the antemortem diagnosis possible. In only one report," however, was a hemangioma suspected on cineangiography; this showed a mobile tumor in the outflow tract of the right ventricle that partially occluded the pulmonary valve during systole. Among other invasive techniques, coronary angiography can be helpful in demonstrating the vascular nature of a cardiac tumor. Other

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erative diagnosis ftricardial cyst

Infundibular pulmonary stenosis Pericardia! cyst

Location tumor

and extent of involvement

Size

(cm.)

Epicardium (anterior 9 by 6 by wall of R V ) ; peri3.5 cardium, pleura, thymus Myocardium (anterior wall of RV plus endocavitary extension) Myocardium (anterior 6.5 wall of RA)

Pericarditis and Myocardium of apex effusion

Extensive

Epicardium at junction of MPA and infundibulum of RV; pedunculated tumor Subendocardium (RV 4.5 by 2.5 outflow); pedunculated tumor Pericarditis and Myocardium (wall of Extensive RV, RA, LV, perieffusion cardium) Mediastinal tumor (rule out thymoma) Tumor of RV outflow tract

Pericardial cyst

Myocardium (wall of LA, interatrial septum, origin of aorta, RPV)

Extensive

Treatment

Pathology

report

Thoracotomy; total excision of Cavernous hemangioma the tumor (shaved off the myocardium) Total excision under TCPB

Vascular hamartoma

Lateral thoracotomy with closure; 2 mo. later, total excision including partial resection of wall of RA under TCPB Biopsy of unresectable tumor of the heart

Vascular hamartoma

Left anterolateral thoracotomy; total excision of the tumor

Infiltrative diffuse capillary and cavernous hemangioma Cavernous and capillary hemangioma

Total excision under hypothermia and TCPB

Capillary hemangioma

At age 20, biopsy of unresectable tumor

Infiltrative capillary hemangioma

Right thoracotomy; biopsy of unresectable tumor of the heart; left thyroid lobectomy; postoperative radiation therapy

Cavernous hemangioma

pulmonary veins. TCPB, Total cardiopulmonary bypass.

noninvasive techniques such as echocardiography or a simple blood pool scan can be helpful in the preoperative diagnosis of a cardiac hemangioma, as well illustrated postoperatively in our case. Such a differential diagnosis is important, since surgery for a hemangioma of the heart requires standby equipment for extracorporeal circulation. Hemangiomas of the heart, even when recognized preoperatively, are not always amenable to surgery. A total of eight such tumors were recognized at surgery, including that in the present case. However, successful resection could be performed only

in the well-circumscribed, small hemangiomas.6, s ' 12' "■ 1 S In 3 patients, including ours, tumor infiltration was too extensive to allow total excision.5' ~ Table I summarizes the course of the patients with vascular tumors of the heart who were operated upon. The outcome of hemangiomas of the heart is unpredictable. Sudden death may result from any of the previously described complications. The hemangioma may involute, stop growing, or proliferate indefinitely while remaining benign and "silent" until the patient dies from other causes. The 5 patients who had total excision of

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the tumor regained normal activity, although one developed a posterior myocardial infarct when the arterial feeder to a right atrial tumor was ligated along its anterior aspect.12 The prognosis for unresectable hemangiomas must be poor. Although supravoltage irradiation therapy was never attempted in these cases, it has been used for a primary malignant hemangioendothelioma of the right atrium 1: The response was merely palliative and, at the time of death from a thalamic metastasis, there was no tumor in the myocardium. We are presently administering conventional x-ray therapy to our patient with the hope that diffuse angiomas of the heart are as radiosensitive as hepatic hemangiomas.

REFERENCES 1 Allaire, F. L., Grimm, G. A., Taylor, L. M., and Pfaff, J. P.: Primary Hemangioendothelioma of the Heart: Report of a Case Treated With Irradiation and Cyclophosphamide, Rocky Mt. Med. J. 6 1 : 34, 1964. 2 Baroldi, G., Colombo, F., and Manion, W. C : Benign Primary Hemangioma of the Right Atrium of the Heart: Report of a Case, Med. Ann. D. C. 36: 287, 1967. 3 Bencini, B.: Angiomi e pseudo-angiomi peric a r d i a come rara causa di emopericardio, Cuore Circ. 20: 645, 1936. 4 Berlov, G. A., and Pigarev, Y. U. G.: Unusual Systemic Tumor-Like Angiomatosis (Malignant Hemangioendothelioma of the Pericardium, Angiomatous Polyp of the Endocardium and Progressive Hemangiomatosis of the Liver), Arch. Pathol. 26: 74, 1964. 5 Contamin, C. H., Denis, B., Mallion, J. M., Martin-Noel, P., Latreille, R., and Barrie, J.:

6

7

8

9

10 11 12

13

A propos d'un hemolymphangiome du coeur, Ann. Chir. Thorac. Cardiovasc. 12: 275, 1973. Delaye, J., Crassard, P., Segneuric, A., Lenglet, J. P., Dufoix, V., Marion, P., and Gonin, A.: Les tumeurs primitives du ventricule droit. A propos d'un cas d'hemangioendotheliome benin opere avec succes, J. Med. Lyon 53: 483, 1972. Franciosi, R. A., Gay, R. M., and Ah Tye, P.: Vascular Hamartoma of the Heart in a Child, Am. Heart J. 79: 676, 1970. Hochberg, L., and Robinson, A.: Primary Tumor of the Pericardium Involving the Myocardium: Surgical Removal, Circulation 1: 805, 1950. Lefas: Mort subite par rupture d'angiome pericardique, Bull. Soc. Anat. (Paris) 73: 464, 1898. Mahaim, I. Les tumeurs et les polypes du coeur, Paris, 1945, Masson & Cie. Manifold, R. F.: Cavernous Angioma in the Wall of the Left Auricle, Lancet 2: 1027, 1915. May, I. A., Hardy, K. L., Char, F., Adams, M. C , Jr.: Vascular Hamartoma of the Right Atrium With Successful Resection, Ann. Thorac. Surg. 1: 64, 1965. Nicks, R.: Hamartoma of the Right Ventricle. J. THORAC.

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14 Pambuccian, G. R., and Bercovici, S.: Angiom al inimii cu tablou de infant miocardic, Med. Interna (Bucur.) 20: 225, 1968. 15 Prichard, R. W.: Tumors of the Heart, Arch. Pathol. 5 1 : 98, 1951. 16 Strauss, R., and Merlis, R.: Primary Tumors of the Heart, Arch. Pathol. 39: 74, 1945. 17 Timme, A. R.: Cavernous Hemangioma of the Pericardium, Cleve. Med. J. 14: 453, 1915. 18 Warembourg, H., Niquet, G., Ribet, M., Lekieffre, J., Thery, C , Faillon, J. M., Gosselin, B., and Ketelers, J. Y.: Hemangiome du coeur, Presse Med. 79: 2073, 1971. 19 Willis, R. A.: Pathology of Tumors, ed. 3, Washington, D. C. 1960, Butterworth & Co., Ltd.