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Ann Thorac Surg 2006;82:1531-1535
© 2006 The Society of Thoracic Surgeons
a Department of Thoracic Surgery, First Hospital of Tsinghua University, Beijing, China
b Cardiothoracic Surgical Unit and Cardiac Pediatric Unit, Grantham Hospital, The University of Hong Kong, Hong Kong, China
Accepted for publication February 14, 2006.
* Address correspondence to Dr Lik-Cheung Cheng, Cardiothoracic Surgical Unit, Grantham Hospital, 125 Wong Chuk Hang Rd, Aberdeen, Hong Kong, China. (Email: lccheng{at}graduate.hku.hk).
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The boy was born at full term with uneventful perinatal course. He was admitted to the pediatric service at 52 days after birth because of repeated convulsions (4 times). The convulsions were well controlled with phenobarbitone. However, the boy had persistent tachycardia and tachypnea, and was found to have a heart murmur. No family history of congenital heart disease, epilepsy, or known history of tuberous sclerosis was noted. Physical examination revealed satisfactory general condition, body weight of 5 kg, with no neurocutaneous markers. There was tachypnea (55 to 66/min) with mild insucking. Serum sodium was only 116 mmol/L (reference range, 134 to 145 mmol/L). He also was suspected to have clinical sepsis, with a white blood cell count of up to 40 x 109/L (reference range, 4.0 to 11.0 x 109/L). Antibiotics were administered, but no improvement was observed. His C-reactive protein was increased up to 180 mg/L (reference range, < 7.6 mg/L). Blood gas analysis showed metabolic acidosis and SaO2 of 80%. Electrocardiogram revealed sinus tachycardia of 160 beats/min. A computed tomographic scan of the brain was normal. Echocardiography showed multiple soft tissue masses in the right ventricle, occupying almost 80% of the volume of the right ventricular cavity. The largest mass measured 1.86 cm x 1.36 cm and extended into the right atrium through the tricuspid annulus, causing obstruction at the right ventricular inlet and decreased blood flow. A patent foramen ovale (0.56 cm in diameter) was present with right to left shunt at a gradient of 12 mm Hg (Fig 1A).
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Histologic examination of the tumor showed scattered large spindle cells and abundant myxoid stroma containing small lymphocytes, histiocytes, occasional plasma cells, and eosinophils (Fig 3A). On immunohistochemical stain, the tumor cells expressed muscle-specific actin, but were negative for CD31, CD34, calretinin, and anaplastic large cell lymphoma kinase protein (ALK-1) (Fig 3B). Based on these findings, a diagnosis of cardiac inflammatory myofibroblastic tumor was made. The baby remained asymptomatic 3 months later with normal C-reactive protein level and no echocardiographic evidence of recurrence (Fig 1B).
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Based on the literature and our data, there were 7 male and 7 female patients, ranging in age from 2 months to 72 years. Although there is a wide range of age, IMT affecting the heart seems to occur mainly in children and adolescents with only 3 reported cases in adults. The lesions range in size from 1.5 to 6.5 cm. Involved sites include right atrium (5 cases), right ventricle (4 cases), left atrium (1 case), left ventricle (2 cases), coronary arteries (2 cases), and pulmonary vein (1 case). Total excision was performed in 7 cases, subtotal excision in 4, and biopsy in 1. Two patients died suddenly, including 1 who died of angiocentric growth of the IMT into the coronary arteries [7], and 1 with an unclear cause of death. Rapid recurrence was found in 1 case and spontaneous recession was noted in another. Concurrent cardiac abnormalities were found in 3 cases (21%), including 1 patient with patent foramen ovale, 1 with atrial septum defect, and 1 with pulmonary valve stenosis, patent ductus arteriosus, and an atrial septum defect.
Histologically, IMT consists of spindle cells with an associated inflammatory cell infiltrate. The spindle cells are arranged in a loose myxoid stroma and have bland cytologic features. No conspicuous mitotic activity or atypia is observed. The inflammatory cells usually are lymphocytes, histocytes, plasma cells, and eosinophils, with plasma cells often predominating. On immunohistochemical analysis, diffuse expression of muscle specific actin may be found.
Although the cause is presently unknown, immunological and infectious mechanisms have been postulated by Stark and colleagues [8]. Data from our patient show that the boy had clinical sepsis concomitant with IMT. It seems that there is a correlation between IMT and inflammatory syndrome. Generally an inflammatory syndrome can be suspected when the serum C-reactive protein level exceeds 10 mg/L [12]. In our case, the serum C-reactive protein level was as high as 180 mg/L. Similar manifestations were also described by Jenkins and colleagues [9]. Because of the association with different inflammatory syndromes, IMTs are presumed to release cytokines that may produce fever, anorexia, anemia, thrombocytosis, and polyclonal hypergammaglobulinemia.
