Los tumores cardiacos (parte de las masas cardiacas). Experiencia monocentro (Parte II) / The heart tumors (part of the cardiac masses). Single centre study (Part II)

Introducción. Las masas cardiacas son una patología poco frecuente, entre las que destacan los tumores cardiacos (TC), que sonen su conjunto los más frecuentes entre ellas. La mayoría de TC es metastático. Entre los TC primitivos, el 75% es benigno. Material y métodos. Se analiza la experiencia con los TC del Hospital Universitario de Canarias (HUC) en Tenerife, a lo largo de 37años y casi 8,000 intervenciones con circulación extracorpórea (CEC), más algunos casos previamente comunicados por el primerautor. Los TC más frecuentes intervenidos han sido los mixomas. Se analiza retrospectivamente esta serie utilizando el programade análisis estadístico SPSS Statistics v20. Resultados. Se operaron 83 mixomas desde marzo1984 (inicio del programa de CEC)hasta diciembre de 2021 (1-6 cada año; 2,13/año). Edad 34-62 años, media 48,73. Varones 33(39,86%). Mujeres 50(60,24%).De ellos, 77 fueron en aurícula izquierda (75 en septo). Tres en aurícula derecha. Otros 3 en septo interauricular con crecimientoen ambas aurículas). Ninguno en ventrículos. Se siguieron 76 pacientes (96,1%). Hubo una muerte intrahospitalaria (1,2%) y dosen el seguimiento por causa no relacionada. En la curva de seguimiento de Kaplan-Meier, el 88% puede llegar a los 36 meses deseguimiento (95% de IC). Se repasan también algunos tumores menos frecuentes. Conclusiones. Aunque los TC benignos lo seanhistológicamente, clínicamente pueden no serlo si causan mortalidad o daño grave. El pequeño riesgo quirúrgico justifica la intervención ante el riesgo de no hacerlo. (AU)

Introduction. Cardiac masses are a rare pathology, among which cardiac tumors (CT) stand out, which are the most frequentamong them. Most tumors are metastatic. Among primitives, 75% are benign. Material and methods. The experience with CT atthe Hospital Universitario de Canarias (HUC) in Tenerife, over 37 years and almost 8,000 extracorporeal interventions, plus somecases previously reported by the first author, are reviewed. The most frequent CTs operated on have been myxomas. This series isretrospectively analyzed using the statistical analysis program SPSS Statistics v20. Results. 83 myxomas were operated on, fromMarch 1984 (beginning of the extracorporeal surgery program) to December 2021 (1-6 each year; 2.13/year). Age 34-62 years,mean 48.73. Men 33 (39.86%). Women 50 (60.24%). Of these, 77 were in the left atrium (75 in the septum). Three in the rightatrium. Another 3 in the interatrial septum with growth in both atria). 76 patients (96.1%) were followed up. There was one in-hospital death (1.2%) and two in follow-up due to unrelated causes. In the Kaplan-Meier follow-up curve, 88% can reach 36 monthsof follow-up (95% CI). Some less frequent tumors are also reviewed. Conclusions. Although benign CTs are histologically benign,they may not be clinically so if they cause mortality or serious damage. The small surgical risk justifies the intervention given therisk of not doing it. (AU)

Vascular tangle in the inter-atrial septum - Is it the source of cardiac myxoma?

BACKGROUND: Cardiac myxomas (CMs) are the most common primary tumors of the heart, said to be derived from pluripotent cardiac stem cells. They are most often attached to the left side of the inter-atrial septum (IAS) and a feature noted at the site of attachment is a conglomeration of thick-walled vessels that has been noted to precede the development of myxomas. AIMS: The present study was conducted to compare histology of the normal inter-atrial septa to the septal flap excised with the myxomas and to evaluate the significance of this 'vascular tangle' in the histogenesis of these tumors. MATERIALS AND METHODS: In a 10-year retrospective analysis of all surgically excised CMs, tumors with attached septal sleeves were selected. This histology was compared to the serial sections of 25 normal age-matched septa obtained from normal hearts at autopsy. RESULTS: Of the 56 myxomas seen in 10 years, 38 tumors (all left atrial in location) were received with a flap of the IAS. All of these cases, irrespective of the sizes of the tumor, showed the presence of conglomeration of thick-walled blood vessels, many of which showed abrupt myxoid change in their walls. Another noteworthy feature in many vessels in all flaps was migration of the myxoid tissue through the vascular walls and the endocardium to mushroom out into the atrial lumen. Such vascular channels were not seen in any of the normal IAS. CONCLUSIONS: Based on these findings, we propose a two-step hypothesis: an initial step that stimulates the pluripotent cells toward vasculogenesis with endothelial and smooth muscle differentiation, and a subsequent step leads to production of abundant mucopolysaccharides that splay apart the smooth muscle cells, which would explain rings, cords or nests of myxoma cells around endothelial lined spaces.

