Ann Thorac Surg 2005;79:709-711
© 2005 The Society of Thoracic Surgeons
Case report
Emergency Management of Severe Right Ventricular Inflow Obstruction Secondary to a Metastatic Cardiac Tumor
Erdinç Naseri, MD*,a,
Bulent Eralp, MDb,
Ibrahim Oztek, MDc
a Department of Cardiovascular Surgery, Tokat Medical School, Tokat, Turkey
b Department of Internal Medicine, Goztepe SSK Hospital, Istanbul, Turkey
c Department of Pathology, GATA, Istanbul, Turkey
Accepted for publication August 28, 2003.
* Address reprint requests to Dr Naseri, Tokat T
p Fakultesi Hastanesi, Tokat, Turkey, 60100
enaseri{at}turk.net
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Abstract
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We report the case of a 21-year-old female with previous history of primitive neuroectodermal tumor of the anterior abdominal wall who developed severe manifestations of right heart failure due to a mass obliterating the right ventricular cavity. She underwent emergent resection of the mass with histopathologic confirmation of metastatic neuroectodermal tumor. To the best of our knowledge, this is the first case of emergent surgical excision of a metastatic cardiac tumor of primitive neuroectodermal origin.
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Introduction
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Cardiac tumors are uncommon and usually benign. Myxomas or other nonmyxomatous benign tumors are mainly treated surgically, with cure in most cases [1–3]. Metastatic cardiac tumors are much rarer and the necessity for surgical excision is extremely uncommon. Primitive neuroectodermal tumor (PNET) is a rare malignancy classified under the heading of round cell tumors. Other malignancies in this category are Ewing's sarcoma, lymphoma, rhabdomyosarcoma, and neuroblastoma. There have been several reports of these tumors giving rise to secondary cardiac seedings. To our knowledge, there is no report of excision of a metastatic PNET in the English literature [5, 6]. We present the case of a young female with previous PNET of the abdominal wall and severe right heart failure due to a mass obliterating the right ventricular cavity.
A 21-year-old female was admitted with severe manifestations of right heart failure. Two years ago, she had undergone the resection of a mass in the anterior abdominal wall. The histopathologic diagnosis at that time was PNET of the anterior abdominal wall. She underwent postoperative radiotherapy and chemotherapy, and her disease was thought to be under control. No information was available about the chemotherapeutic protocol at that time. Postoperative computed tomography (CT) of the abdomen showed no pathologic findings. No metastases were detected at postoperative control examinations. She was well without any symptoms until one month before admission, when she noticed pretibial edema of increasing severity followed by ascites and a generalized weakness. On admission she was in severe respiratory distress. Her pulse was barely palpable and blood pressure was 60/40 mm Hg. Both eyelids were puffy and she had severe perioral cyanosis. Heart sounds were distant. There was a massive hepatomegaly and tremendous ascites and pretibial edema. Transthoracic echocardiography (TTE) performed elsewhere showed an intracardiac mass, either a thrombus or a tumor. Repeat TTE in our clinic showed a mild pericardial effusion and a mass obliterating the right ventricular cavity (Figure 1). Although differential diagnosis of the thrombus from a tumor could not be made, the patient's general condition did not allow further diagnostic evaluation.
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Fig 1. Preoperative transthoracic echocardiographic view of the heart (apical four chamber view). (RA = right atrium; RV = right ventricle; LV = left ventricle; T = tumor mass.)
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The patient was intubated and a decision to remove the mass was made. At operation, the right atrium and the superior vena cava were both dilated and a moderate serous pericardial effusion was present. The apex of the right ventricle was rigid and akinetic with several white plaques. After standard aortic and bicaval cannulation and under normothermic cardiopulmonary bypass, the aorta was cross-clamped and cardiac arrest was induced by the warm antegrade blood cardioplegia. An oblique right atriotomy revealed a grape-like mass protruding through the tricuspid valve with no valvular destruction.
The bulk of tumor was very fragile and had occupied most of the right ventricular cavity. It originated from the anterior wall of the right ventricle with no clear boundary from the healthy myocardium. Near-complete resection of the tumor was attained through the right atrium and the pulmonary artery, but its attachment to the ventricular wall was left intact. Because of the extensive nature of the process, no attempts at complete resection of the diseased right ventricular wall and further patch reconstruction were made.
Postoperatively, central venous pressure dropped to 12 mm Hg from the preoperative value of 38 mm Hg. Recovery was uneventful with near-complete dissapearance of ascites, hepatomegaly, and pretibial edema within days. Immunohistochemical examination (Mic-2, CD99) of the biopsy specimen confirmed the diagnosis of PNET (Figure 2). Postoperative TEE revealed right ventricular wall thickening, probably due to tumor infiltration, but no intracavitary space-occupying mass. Further studies for extracardiac metastases were negative.
