Benign Primary Cardiac Tumors Research Articles

Herztumoren: Magnetresonanztomographie und Mehrschicht-Spiral-CT

Transthoracic echocardiography is usually the initial diagnostic test in patients with a suspected cardiac mass. However, this technique is restricted by its small field of views and insufficient acoustic window in some patients. Magnetic resonance imaging (MRI) and, since its introduction, multislice spiral computed tomography allow for detailed delineation of intra and pericardiac tumors, their extent, and their influence on cardiac function. Primary benign and malignant cardiac tumors have several characteristic features in MR imaging. Assessment of such features may narrow down the differential diagnosis or even allow for reliable diagnosis in selected cases. Many such features can also be assessed using MSCT. This article provides an overview of examination protocols of MRI and CT for cases in which a cardiac mass is suspected and describes the appearance of primary and secondary cardiac masses as well as intracavitary thrombi.

Recurrence of a right ventricular hemangioma

The majority of cardiac tumors are benign, with only 25% to 30% being histologically malignant. Only 0.8% to 5% of all benign primary cardiac tumors are hemangiomas. Cardiac hemangiomas are vascular tumors, composed of capillaries or cavernous vascular channels. Patients usually have a variety of symptoms depending on location and extension of the tumor. Diagnosis and management of these tumors are difficult and delayed and require multiple investigations. We report a case of late recurrence of a cardiac hemangioma of the right ventricle from surgical treatment at our institution 10 years previously.

Cardioembolic stroke due to papillary fibroelastoma

Papillary fibroelastoma (PFE) is a rare primary benign cardiac tumor with significant embolic potential. Diagnosis and requisite surgical treatment may be neglected unless transesophageal echocardiography (TEE) is performed. A 72-year-old man with hypertension and diabetes had a right middle cerebral artery stroke of unclear cause after standard work-up. Subsequent TEE indicated an aortic valve PFE. The TEE may be the only diagnostic modality to identify embolic lesions such as PFE and must be strongly considered in cryptogenic large vessel stroke. Copyright © 2001 by National Stroke Association

Therapy for primary cardiac tumors: is there a role for heart transplantation?

Primary cardiac tumors are rare clinical entities. Benign tumors are often amenable to surgical excision, whereas malignant tumors are seldom resectable. The literature has reported that 28 patients have undergone orthotopic heart transplantation for inoperable primary cardiac tumors. The results of these transplants are presented in this article. Of the 28 patients who underwent orthotopic heart transplantation for primary cardiac neoplasms, 7 patients had benign histology (fibroma-5, rhabdomyoma-1, and pheochromocytoma-1) and 21 patients had malignant histology (sarcoma-15, malignant fibrohistiocytoma-3, and lymphoma-3). Mean survival in the patients with benign histology was 46 months, and the mean survival in the patients with malignant histology was 12 months. However, there were seven patients with malignant histology who had survived for a mean of 27 months without evidence of recurrent disease. An awareness by clinicians of the presenting clinical picture of these tumors is warranted in view of the potential for cure by resection or transplantation. Patients with benign primary cardiac tumors appear to benefit from the complete resection afforded by cardiectomy and transplantation. The role of transplantation for patients with malignant tumors remains unclear. Further experience and continued follow-up of these patients is necessary to ascertain the role of cardiac transplantation, radiation, and chemotherapy in the management of patients with primary tumors of the heart.

A Case of Left Atrial Myxoma Presenting as Acute Pulmonary Edema

Myxomas are the most common primary benign cardiac tumor. In general, myxoma has a benign growth and chronic entity, so symtoms of myxoma are vague and mild. Therefore the association of an atrial myxoma with acute pulmonary edema and syncope appears to be very rare. Even though very rare, detection of myxoma is important because it is usually fatal in several cases if unrecognized and untreated. We experienced a case of left atrial (LA) myxoma presenting as acute pulmonary edema and syncope of unknown cause in 47 years old female patient. A mass located on left atrium was excised en bloc, and the patient recovered uneventfully. We report the case along with literature review.

Pediatric primary benign cardiac tumors: A 15-year review

An increase in the incidence of primary cardiac tumors has been reported since the development or enhancement of noninvasive imaging modalities. We identified 56 children with primary cardiac tumors. Forty-four (78%) children had rhabdomyomas, 6 (11%) fibromas, 1 (2%) pericardial teratoma, 1 (2%) epicardial lipoma, 1 (2%) multicystic hamartoma, and 3 (5%) unspecified tumors. The mean age at diagnosis was 19 ± 35 months (median 4.7 months, range 0.03 to 204 months), excluding 12 patients who were given the diagnosis before birth. Among 27,640 patients assessed for cardiac disease, the incidence of tumors was 0.06% (1980 to 1984), 0.22% (1985 to 1989), and 0.32% (1990 to 1995). Diagnosis was made in 55 of 56 patients by echocardiography. Catheterization was performed in five patients and magnetic resonance imaging in nine. No tumor-related deaths occurred. Nine patients had surgery because of hemodynamically significant obstruction or arrhythmias. Partial or complete regression occurred in 24 (54%) of 44 patients with rhabdomyomas. Overall, the prognosis was excellent. Individualized surgery allowed early safe treatment of symptomatic tumors. (Am Heart J 1997;134:1107-14.)

MRI of primary malignant cardiovascular tumors.