[Infiltration of Cardiac Vessels by Lung Cancer: Incidence, Classification, Operative Technique with Heart Lung Bypass, and Results]. / Infiltration der herznahen Gefäße durch Lungenkarzinome: Inzidenz, Klassifikationen, operative Technik unter Einsatz der Herz-Lungen-Maschine und Ergebnisse.

Carcinomas of the lung that infiltrate the blood vessels close to the heart (left atrium, pulmonary artery and aorta) without spreading to mediastinal lymph nodes or developing distant metastases are rare overall. Such situations are often classified as primarily inoperable by interdisciplinary tumour boards. This is only the case if, for technical reasons, an experienced thoracic surgeon does not feel able to perform a resection with a surrounding margin of healthy tissue. The surgical strategy to be employed must be chosen individually depending on the infiltrated structure. Complete tumour staging should always be carried out. This also helps in deciding whether neoadjuvant chemotherapy should be given before resection. A heart-lung machine must always be used if larger defects occur due to the resection of blood vessels close to the heart. Using a heart-lung machine in the case of tumour resection does not lead to problems of tumour cell dissemination. Nevertheless, the duration of use of the heart-lung machine should be kept to a minimum, also because of the anticoagulation required. The cardiac defects can be closed securely with the bovine patching materials that are now available. Postoperative morbidity and mortality are low after such resections. Curative resection of blood vessels close to the heart infiltrated by carcinomas of the lung can lead to 5-year survival rates of up to 50 %.

Cardiac Tumors: Clinical Perspective and Therapeutic Considerations.

BACKGROUND: Cardiac tumors rare cardiac disorders with an overall incidence rate < 0.33%. Cardiac tumors can be classified as primary or secondary depending on the origins of tumors. Primary cardiac tumors (5% incidence) are rare compared with the secondary (95%, metastases of the heart) cardiac tumors. OBJECTIVE: Given that cardiac tumors exhibit some nonspecific symptoms compared with other heart diseases, clinical diagnosis of cardiac tumors is rather challenging. Thus we will try to review the classification and pathogenesis of cardiac tumors. CONCLUSION: Current evidence revealed that 75% of cardiac tumors are considered benign (myxoma, fibromas, lipomas, rhabdomyomas, hemangiomas, teratomas, papillary fibroelastomas, pericardial cysts or cystic tumor of atrioventricular node). Clinical differential diagnosis of cardiac tumors is mainly based on imaging techniques including transthoracic and transesophageal echocardiograms, computed tomography (CT) scans and magnetic resonance imaging (MRIs). This mini-review tries to summarize recent understanding of the pathogenesis and therapeutics of cardiac tumors.

[Solid lesions of heart valves].

AIM: To analyze clinical-diagnostic and surgical aspects of cardiac valve solid lesions as well as long-term results of surgical treatment. MATERIAL AND METHODS: Clinical, diagnostic and surgical aspects of treatment of 51 patients with tumorous and pseudotumorous lesions of cardiac valves were analyzed. RESULTS AND DISCUSSION: Hospital complications were observed in 17.7% of patients. Mortality rate was 3.9%. 1- and 28-year overall survival was 100% and 94.1% respectively. Quality of life was good, satisfactory and unsatisfactory in 73.5%, 20.4% and 6.1% of cases respectively. CONCLUSION: Timely diagnostics of tumorous and pseudotumorous lesions of cardiac valves is very difficult but possible based on short history, positional dependence of symptoms, embolic events without rhythm disturbances. Additional instrumental methods including TTE, TEE, CT and MRI are necessary to define diagnosis. Intraoperative diagnostics and immediate histological survey are useful to determine volume of surgery in each case. Successful surgical treatment implies urgency, hypothermic perfusion and pharmaco-cold cardioplegia during operation.

The 2015 WHO Classification of Tumors of the Heart and Pericardium.

This article reviews the nomenclature of benign and malignant neoplasm of the heart and pericardium in the 4th edition of the World Health Organization's Classification, with emphasis on differences since the 3rd edition of 2004. The tumours are divided into benign, malignant, and intermediate tumors of uncertain behavior, with separate sections on germ cell tumours and tumors of the pericardium. There are important updates in the sarcoma classification, with emphasis on the most common site, the left atrium. The importance of the new genetic finding in cardiac myxomas, namely somatic mutations in the PRKAR1A gene underscores the importance of this alteration in the pathogenesis of these tumors. Challenges on the classification of each entity are discussed.

