A rare morphology of the cardiac fibroma in a child: a case report.

Here we report a rare morphology of a cardiac fibroma in a child. A 2-year and 8-month-old toddler came for "chronic constipation" and was found to have a heart murmur on cardiac auscultation. Further transthoracic echocardiography suggested "a strong echogenic mass in the left ventricular wall, with some part of "a string of beads" in shape extending into left ventricle outflow tract", which was atypical for either a tumor, thrombus or vegetation. The child underwent resection of the mass and mitral valvuloplasty. Pathological examination confirmed the mass as a cardiac fibroma.

Siblings with Gorlin-Goltz syndrome associated with cardiac tumors: a case report and review of literature.

Primary cardiac tumors in children are very rare and may be associated with severe arrhythmias and sudden infant death syndrome. These cardiac arrhythmias vary depending on the location and size of the tumor. Sixty-four percent of children with cardiac fibroma, the second most common benign cardiac tumor in children, have ventricular arrhythmias, affecting therapeutic management and risk profile of these children. We report on two siblings with cardiac fibromas whose clinical presentations differed depending on their locations and size of the tumors. The first child, a three-year-old girl, was diagnosed with a cardiac fibroma in the left ventricle at the age of 8 months after surviving resuscitation due to ventricular fibrillation. Secondary prophylactic implantation of an ICD was performed. On propranolol, no further malignant arrhythmias have occurred to date. The seven-month-old brother was diagnosed postnatally with a cardiac tumor adjacent to the right ventricle. A few weeks after birth, the boy had refractory supraventricular tachycardia and ventricular arrhythmia that only resolved with amiodarone. In genetic testing, Gorlin-Goltz syndrome was diagnosed in both children. Conservative pharmacological therapy is a therapeutic strategy for asymptomatic patients with cardiac fibromas. The anti-arrhythmic medication depends on the location of the tumor. Implantation of an ICD should be performed in cases of malignant arrhythmias. In rare cases, there is an association between cardiac tumors and genetic syndromes, such as Gorlin-Goltz syndrome. These should always be considered when such a tumor is diagnosed.

Calcified right ventricular fibroma in an adult.

BACKGROUND: Cardiac fibromas are benign tumours of the heart and are composed of fibroblasts and collagen. They are one of the most common cardiac tumours encountered in children and adolescents but are rare in adults. CASE REPORT: The patient was a 57-year-old man who presented, complaining of a two-year history of chest tightness at rest. Transthoracic echocardiography revealed a severely calcified mass protruding from the anterior wall of the right ventricle near the apex. The patient was referred for tumour resection. Postoperative histopathological examination identified the calcified mass to be a cardiac fibroma. The patient's postoperative recovery was unremarkable and he was discharged eight days after surgery. During follow up, he has been free from any troublesome symptoms. CONCLUSIONS: Pre-operative diagnosis using various imaging modalities and early surgery are key to optimising the prognosis of patients with a cardiac fibroma.

Ventricular fibrillation and arrhythmia associated with cardiac fibromas involving both ventricles in a 1.5-year-old girl.

This article reports the autopsy findings of a 1.5-year-old girl with no history of previous hospital admission who suddenly collapsed at home. After 45 minutes of resuscitation efforts, the cardiac activity was restored. During hospitalization, she had ventricular arrhythmia and extremely elevated cardiac troponin levels. Internal examination and immunohistochemistry revealed cardiac fibromas of the right and left ventricles and extensive hypoxic myocardial damage. The right ventricular fibroma demonstrated interdigitating and entrapped myocardium visible at the edges and within the central portion of the tumor. The left ventricular fibroma originated in the subepicardial region and propagated towards the endocardium.

Giant fibroma of left ventricular apex with T wave inversion: 2 cases report and literature review / 中国综合临床

Objective:To investigate the clinical features, diagnostic methods and treatments of left ventricular apical fibroma.Methods:The clinical manifestations, ECG, imaging features and treatment plans of 2 patients with giant fibroma of left ventricular apex diagnosed in September 2020 and May 2022 were analyzed retrospectively, and the related literature was reviewed.Results:Both patients had slight chest distress and discomfort after activities. The ECG showed T-wave inversion of different degrees, which were misdiagnosed as “myocarditis” and “coronary heart disease” respectively. The cardiac magnetic resonance imaging and echocardiography showed left ventricular apical mass. Coronary artery stenosis was not found in coronary angiography. One patient required conservative treatment, and there was no significant change in clinical symptoms and tumor size in the follow-up for half a year; Another patient underwent cardiac mass removal, and the pathological examination after operation confirmed that it was cardiac fibroma, and there was no recurrence in the follow-up 2 years.Conclusion:Fibroma of left ventricular apex is a rare cardiac tumor, which is easy to be missed and misdiagnosed, and is one of the rare causes of T-wave inversion. Cardiac magnetic resonance imaging, CT and echocardiography are commonly used imaging methods to diagnose cardiac fibroma, and surgical resection is an effective treatment.

