Cardiac tumours – a brief review

This short review of cardiac tumours presents a case that clearly demonstrates the manifestation of embolic and cardiac symptoms of an intracardiac mass. Acute onset and rapid progression of a neoplastic process in the heart leading to arrhythmia, cardiac conduction disorders and heart failure combined with highly mobile fragments of tumour, which can cause emboli in cerebral vessels, are characteristic signs of an intracardiac mass. Early diagnosis and immediate treatment may improve the long-term prognosis, but overall the prognosis is poor. Cardiac tumours present to the cardiologist when the patient presents with cardiac symptoms, and the neurologist when there are cerebral symptoms. Most cardiac masses are not amenable to percutaneous biopsy; therefore, definitive diagnosis often awaits surgical excision.Key words: classification, heart tumour, sarcoma     

Lymphadenitis due to nontuberculous mycobacteria in children: presentation and response to therapy.

The most common manifestation of infection due to nontuberculous mycobacteria (NTM) in children is cervical lymphadenitis in an otherwise healthy patient. We identified and reviewed 19 cases of proven or presumptive lymphadenitis due to NTM seen at our hospital over the course of 13 months. Nine patients underwent initial surgical excision of involved lymph nodes. Ten children did not have involved lymph nodes excised initially and were treated with macrolide-containing antibiotic regimens. Of these patients, five required subsequent surgical excision and five were cured with combination chemotherapy. Six patients underwent radiographic imaging of the head and neck that revealed asymmetrical adenopathy with ring-enhancing masses but minimal inflammatory stranding of the subcutaneous fat, a finding that may distinguish adenitis caused by NTM from staphylococcal and streptococcal adenitis. Our data suggest that if surgical excision is not considered feasible, antimicrobial therapy for adenitis due to NTM may be beneficial for some patients.     

Contribution of complementary examinations in the diagnosis and treatment of emboligenic cardiopathies. Retrospective study apropos of 46 cases

The aim of this prospective study was to assess the value of complementary investigations in the diagnosis and follow-up of embolic heart disease. Forty-six patients having presented a systemic embolism cardiac origin underwent a standard work up which included clinical examination, ECG, chest X-ray and 2D echocardiography. Other investigations were carried out in some patients: CT cardiac scan (11 cases), gamma scintigraphy with Indium III labelled platelets (6 cases) and angiocardiography (12 cases). The diagnosis of an embolic cardiac lesion was made after the standard investigations in 82% of cases. The remaining 18% of cases hall had echocardiographic abnormalities and enable the diagnosis of clinically imapparent conditions: mitral valve prolapse, aneurysm of the interatrial septum, valvular calcification and cardiomyopathy. A potentially embolic mass was visualised in 18 patients; 6 valvular vegetations, 12 left atrial or ventricular thrombi. The other specialised radionuclide, angiographic and CT investigations only confirmed the echocardiographic diagnosis of intravavitary thrombosis. These results were confirmed surgically in 19% of cases. This study shows that complementary investigations especially echocardiography, allow diagnosis of latent embolic cardiac lesions, some of which may benefit from surgical treatment. In addition, potentially embolic intracardiac masses may be visualised, so confirming the origin of systemic emboli. When surgery is not indicated, echocardiography is a good method of following up the results of medical treatment in some of these masses.     

Cardiac Epithelioid Leiomyosarcoma and the Role of Cardiac Imaging in the Differentiation of Intracardiac Masses

Primary malignant cardiac neoplasms are extremely rare and can be difficult to differentiate from other intracardiac masses. We present a case of left atrial epithelioid leiomyosarcoma and review the imaging modalities available to diagnose and characterize intracardiac masses. We demonstrate how multimodal imaging aided diagnosis and proved crucial to establishing the tumor's location, degree of invasion, and involvement of surrounding structures prior to surgical resection. Copyright © 2010 Wiley Periodicals, Inc.     