Surgery for primary intrapericardial tumors in adults.

Primary intrapericardial tumors in adults, whether benign or malignant are rare. Surgical treatment for these tumors are reviewed together with their incidence, classification, clinical features, and diagnosis.

Clinical classification of cardiovascular tumors and tumor-like lesions, and its incidences.

Tumors of the heart and the great vessels are very rare disease, and there are many disorders such as tumors originated from the heart and great vessels, metastatic tumors, and tumor-like lesions which do not fit into the usual concept of tumor or neoplasm; thus, it is very difficult to classify these tumors. We proposed a new classification of cardiovascular tumors for clinical use based on the accumulated biological analyses and clinical data of the reported literatures and our own study as benign tumors, malignant tumors, ectopic hyperplasia/ectopic tumors/others, and tumors of great vessels, with reference to the series of Atlas of tumor pathology of the Armed Forces Institute of Pathology and the recent World Health Organization classification of cardiac tumors issued in 2004. More than 50 disorders have been reported as tumors originated from the cardiovascular system, and various metastatic tumors from nearby organs, distant lesions, and intravascular extension tumors to the heart were reported. Based on the new classification, we reviewed epidemiology and incidence of cardiovascular tumors. Metastatic tumors are more frequent than tumors originated from the heart and great vessels, and cardiac myxoma is the most frequent tumors in all cardiac tumors.

Primary and metastatic cardiac sarcomas: a 12-year experience at a German heart center.

BACKGROUND: Primary and metastatic cardiac sarcomas represent a heterogeneous group of rare neoplasms with varying clinical course and diverse histogenetic line of differentiation. To date, there exist no uniform guidelines for their surgical and/or oncological treatment. METHODS: We evaluated retrospectively all patients undergoing cardiac surgery for primary or secondary cardiac sarcoma in the period 1999-2011 at the Erlangen Heart Centre to analyze their clinicopathological spectrum, treatment and outcome. RESULTS: Five patients (3 women & 2 men; mean age: 46 years; range: 28-81 years) had primary cardiac sarcomas (6.7% of primary cardiac tumors) and 4 had cardiac metastasis from soft tissue sarcoma (1 case each of osteosarcoma, myxoid liposarcoma, alveolar soft part sarcoma and pleomorphic spindle cell sarcoma). Primary sarcomas were located in the left atrium (n=3), left ventricle (n=1) and right atrium (n=1). Histological types were myxosarcoma (3), pleomorphic undifferentiated sarcoma (1) and angiosarcoma (1). Four patients died at 2-64 months (mean, 24.5 months). Sarcoma metastasis to the heart developed at a mean of 109.5 months from initial diagnosis (range, 5-240 months). Three of them died of disease at a mean of 14 months after cardiac surgery and one is disease free 34 months after heart transplantation for metastasis. CONCLUSIONS: Primary and metastatic cardiac sarcomas are very heterogeneous in their histological appearance, clinical presentation and course of the disease. Radical surgery combined with chemoradiation is promising in patients with resectable disease and may significantly prolong survival. Cardiac transplantation represents an emerging strategy for patients with isolated unresectable cardiac involvement.

Multidetector CT of the heart: spectrum of benign and malignant cardiac masses.

Recent innovations in multidetector 3D CT have improved the spatial and temporal resolution of cardiac computed tomography (CT), which results in greater frequency of mass lesion identification and provides a new opportunity for more precise characterization. Although cardiac masses are rare entities, patients with cardiac masses may present with acute symptoms and may be encountered by emergency radiologists. The objectives of this article are to review the CT appearance of cardiac masses and the role of volumetric MDCT with 3D rendering to define characteristic features and provide a practical approach to formulating a differential diagnosis.

Right heart sarcomas.