Of the 14 cases reported, 2 were asymptomatic, 10 had varying clinical presentations, and in the remaining 2 there were no clinical features described. In the 10 cases with clinical features, 4 manifested constitutional symptoms including fever, anemia, vasculitis, polyarthritis, and vascular thrombosis. These associated constitutional symptoms and signs often resolved once the tumor masses were excised. Apart from these constitutional symptoms, IMT may display a variety of obstructive clinical features, depending on the site at which the tumor originates, its size, and the mobility of the tumor. Typically the clinical findings are related to the tumor's ability to impede the filling of the ventricles. Therefore symptoms and signs may mimic those of mitral or tricuspid valve stenosis. Usually the patients present with pallor, diaphoresis, dyspnea, tachycardia, and decreased oral intake. Less commonly the tumor impedes the valve leaflet coaptation causing regurgitation of the valve or obstructs outflow of blood causing congestive heart failure. In one reported case, the patient presented with complete heart block suggesting the involvement of atrioventricular node [13]. Our patient had tachycardia and tachypnea, probably resulting from tricuspid valve obstruction.
Cardiac IMT is usually benign in nature. One curious feature of this tumor is its potential for regression. Pearson and colleagues [5] reported a case in which a decrease of 40% in size occurred during an 11-month period in an inoperable lesion. However the behavior of IMT is somewhat less predictable because of the possible rapid recurrence [11]. It may even cause serious morbidity or mortality by intracardiac mechanical obstruction or by the involving of vital structures, such as the mitral valve and coronary arteries [10]. Overall the prognosis of cardiac IMT seems to be related more to its location than to its neoplastic potential.
Echocardiography is the imaging modality of choice for primary diagnosis. Tumor location and characteristics can be observed by two-dimensional transthoracic echocardiography. Transesophageal echocardiography may be helpful for further delineating the tumor. The diagnostic value of computed tomography and magnetic resonance imaging for IMT has not been widely reported. Narla and colleagues [16] reported a case with cardiac IMT highlighting the magnetic resonance imaging characteristics. The lesion was slightly hyperintense relative to the cardiac muscle on T1-weighted images, moderately hyperintense on T2-weighted images, and heterogeneously enhanced after administration of contrast material. But these findings are not specific for IMT. The definitive diagnosis is usually made on histologic examination after surgical resection.
The IMT should be included in the differential diagnosis of any primary cardiac tumor, but the rarity of cardiac IMT poses a diagnostic challenge for clinicians and even for the pathologists. In children, the clinical and morphologic features of IMT often overlap with other intracardiac tumors such as rhabdomyoma, which is the most common cardiac tumor in children. Most rhabdomyomas occur in children younger than 1 year of age. Approximately one half of the patients with rhabdomyoma have tuberous sclerosis, and approximately one half of patients with tuberous sclerosis develop rhabdomyoma. They invariably involve the ventricles. More than 90% of rhabdomyomas are multicentric [17]. Histologic examination usually reveals classic "spider cells," which are not encountered in IMT.
With respect to treatment, surgical resection seems to be the treatment of choice. Of the 14 cases described so far, 12 cases underwent surgery. The tumor is resected as much as possible on cardiopulmonary bypass. Tumors in the ventricle may be resected without additional full-thickness excision of a portion of the ventricular wall to preserve the vital structures.
The results of surgical excision are good with a low morbidity and mortality. Recurrence of IMT is rare, with only one case reported [11]. This patient suffered recurrence at the same site 7 months after the first resection, and underwent a second resection. No further recurrence was observed 18 months after the second resection. Regular echocardiography follow–up is recommended after the operation. We believe that recurrent IMTs should be resected again when possible. Other treatment modalities such as steroids, have been described, but have yielded inconsistent results.
In summary, cardiac IMT seems to be a neoplastic lesion with a benign clinical course. However, it may cause catastrophe when critical sites such as the mitral valve and coronary arteries are involved. It has a predilection for younger patients. The cause and the pathogenesis of the lesion are still unknown. Whenever feasible, surgical resection should be the mainstay of treatment. Other treatment modalities have not yielded definitive results. It is important for doctors to be alert to the possibility of aggressive behavior or recurrence after resection.
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This article has been cited by other articles:
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  B. Solomon, E. A. Grossi, D. Monteith, R. M. Donnino, B. Srichai, S. L. Dellis, and A. C. Galloway Extended Cardiac Resection for Obstructing Pseudotumor Due to Ormond Disease Ann. Thorac. Surg., August 1, 2010; 90(2): 636 - 638. [Abstract] [Full Text] [PDF]
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 M. Tomiyama, S. Nakatani, H. Ishibashi-Ueda, C. Yutani, and M. Yamagishi Inflammatory Pseudotumor of the Heart Ann Intern Med, September 4, 2007; 147(5): 351 - 352. [Full Text] [PDF]
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