[Heart imaging by CT-scan and MRI in cardiac tumors]. / L'imagerie en coupe dans le bilan des tumeurs intra-cardiaques.

The etiology of cardiac masses is often oncological or thrombotic, rarely inflammatory. Among heart tumors, the vast majority are metastatic. We describe the most frequent benign primary cardiac tumors and the most frequent malignant primary cardiac tumors and give information about the advantages of using a multi-modality approach for the accurate diagnosis of a cardiac mass using Computed Tomography Scanner and Magnetic Resonance Investigation.

Case report: multi-modality imaging of a right ventricular fibroma in a teenager.

Background: Cardiac fibroma is a rare primary benign tumour of the heart. It often causes arrhythmia, endangers the lives of patients, and has a worse prognosis than other benign tumours. We report a 14-year-old female patient with a right ventricular fibroma. Various preoperative imaging examinations showed that the lesion was benign, and postoperative pathology confirmed that the lesions were fibroma. Case summary: A 14-year-old female patient visited her doctor for more than 5 months because of a heart murmur. Echocardiography revealed a slightly hyperechoic mass in the right ventricle, and on myocardial perfusion contrast imaging, the lesion showed equal enhancement. And the lesion also showed enhancement on contrast-enhanced gated cardiac computed tomography (CT). Contrast-enhanced magnetic resonance imaging (MRI) of the heart revealed that the lesion was isointense on T1-weighted image (T1WI), and isointense to slightly hyperintense on T2-weighted image (T2WI). The lesion was significantly homogeneously enhanced on a delayed enhancement scan. A positron emission tomography-CT (PET-CT) with 18F-fluorodeoxyglucose (18F-FDG) demonstrated that the mass showed lower levels of 18F-FDG uptake. These features suggested this lesion was a benign lesion. The postoperative pathology suggested the lesion was a right ventricular fibroma. The patient was discharged 14 days after surgery and remains disease-free and asymptomatic 14 months after surgery. Discussion: Cardiac fibromas are histologically benign, but they can cause obstruction and malignant arrhythmia. The gold standard for diagnosing fibroma is pathology. However, in the absence of pathology, it is necessary to use various imaging methods to evaluate the lesions to distinguish between benign and malignant tumours.

From Goliath to David: Interventricular fibroma.

We describe an interventricular septum mass in 1 years old child, followed during 14 years. The mass did not grow up over time, the patient did not experienced any arrythmia, and did not developed heart failure. A complete diagnosis of interventricular Fibroma was made at the age of 14 years old when the patient underwent to cardiac MRI. A close follow up was in this case the winner strategy, saving him from an early unnecessary cardiac surgery.

A rare case of cardiac fibroma diagnosis in Gorlin-Goltz syndrome with information on management.

Gorlin-Goltz syndrome is a rare autosomal dominant disease characterized by odontogenic keratocysts and basal cell carcinoma as well as ophthalmic and neurological implications. The following article presents the case of a 20-year-old female with Gorlin-Goltz syndrome incidentally found to have a cardiac mass. An ECG showed diffuse T-wave inversions in the lateral leads despite a lack of any acute coronary symptoms in the patient. Echocardiogram, cardiac MRI and CT scan confirmed a nonvascularized, smoothly marginated mass arising from the left ventricular apex without any hemodynamic compromise. A whole-body PET scan further demonstrated localized hyperactivity associated with a cardiac fibroma without any evidence of metastasis. The cardiac fibroma was surgically excised for definitive management to prevent the possibility of sudden cardiac death and congestive heart failure.