Primary cardiac tumors are unusual, and primary cardiac sarcomas constitute a rare subset of these. In cardiac sarcoma, unlike many malignancies, the histologic cell type does appear to affect the treatment options or prognosis in a significant way. The presenting symptoms, treatment options and, indeed, prognosis are largely controlled by the tumor's anatomic location. We have proposed a classification system based on anatomic location that divides cardiac sarcoma into left heart, right heart and pulmonary artery sarcomas. In our experience, right heart sarcoma tends to be bulky, grow in a more exophitic manner, be more infiltrative, and metastasize earlier than left heart or pulmonary artery sarcoma. Right heart sarcoma also presents less often in congestive heart failure or with compromised hemodynamic status than left heart and pulmonary artery sarcoma, which are usually highly symptomatic at presentation. The prognosis for right heart sarcoma without surgery is dismal. Complete surgical resection remains the goal of therapy and the only treatment modality shown to increase survival. Complete surgical resection is complicated both by the bulky infiltrative nature of right heart sarcoma and the high incidence of metastatic disease at presentation. The current approach of our cardiac sarcoma group to right heart sarcoma has been to begin neoadjuvant chemotherapy once a definitive tissue diagnosis of sarcoma is achieved. After 4 to 6 rounds of chemotherapy, the patient is considered for surgical resection. This standardized treatment has been approved in our IRB protocol: a clinical trial to assess the safety and Efficacy of a novel radical tumor reSection Procedure used in conjunction with nEoadjuvant chemotheRapy to treat malignant primary right heart cardiac tumOrs - the ESPERO trial. This protocol is designed to compare our existing 24 index cases of surgical resection of right heart sarcoma using a nonstandardized treatment plan, with routine neoadjuvant chemotherapy, and a standardized treatment plan to see if the rate of microscopically complete resection can be improved from its current level of 33% and if this will improve patient survival. In this reveiw, we will discuss the experience with right heart sarcoma.

Left heart sarcomas.

Primary tumors of the heart are uncommon, with roughly 75% benign and 25% malignant. Most of the malignant tumors are sarcomas and historically have had a very poor prognosis. These tumors tend to occur in young patients with a mean age of 40 years. Making a diagnosis of cardiac sarcoma can be difficult due to its rarity and the nature of the symptoms. For left heart sarcomas, almost all patients are symptomatic by the time the diagnosis has been made. Symptoms are dependent on the location and the extent of the tumor and are not related to tumor histology; similarly, histologic cell type has not been found to be related to prognosis in most studies. We therefore previously proposed a classification system for primary cardiac sarcoma based on anatomic location, dividing primary cardiac sarcoma into right heart, pulmonary artery and left heart sarcomas. Left heart sarcoma presents a technical anatomic challenge: the left atrium, being the posterior heart chamber, allows somewhat limited access using routine surgical approaches. The role of chemotherapy or radiotherapy remains unclear and unproven, leaving complete surgical resection as the only mode of therapy with a proven survival benefit. Our review of the published literature showed frequent local recurrence and poor long-term survival in left heart sarcomas. Our hypothesis was that the left atrium had limited anatomic accessibility for large complex resections and reconstructions, and this led cardiac surgeons to do a more limited tumor removal with an increased chance of local recurrence and a detrimental effect on survival. To address this technical challenge, our group introduced the surgical technique of cardiac explantation, ex vivo tumor resection, cardiac reconstruction, and subsequent cardiac reimplantation or cardiac autotransplantation for left heart sarcoma in an attempt to improve the completeness of local resection, decrease local recurrence, and extend patient survival. This review discusses the approach of the cardiac sarcoma group at the Methodist DeBakey Heart & Vascular Center and the MD Anderson Cancer Center to the diagnosis and treatment of left heart sarcoma, as well as our current patient outcomes.

The classification and surgical strategy of intracardiac leiomyomatosis.

BACKGROUND: There is a great deal of heterogeneity in the surgical strategy to treat intracardiac leiomyomatosis (ICL), leading to a need to create a theoretical tool to clarify this situation. METHODS: The data of 14 cases of ICL surgically treated in Anzhen Hospital from February 1995 to February 2009 were retrospectively reviewed. A system for classifying ICL was proposed based on four features of the lesion: size of intracardiac component; extent of inferior vena cava (IVC) involvement; venous pathway from uterus to IVC; and laterality of the lesion in the pelvis. The 14 cases of ICL were treated through multiple surgical strategies. RESULTS: There were no operative deaths. The follow-up was 73.1 +/- 59.2 months and one patient died from recurrence due to incomplete excision 5 months after the primary procedure. The 5-year survival rate calculated through Kaplan-Meier survival curve was 93.16 +/- 4.98%. Of the surviving patients, 13 had ICL, 10 were in the New York Heart Association (NYHA) class I, and three were in NYHA class II. CONCLUSION: The surgical treatment of ICL can obtain a good mid- to long-term survival rate and satisfactory heart function, and the proposed classification system for ICL may be helpful to guide the selection of the surgical strategy for ICL, and may serve as the future basis for standardising the reporting of ICL management.

[The anatomic pathological classification for intracardiac leiomyomatosis and the choice of its surgical strategy].