Cardiac myxomas: results of 14 years' experience

From January 1968 to July 1983, 38 patients with an intracardiac myxoma underwent surgical excision of the tumor at our Institution. There were 15 males and 23 females, ranging in age from 17 to 68 years (mean 47.7). In all but 2 patients, the presence of an intracardiac myxoma was documented preoperatively by angiographic and/or echocardiographic study. The first 2 patients of this series were referred with the diagnosis of mitral stenosis, and the myxoma was an unexpected surgical finding. In 32 cases (84.2%) the myxoma was located in the left atrium, in 4 (10.5%) in the right atrium and in 2 (5.3%) in the right ventricle. Surgery was performed on an emergency basis in 36 patients. Two patients (5.2%) both with a left atrial myxoma, died after surgery: one died intraoperatively of hemorrhage and the other after one month of bowel infarction. Follow-up of the 36 survivors ranges from 3 months to 14.5 years (average 5.2 yr); all were controlled by means of clinical and echocardiographic investigations, and no evidence of tumor recurrence was detected. Surgery for intracardiac myxoma, which should be undertaken on an emergency basis, carries a low operative risk; excision of the tumor appears to be curative with no recurrences at long-term follow-up; non-invasive tools are of the utmost importance in both the preoperative assessment and follow-up of these patients.     

Cystic tumor in the left atrium: an unusual aspect of a myxoma

Most cardiac tumors are benign, whereas up to 50 % of the diagnosed cases are histologically myxomas. The common clinical signs are rhythm disturbances, myocardial ischemia, pulmonary edema, syncope and cardiac arrest. They do normally lead to the diagnostic hypothesis of an intracardiac mass. The primary modality for imaging is echocardiography which usually confirms the suspected diagnosis. But in rare cases there are masses which cannot be exactly identified by this technique. Here we present a patient with an atypical echocardiography of an unusual intracardiac tumor.     

Surgical treatment of atrial thrombosis. A transesophageal echocardiogram study]

Transesophageal echocardiogram is the procedure of choice in the evaluation of masses located in the cardiac chambers. In this paper three cases of atrial thrombosis are presented: a) a free floating ball thrombus in the left atrium in a patient with moderate mitral stenosis; b) three thrombotic masses adherent to a central venous catheter; c) three thrombotic masses attached to Chiari s network in a patient operated for an ostium secundum atrial septal defect one year before. In all cases transesophageal echocardiogram has played a key role in the diagnosis. Medical therapy has been inadequate and so surgical removal has been performed. Unsuccessful medical therapy, a new episode of thrombosis, intracardiac permanent catheter infections and high risk of systemic and pulmonary embolism are indications for thrombectomy.     

Concomitant lipomatous hypertrophy and left atrial mass: Distinguishing benign from malignant

Masses in and near the interatrial septum may be either benign or malignant. The most common mass near the interatrial septum is lipomatous atrial septal hypertrophy (LASH). LASH can be present in patients with intracardiac malignancies, myxomas, lipomas, or other cardiac masses. It is important to recognize the transesophageal echocardiography (TEE) characteristics of these pathologies to arrive at an accurate diagnosis with an appropriate plan for intraoperative resection. At the authors’ institution, patients have been referred for surgery due to a finding of significant LASH masquerading as a left atrial myxoma. In challenging cases, TEE offers a thorough evaluation of the interatrial septum to delineate between multiple intracardiac masses.     

Successful treatment of recurrent intracardiac thrombus in Behçet's disease with immunosuppressive therapy

Beh?et's disease (BD) is a chronic multisystem inflammatory disorder characterized by recurrent oral and genital ulcers, skin eruptions and uveitis. Neurological, gastrointestinal, and musculoskeletal systems are also involved. Although venous and arterial vasculitis occur in up to one-third of patients, intracardiac thrombus is a very rare complication. We herein report the case of a 46-year-old man with BD who presented with a large right atrial thrombus. Within a month after surgical removal, the thrombus recurred and was successfully treated with immunosuppressants that included prednisolone and cyclophosphamide.     