Cardiac fibroma presents as a benign tumor of the heart. Although tumors of the heart are uncommon, patients who have Gorlin­Goltz syndrome are at a higher risk of developing these fibromas. In the following article, the authors discuss the presence of this rare cardiac fibroma in a 20-year-old female patient with Gorlin­Goltz syndrome. The patient's tumor was found incidentally during a CT scan after she presented with symptoms of flank pain. A follow-up was later conducted with cardiology and cardiac surgery. Several tests were performed to scan the exact tumor location in the heart. Finally, the tumor was removed, and the patient recovered after surgery. Later, the patient was diagnosed with depression and needed medicine to cope with emotional trauma from multiple surgeries.

A spiral cystic fibroma originating from left ventricular fascicular muscle: a case report.

BACKGROUND: In adults, cardiac fibromas are fairly rare, mostly round in shape, and few cases of ventricular fibromas of other morphology have been reported. CASE PRESENTATION: We report a case of a 47-year-old male patient admitted with recurrent nocturnal paroxysmal dyspnea, diagnosed by transthoracic cardiac ultrasound, transesophageal ultrasound, and computed tomography (CT) as a left ventricular occupancy with a spiral shape resembling a conch with a fixed base and a free distal end. CONCLUSION: This case reports a rare but noteworthy morphological features of the adult uncommon ventricular tumor pathological type. Furthermore, the patient had no notable postoperative issues and was followed up on for a year following surgery, with no residual tumors or arrhythmias discovered during the examination.

Asymptomatic cardiac tumour with premature ventricular contraction in an athlete: case report.

Cardiac fibromas are the second most common benign primary tumour of the heart in the children; the clinical features include chest pain, arrhythmia, low cardiac output due to outflow tract obstruction, and sudden cardiac death. Sports are associated with an increased risk for sudden death in athletes who are affected by cardiovascular conditions predisposing to life-threatening arrhythmias. We present a case report of 10-year-old asymptomatic boy who was referred to the paediatric cardiology department by his general practitioner for cardiac examination before participation in competitive sports. The electrocardiogram showed premature ventricular contractions originated from inferior of left ventricle. A mass was detected by 2D transthoracic echocardiography, and it was found to be compatible with fibroma on MRI. In some cases, cardiac tumours are asymptomatic as in our patient. Electrocardiogram abnormalities require detailed cardiac imaging with echocardiogram, and if necessary CT/MRI. In this article, we emphasise that detailed cardiac examination of individuals before participating in competitive sports is vital.

Cardiac fibroma: A clinicopathologic study of a series of 12 cases.

BACKGROUND: Cardiac fibroma (CF) is a rare tumor that has not been widely reported. This study investigated the clinical findings, histologic features, and differential diagnosis of CF. METHODS: A total of 12 CF cases were studied and reviewed using hematoxylin and eosin (H&E), special staining and immunohistochemical staining. The ALK gene was tested in 4 cases of cardiac fibroma with significant inflammatory cells. Clinicopathological data were retrospectively analyzed and followed up. RESULTS: The cases occurred in six males and six females ranging in age from 0.5 to 55 years (median, 5 years). The tumors were grossly single and solid (1-17 cm; mean 5.6 cm). The clinical signs and symptoms depended largely on the location of the tumor. Microscopically, the CFs observed were composed of monomorphic spindle cells and abundant collagen. The spindle cells demonstrated little or no atypia. The histology of CFs in infants and young children showed some differences from those in adults. Infants and young children with fibromas exhibited cellular types with more inflammatory infiltration. All tumors expressed vimentin markers. Eleven of 12 cases (91.7%) were positive for SMA by immunohistochemistry. ALK immunostaining and ALK-FISH tests showed negative results. Follow-up information was available for all patients. The mean postoperative follow-up was at 3 years (range 2 months-8.8 years). All patients were alive with no evidence of disease. CONCLUSIONS: Our study shows that CFs exhibit a wide morphological spectrum of soft tissue tumors with fibroblastic or myofibroblastic differentiation and/or components. Infants and younger pediatric patients with fibromas have tumors that are more hypercellular and more likely to be misdiagnosed with aggressive or malignant lesions than adults. Finally, the data indicate that CF exhibits benign behavior and that local resection is safe and effective.