OBJECTIVE: To approach an anatomic pathological classification scheme for the intracardiac leiomyomatosis (ICL), and to guide the choice of the surgical strategy for ICL treatment. METHODS: Retrospectively reviews the data of 13 cases of ICL from February 1995 to March 2007 in Anzhen Hospital. They were surgically treated under the CPB with different surgical strategy, and a quaternary classification scheme was used to classify them according to their anatomic pathological features of ICL. RESULTS: There was no operative death. One patient with YBOR type of ICL died from recurrence due to the incomplete excision 5 months after the primary operation. The 5 yrs survival rate calculated by the Kaplan-Meier survival curve was 93% +/- 5%. Of the surviving 12 patients, 9 were in NYHA functional class I, and 3 in class II. CONCLUSION: The surgical treatment of ICL can get a good mid-long term survival rate and living quality, and a quaternary classification scheme for ICL can be used to guide the choosing of surgical strategy and to understand the symptoms of ICL.

Classification and segmentation of intracardiac masses in cardiac tumor echocardiograms.

This paper describes an automatic method for classification and segmentation of different intracardiac masses in tumor echocardiograms. Identification of mass type is highly desirable, since to different treatment options for cardiac tumors (surgical resection) and thrombi (effective anticoagulant treatment) are possible. Correct diagnosis of the character of intracardiac mass in a living patient is a true challenge for a cardiologist; therefore, an objective image analysis method may be useful in heart diseases diagnosis. Image texture analysis is used to distinguish various types of masses. The presented methods assume that image texture encodes important histological features of masses and, therefore, texture numerical parameters enable the discrimination and segmentation of a mass. The recently developed technique based on the network of synchronized oscillators is proposed for the image segmentation. This technique is based on a 'temporary correlation' theory, which attempts to explain scene recognition as it would be performed by a human brain. This theory assumes that different groups of neural cells encode different properties of homogeneous image regions (e.g. shape, color, texture). Monitoring of temporal activity of cell groups leads to scene segmentation. A network of synchronized oscillators was successfully used for segmentation of Brodatz textures and medical textured images. The advantage of this network is its ability to detect texture boundaries. It can be also manufactured as a VLSI chip, for a very fast image segmentation. The accuracy of locating of analyzed tissues in the image should be assessed to evaluate a segmentation technique. The new evaluation method based on measurement of physical textured test objects was proposed. Firstly, a series of object images was obtained by the use of different devices (scanner, digital camera and TV camera). Secondly, the images were segmented using oscillator network and feedforward artificial neural network. Thirdly, geometrical test object parameters were estimated and compared to its true values. The experiment was repeated also for ultrasound images, which represented rectangular cross-section of synthetic sponge submerged in water. In addition, classification and segmentation of selected benign tumor echocardiograms were performed. Oscillator network was used with network weights defined for both whole texture region and texture boundary detection for the tumor segmentation. The latter method provides much faster segmentation with the similar accuracy. The obtained segmentation results were discussed and compared to the artificial neural network classifier. Finally, it was demonstrated that the network of synchronized oscillators is a reliable tool for the segmentation of the selected intracardiac masses, since it gives a relatively accurate location of analyzed tissues. The advantage of the proposed method is its resistance to changes of the visual information in the analyzed image and to noise and artifacts, often present in echocardiograms.

[Cardiac tumors]. / I tumori cardiaci.

Primary cardiac tumors are rare and their subdivision often difficult because of their unknown origin. In the most recent classification, cardiac tumors are divided into benign (about 75% and malignant neoplasms in relationship to their tissue differentiation (rhabdomyoma, haemangioma, etc.) or uncertain aetiology (myxoma, papillary fibroelastoma). Primary malignant tumors are maimly represented by sarcomas. The most frequent tumor is cardiac myxoma, which by itself represents about 50% of all primary cardiac neoplasms. Although non-invasive technologies as trans-esophageal ecocardiography allow the detection and exact localization of cardiac mass, clinical diagnosis is often tardive. This is due, besides the intrinsic rarity, to two main factors: first, the tumor is often asymptomatic (incidental autopic finding) or; alternatively, it may show aspecif symptoms mimicking heart failure or other pathologies. In this article, clinicopathological features of main primary cardiac tumors are presented. Investigation of the histogenesis of some of these neoplasms is still a primary field of research.

Usefulness of magnetic resonance imaging of cardiac and paracardiac masses.

In this study, magnetic resonance imaging was compared with histology to evaluate the usefulness of magnetic resonance imaging to distinguish malignant from benign cardiac and paracardiac masses in 55 patients. Tumor location, tissue composition, and pericardial or pleural effusion all were identified as key predictors of lesion type. Observers were accurate in the prediction of lesion type (area under curve 0.88 and 0.92), and there was good interobserver variability (Cohen's kappa = 0.64).