A case of Behçet’s disease with various gastrointestinal findings

Behçet’s disease (BD) is a systemic inflammatory disorder involving the oral and genital mucosa, skin, eyes, gastrointestinal tract, joints, vasculature, and nervous. The gastrointestinal lesions accompanying with BD can extend along the full length of the tract, from the mouth to the anus, and show various clinical characteristics which is difficult to be differentiate from the other inflammatory bowel diseases including ulcerative colitis and Crohn’s disease. Hence, the differential diagnosis is difficult and can affect treatment outcomes. We report here, a BD case showing varied gastrointestinal lesions and endoscopic findings. The information, endoscopic findings, differential diagnosis, and treatment approach in this case might help physicians to distinguish from other diseases and to manage similar cases     

Clinical and pathological characteristics of patients with pulmonary inflammatory pseudotumors: An 18-year retrospective study of 31 cases

To investigate the clinical and pathological characteristics in patients with pulmonary inflammatory pseudotumors (PIP).This retrospective study included 31 patients with PIP from 2001 to 2019. Preoperative computed tomography scan was performed in all patients. Clinical and pathological characteristics were collected and analyzed.Thirty-one patients (16 female and 15 male) were recruited, with a median age of 57 years (range, 11–72 years). Eight (25.8%) patients were asymptomatic, and the others had symptoms characterized by cough with sputum, chest and back pain, dry cough, fever and blood in sputum, or hemoptysis. All cases were single lesions, including 23 cases in the right lung, and 8 cases in the left lung. Computed tomography scan demonstrated irregular lobulated nodules or masses in 14 patients, and regular round or oval nodules or masses in 11 cases. The blurred edge of tumors and spiculation was found in 12 cases. Microscopic results were characterized by the collection of inflammatory mesenchymal cells. Immunohistochemical examination showed vimentin, smooth muscle actin, and anaplastic lymphoma kinase positive. Complete tumor resection was obtained in all cases. No recurrence or metastasis was observed during the follow-up period.PIP has a variety of manifestations. Preoperative diagnosis is difficult to reach. The final diagnosis still depends on the pathological and immunohistochemical examination. Complete surgical resection is the main treatment at present, and the overall prognosis is good.     

Single or multiple perforations with varying locations as a complication of intestinal Behçet's disease: Report of three cases

Intestinal ulcers in Behçet's disease (BD) tend to cause perforation with significant morbidity. The optimal surgical procedure in such cases is controversial and the postoperative period can be eventful with an unpredictable course. We report three cases of perforations with varying locations in three patients with long-standing Behçet's disease. Two patients required two and one patient required four operations. It is emphasized that the clinician must be alert in a patient with BD when abdominal symptoms accompany the clinical picture. As soon as the diagnosis is reached, surgical intervention with limited resection must be performed. Endoscopic examination and careful medication play major roles in the follow-up.     

Behçet’s disease and Chiari’s network

Behçet’s disease (BD) is a chronic, multisystemic, inflammatory process of uncertain origin. Diagnosis criteria consist of recurrent oral ulceration plus two of the following: genital ulceration, ocular and skin lesions, and positive pathergy test. Pulmonary embolism and, especially intracardiac thrombi, are very rare. We report the case of a patient with BD presenting with bilateral pulmonary embolism and intracardiac mass in right atrium.     

Left atrial myxoma: excision guided by transesophageal cross-sectional echocardiography

We present a patient with a left atrial myxoma in whom the intraoperative use of transesophageal cross-sectional echocardiography proved helpful in selecting the most appropriate surgical approach, thus allowing expeditious and complete excision of the tumor. This technique, which permits continuous monitoring of cardiac function throughout open heart procedures, appears particularly valuable in patients with intracardiac masses.     

Intra-abdominal inflammatory myofibroblastic tumor: Spontaneous regression

Inflammatory myofibroblastic tumors are usually treated by surgical resection. We herein report two cases of intra-abdominal inflammatory myofibroblastic tumors that were unresectable and underwent spontaneous regression without any treatment. Our case report and literature review show that regression is more common in the middle-aged and older male populations. Abdominal discomfort and fever were the most common symptoms, but the majority of patients had no obvious physical signs. There was no specific indicator for diagnosis. The majority of the lesions regressed within 3 mo and nearly all of the masses completely resolved within 1 year. We conclude that the clinical characteristics of inflammatory myofibroblastic tumors are variable and, accordingly, the disease needs to be subdivided and treated on an individual basis. Surgery is always the first-line treatment; however, for those masses assessed as unresectable, conservative therapy with intense follow-up should be considered.     