Surgical experience of pediatric cardiac fibromas / 中华胸心血管外科杂志

Objective:To summarize single-center experience and short-term outcomes of surgical treatment of pediatric cardiac fibromas.Methods:There was a retrospective study of 10 patients who underwent surgical treatment of cardiac fibromas between January 2018 and October 2021. Fibromas were located in the left ventricle in 7 cases, and in the right ventricle in 3 cases. Mean tumor diameter was(5.6±2.0) cm.Results:Median age at surgery was 3.1 years old(5 months-9 years old). Nine patients received complete resection, and the other one received partial resection. One patient with giant left ventricular fibroma required extracorporeal membrane oxygenation support for ventricular fibrillation after weaning from cardiopulmonary bypass, and was successfully weaned on the third postoperative day. There was no early mortality. Median follow-up time was 6 months(1 month-3 years). No tumor recurrence nor ventricular arrhythmia was documented during the follow-up period.Conclusion:Pediatric cardiac fibromas can be usually treated by complete resection, with relief of cardiac dysfunction and ventricular arrythmia after surgery, and with satisfactory results.

Hypertrophic Cardiomyopathy with a Twist.

Hypertrophic cardiomyopathy is a relatively common inherited cardiac disorder, with echocardiography still being the initial imaging method for its diagnosis. Cardiac fibroma is a primary neoplasm that most commonly presents in childhood. We present a patient who was investigated for typical cardiac symptoms who went on to have cardiac magnetic resonance imaging and was found to have both of these conditions.

Aortic coarctation with cardiac fibroma in a young patient: a case report.

BACKGROUND: Cardiac fibroma and aortic coarctation are rarely observed concomitantly in the same patient. We report a case of cardiac fibroma with aortic coarctation treated with a hybrid surgical procedure. To the best of our knowledge, this is the first case of these two abnormalities existing in one patient. CASE SUMMARY: A 22-year-old female patient visited the clinic with a 10-year history of hypertension. Physical examination revealed blood pressure of the upper extremities 50 mmHg higher than that of the lower extremities. Computed tomography angiography revealed a post-ductal-type aortic coarctation at the beginning segment of the descending aorta along with a 7.7 cm × 5.1 cm left ventricular mass. Transthoracic echocardiogram showed a mass at the middle segments of the lateral wall and apex and posterior wall of the left ventricle. Cardiac magnetic resonance imaging also showed the mass with hypointense signal on T1, hyperintense signal on T2, and intense signal on late gadolinium enhancement. No evidences of metastatic lesions were observed on 18F-fluorodeoxyglucose positron emission tomography. The patient underwent a hybrid surgery involving aortic stent implantation and complete left ventricular mass removal. The gradient between stenosis returned to <10 mmHg after the procedure. Pathologic findings revealed cardiac fibroma. DISCUSSION: It is rare to encounter a patient suffering from both cardiac fibroma and aortic coarctation. No evidences indicated a single cause or syndrome resulting in the coexistence of these two abnormalities. A hybrid surgery involving aortic stent implantation and complete cardiac mass resection could optimize the treatment in such cases.

Primary cardiac fibroma in infants: A case report and review of cases of cardiac fibroma managed through orthotopic heart transplant.

Cardiac fibromas (CF) are the second most common cardiac tumors in children. They can be aggressive tumors despite their benign histopathologic nature, accounting for the highest mortality rate among primary cardiac tumors. CF usually show a progressive growth and spontaneous regression is rare. Therefore, a complete surgical excision is the preferred therapeutic approach when patients become symptomatic or if mass-related life-threatening complications are anticipated, even in asymptomatic patients. However, some cases are not good candidates for surgical excision due to the impossibility of preserving a normal cardiac anatomy or function after the tumor resection. Orthotopic heart transplantation (OHT) can be an exceptional but adequate alternative for some giant unresectable CF in children. In this article, we report our experience with the case of a 7-month-old infant with a giant unresectable cardiac fibroma who was successfully managed through OHT.

Case report: surgical resection of right ventricular cardiac fibroma in an adult patient.

BACKGROUND: Cardiac fibromas are rare benign cardiac neoplasms, most frequently occurring in the pediatric population; with very rare cases identified in adults. The tumors are comprised of spindled cells with myofibroblastic ultrastructural features embedded in generally collagenous and elastic stroma. The tumors are intramural in the ventricles, most commonly the left ventricle. Clinical symptoms vary by location and size of tumor and some are asymptomatic. Surgical resection is curative, but rare cases require cardiac transplantation. CASE PRESENTATION: We report an asymptomatic, large, right ventricular fibroma in a 64-year-old woman. The patient underwent open incisional tumor biopsy via lower hemi-sternotomy, followed by complete tumor resection via full sternotomy a week later after confirming the tumor is benign. The tumor was resected using cardiopulmonary bypass, and the defect of right ventricular free wall was repaired using a prosthetic double-patch technique. The postoperative course was uneventful. The patient was discharged to home on day 4 post-complete tumor resection. CONCLUSION: This report expands the existing literature for better comprehension and detection of cardiac fibroma patients and also highlights the various imaging modalities, surgical management, and histological analysis.