The purpose of this study was to describe the MR features of primary malignant cardiac tumors, which may aid in differentiation from primary benign cardiac tumors. Seven patients with primary malignant cardiac tumor were imaged with MR. All studies were performed on a 1.5 T MR imager using variable combinations of SE T1-weighted cine gradient MR and post-Gd-DTPA SE T1-weighted MR with fat saturation sequences. The following features were analyzed on images: length of attachment to cardiac structures, size of tumors, involvement of more than one chamber, extension to pericardium or beyond the heart, and necrosis of the mass. All cases had a wide point of attachment. Six of the seven tumors were large in size and occupied almost the entire cardiac chamber. There was involvement of more than one cardiac chamber or great vessel in three patients. Pericardial or extracardiac extension occurred in four and two patients, respectively. Tumor necrosis was evident in two patients. All patients had at least one of these signs of malignant tumor. Primary malignant cardiac tumors have distinctive MR features that can be used to differentiate them from primary benign cardiac tumors.

Echocardiographic evaluation of papillary fibroelastoma: A case report and review of the literature

Papillary fibroelastomas comprise approximately 7.9% of benign primary cardiac tumors. Although papillary fibroelastomas were at first discovered incidentally at autopsy or during heart surgery, these tumors are increasingly being identified by echocardiography. This article reviews those papillary fibroelastomas detected by transthoracic or transesophageal echocardiography and discusses the echocardiographic features of these tumors, associated symptoms, and management. Echocardiography is important in influencing management decisions regarding excision, valve replacement, and valve repair.

Spontaneous regression of cardiac rhabdomyoma

Multiple cardiac rhabdomyomas in a neonate with tuberous sclerosis were first described by Von Recklinghausen in 1862. These hamartomas are the cardiac tumors most frequently encountered during infancy and childhood. Rhabdomyomas account for 45% of primary heart tumors in children and represent 53% of primary benign childhood cardiac tumors. 1 Approximately 30% of patients with tuberous sclerosis have cardiac rhabdomyomas. 2 Their natural history is unclear because most reviews on this subject are based on autopsy data. The prognosis for cardiac rhabdomyomas is believed to be grim because of reported fatality rates of 53% by the first week of life and 78% by 1 year of age. 2,3 With widespread use of echocardiography in pediatrics during the last 2 decades, it has become clear that rhabdomyomas result in a wide spectrum of clinical manifestations, ranging from a total absence of symptoms to intrauterine or sudden postnatal death. Also reported are hydrops fetalis, dysrhythmias, inflow or outflow obstruction, congestive heart failure and possibly cerebral embolization. Histologic examination of these masses in 1923 was suggestive of spontaneous regression. 4 Isolated clinical reports of spontaneous regression have recently appeared. 5 We now describe a series of 5 infants with tuberous sclerosis who had close documentation of the size of their 13 tumors.

Lipomatous hypertrophy of the cardiac septum — a possible cause of sudden death in adults —

Cardiac Lipomas, accounting for 10% of primary benign cardiac tumours are usually asymptomatic, although they can sometimes be associated with a plethora of manifestations. Our patient was a 16 year old asymptomatic female with marked lipomatous hypertrophy of the interventricular septum protruding into the right ventricle, diagnosed incidentally during the ECHO evaluation for a haemic systolic murmur.

PRIMARY MYOCARDIAL FIBROMAS: REPORT OF 2 CASES.

Primary cardiac tumors, though uncommon, are not as rare as one might think. The recent literature contains several excellent review articles that classify and tabulate the various types of primary benign and malignant cardiac tumors (1, 7, 8, 10, 13). This communication presents 2 cases of primary benign cardiac tumors, i.e., myocardial fibromas. In these the x-ray examination showed an unusual pattern of calcification within the tumor. This has not been described in other case reports of cardiac fibromas or other primary cardiac tumors (5, 12, 16, 17). In earlier literature the term fibroma referred to papillary tumors of the cardiac valves. As described by Yater and Hertzog (1, 2), these were essentially fibrous papillomatous growths with an endothelia covering arising from the surface of a valve. Prichard, in his excellent review article (7), pointed out the difficulty of classification in regard to fibromas versus hamartomas, but he also considered fibromas papillary fibrous tumors arising from heart valves. More recently, fibroma has been used to describe a fibrous neoplasm sometimes called fibrous hamartoma, a nonencapsulated but well demarcated intramyocardial lesion which appears very similar to a leiomyoma in gross (9). They are most common in the first few years of life and most often primary in the left ventricular wall or intraventricular septum (5, 12, 16, 17). Neither the reviews nor the case reports, however, described calcification within the tumor mass. Central and peripheral calcification is seen frequently in myxomas, the most common primary cardiac tumors, and almost always in intracavitary polypoid tumors in the atria (7). Case Reports Two cases of primary benign cardiac tumors are described. The pathologic diagnosis in each case was confirmed by the Armed Forces Institute of Pathology. Case I. This 17-month-old white male was the product of a normal pregnancy but was delivered by cesarean section because of abnormal third trimester vaginal bleeding in the mother. During the first month of life the infant was normal, but from one to four months of age he suffered severe respiratory infection with congestive heart failure and cyanosis. This responded to digitalization. The patient did well until the age of sixteen months when he was seen for pneumonia. Chest radiographs were abnormal, with a homogeneous density in the left lower lobe confluent with the heart shadow. Diagnosis by the referring physician was cyst of the lung, possibly dermoid cyst, and the infant was referred to Wilford Hall USAF Hospital. Admission examination revealed a normally developed child in no acute distress. The pulse was 120 and regular. Oily skin and a fine scaly erythematous rash were present. Respiration was labored with left lower lobe râles. Sinus rhythm was normal without murmurs. The liver was palpable 2 finger-breadths below the right costal margin.