Cardiac pseudotumor revealing Behçet's disease

INTRODUCTION: Cardiac thrombosis is a rare complication of Beh?et's disease (BD), which may present as a cardiac tumor. Its discovery precedes, in half of the cases, the diagnosis of BD. The high mortality may be associated to postsurgical complications and/or an associated involvement of pulmonary arteries. CASE REPORT: We present the case of a 31 years old Caucasian French woman, with a history of venous thromboembolic disease, who had surgery after the discovery of a right ventricle tumor. That was an organised thrombus with endomyocardial fibrosis and a diagnosis of Beh?et's disease was made after the surgery. The outcome was favourable under medical treatment associating corticosteroids, colchicine and antivitamin K (AVK), without relapse four years later. CONCLUSION: The discovery of an intracardiac mass in a young patient must evoke the diagnosis of cardiac thrombus and Beh?et's disease, even in the absence of predisposing ethnic or geographic factor.     

Diaphragmatic hernia mimicking an atrial mass: a two-dimensional echocardiographic pitfall

Two-dimensional echocardiography has become the diagnostic method of choice for identifying intracardiac masses. However, adjacent extracardiac structures may closely mimic intracardiac masses on the two-dimensional echocardiogram. Five cases of a previously unrecognized phenomenon in which a diaphragmatic hernia mimicked an intraatrial mass are reported. Techniques to identify a diaphragmatic hernia properly on two-dimensional echocardiographic examination and distinguish it from intracardiac masses are discussed.     

Two-dimensional echocardiographic demonstration of left atrial thrombi in patients with prosthetic mitral valves.

Although M-mode echocardiography (MME) is not a reliable method for detecting left atrial thrombi, recent reports suggest that two-dimensional echo (2DE) may be more effective than MME in identifying intracardiac thrombi. In three patients with prosthetic mitral valves who presented with either arterial embolization or prosthetic valvular dysfunction, 2DE demonstrated left atrial masses consistent with thrombi, while MME was either negative (two patients) or suspicious (one patient) for left atrial thrombus. Thrombi were documented by surgical or postmortem examination in all cases. Clear delineation of the atrial cavity and the margins of the masses, visualization on multiple echocardiographic views and comparison of serial examinations were helpful in identifying these masses as thrombi. In addition, the masses visualized had certain patterns of motion which seem unique and may allow characterization of atrial masses as thrombi.     

Kimura’s disease—an unusual cause of lymphadenopathy in children

Kimura’s disease is a rare inflammatory disorder of unknown cause primarily seen in young Asian males. The disease is characterized by a triad of painless subcutaneous masses in the head or neck region, blood and tissue eosinophilia, and markedly elevated serum immunoglobulin E levels. We describe a 3-year-old Indian boy with Kimura’s disease who presented with generalized lymphadenopathy. The diagnosis was based on the characteristic histopathologic findings after surgical excision in conjunction with peripheral eosinophilia and elevated serum immunoglobulin E levels.     

Clinical presentation and non-invasive diagnosis of right heart masses.

Right-sided cardiac masses do not have a uniform clinical presentation. Successful diagnosis is dependent on a high index of suspicion and appropriate echocardiographic examination. Five cases of right sided intracardiac masses have been detected during the period that echocardiography has been routinely available to us--two of right atrial myxoma, one of right atrial thrombus, and two of right ventricular tumour. M-mode echocardiography identified four of the five cases. Two-dimensional echocardiography was necessary to establish the non-invasive diagnosis in the fifth case. Echocardiography should be used in any patient in whom a right sided mass is suspected, or in those patients presenting with signs of tricuspid regurgitation, tricuspid stenosis, pulmonary stenosis, cyanotic heart disease, progressive right heart failure, constrictive pericarditis, or pulmonary emboli without obvious source.