Primary cardiac fibroma of the right ventricle in an adult: report of one case.

Cardiac fibroma is a rare primary benign cardiac tumor, especially in adults. It often occurs in the interventricular septum and free wall of the left ventricle and is solitary and space-occupying with clear boundaries. Here we report a 27-year-old male with a cardiac fibroma in the right ventricle, with extensive infiltrative growth. He was admitted to hospital with the complaint of exertional chest tightness, shortness of breath, hemoptysis, and edema of lower extremities. Ultrasound showed a large right ventricular mass blocking the outflow tract. The patient underwent palliative resection. Pathologic examination and Masson staining showed that collagen tissue proliferated and infiltrated myocardial fibers. The final diagnosis was cardiac fibroma.

A case report of primary cardiac fibroma: an effective approach for diagnosis and therapy of a pathologically benign tumour with an unfavourable prognosis.

BACKGROUND: Primary cardiac fibroma is exceedingly rare. This condition involves a significant risk of life-threatening arrhythmias during follow-up and its prognosis is not as favourable as other benign tumours. We report a case of cardiac fibroma that was preoperatively diagnosed with echocardiography and magnetic resonance imaging. This fibroma was excised early as a preventative measure to avoid sudden death. CASE SUMMARY: A 46-year-old woman presented to our hospital with a 1-year history of chest tightness at rest. Echocardiography showed a large, isoechoic, well-circumscribed mass within the left ventricular myocardium with calcified tissue. Magnetic resonance imaging showed an intramural ventricular mass with iso signal intensity on T1-weighted imaging and low-signal intensity on T2-weighted imaging. There was no enhancement on first-pass perfusion imaging and homogeneous hyperenhancement on late gadolinium enhancement imaging. These features suggested a diagnosis of cardiac fibroma. Complete resection was performed to avoid sudden death and pathological analysis confirmed the tumour as cardiac fibroma. The patient was discharged 9 days after surgery and remains disease-free 5 months after surgery. DISCUSSION: Cardiac fibroma is a pathologically benign tumour with an unfavourable prognosis because of lethal arrhythmias, which can be controlled by its resection. Thus, it is important to preoperatively distinguish cardiac fibroma from other benign tumours, in order to prioritize surgical intervention for those with cardiac fibromas. Preoperative diagnosis with echocardiography and magnetic resonance imaging and early preventative surgery are the keys to improve prognosis of patients with cardiac fibromas.

Surgical results and pathological analysis of cardiac fibroma in the adolescent and the adult.

BACKGROUNDS: Disparities may exist between the adolescent and the adult patients with cardiac fibromas in the symptoms, surgical outcomes, and pathological characteristics. The aim of this study was to compare short and midterm surgical outcomes of cardiac fibromas and to compare the biomarker expressions of tumor tissue samples between the adult and the adolescent. METHODS: Consecutive patients with the diagnosis of cardiac fibroma were admitted and received surgeries. Primary outcomes included in-hospital mortality, low cardiac output, and readmission due to heart failure. The expression of PCNA and Ki67, two widely adopted indicators of cell proliferation, were evaluated in tissue samples. RESULTS: A total of five adolescent patients and five adult patients diagnosed as cardiac fibroma were admitted and given surgeries. When compare with the adults, the adolescent patients were more likely to present symptoms on admission (P = .048). Postoperative low cardiac output syndrome was significantly higher in the adolescents than in the adults (80.0% vs 0.0, P = .048). The tumor volume relative to ventricular end diastolic diameter had good discriminative ability for low cardiac output (c statistics: 0.96). Pathologically, the percentage of PCNA-positive cell nuclei was significantly higher in the adolescents than in the adults (36.04% ± 10.54% vs 4.15% ± 3.93%, P = .001). However, there were no Ki67-positive nuclei in the 10 cases. CONCLUSIONS: In the current study, we found that postoperative low cardiac output was more likely to occur in the adolescent patients than in the adult patients. When compared with the adult patients, significantly more PCNA-positive nuclei were observed in the adolescents.