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Fig 2. Immunohistochemical staining of the excised tumor under light microscopy (Mic 2-CD 99 staining, x100 magnification).
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Beginning 3 weeks after the operation, 4 courses of chemotherapy, consisting of vincristine, epirubicin and iphosphamide, were adminstered. There was no indication of recurrence of the tumor 3 months after the operation. Cardiac magnetic resonance imaging (MRI) showed right ventricular wall thickening due to tumor infiltration but no intracavitary mass. At 6 months, the patient was readmitted with cardiogenic shock. Control TTE showed a huge mass in the right atrium and ventricle. The cardiogenic shock progressed and she died within hours. Postmortem examination could not be made.
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Comment
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Cardiac tumors are rare [1], and can be divided into primary and secondary tumors. Myxomas constitute up to 70% of all cardiac tumors. They are usually located in the atria, more often in the left side. Systemic metastasis is rare and surgical resection is the treatment of choice, with excellent long-term survival [2, 3].
Secondary cardiac malignancies can be due to either local extension of the adjacent neoplastic processes or metastasis from a distant organ. Cardiac metastasis can be the first manifestation of an extracardiac malignancy. These are mostly due to the space-occupying properties of the intracavitary tumors or to dysfunction of the cardiac valves. Rarely, the myocardial infiltrative tumors in the ventricles produce signs and symptoms of myocardial insuffiency [4].
In a selected group of patients whose primary tumor is well confined and progresses slowly with no evidence of widespread disease, resection of the cardiac metastasis can improve survival [5].
Although TTE or TEE has become the mainstay of the diagnosis of cardiac tumors, important limitations are present, such as the difficulty of evaluating tumor extension in the adjacent mediastinal structures. Furthermore, the acoustic impedance of a cardiac tumor may mimic that of a thrombus. CT and MRI are very valuable diagnostic tools in these circumstances. MRI distinguishes the signal intensity of blood from that of the other tissues. It also delineates the cardiac chambers, helping to determine whether the patient is a surgical candidate. In this patient, severely deteriorated hemodynamic status necessitated immediate surgery without MRI.
PNET is a malignancy of the peripheral nervous tissues classified as a round cell tumor. Complete surgical resection if feasible, together with radiotherapy and chemotherapy, can confine the disease in some cases. All the tumors classified as round cell tumors very infrequently give rise to secondary cardiac metastases. Prognosis mostly depends on the fate of the primary site, and the surgical intervention toward the cardiac secondary is not needed. Primary PNET of the heart has been reported and treated by cardiac transplantation [6]. Although our patient was very young, after the stabilization of her condition she could not benefit from cardiac transplantation because of the lack of donor heart .
Although surgical excision does not cure the primary disease in metastatic tumors of the heart, it should be attempted in circumstances when either the primary disease is under control or there is immediate life-threatening danger due to an intracavitary space-occupying lesion [7]. In our case, the primary site was under control. Severe manifestations of right heart failure necessitated emergency debulking of the mass. The final step in the treatment of this patient would be cardiac transplantation; however, unfortunately, she could not benefit from this because of the lack of donor organ.
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References
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- Kamiya H, Yasuda T, Nagamine H, et al. Surgical treatment of primary cardiac tumors: 28 years' experience in Kanazawa University Hospital. Jpn Circ J. 2001;65:315–319[Medline]
- Kaynak K, Besirli K, Arslan C, Ozguroglu M, Oz B. Metastatic cardiac myxoma. Ann Thorac Surg. 2001;72:623–625[Abstract/Free Full Text]
- Baba HA, Engers R, Heintzen MP. Right atrial metastasis as primary clinical manifestation of hepatocellular carcinoma. Int J Cardiol. 1995;47:281–284[Medline]
- Papa MZ, Shinfeld A, Klein E, Greif F, Ben-Ari G. Cardiac metastasis of liposarcoma. J Surg Oncol. 1994;55:132–134[Medline]
- Charney DA, Charney JM, Ghali VS, Teplitz C. Primitive neuroectodermal tumor of the myocardium: a case report, review of the literature, immunohistochemical, and ultrastructural study. Hum Pathol. 1996;27:1365–1369[Medline]
- Chu MW, Aboguddah A, Kraus PA, Dewar LR. Urgent heart surgery for an atrial mass. Metastatic hepatocellular carcinoma. Ann Thorac Surg. 2001;72:931–933[Abstract/Free Full Text]
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Abstract
Introduction
