Linfoma cardiaco primario / Primary cardiac lymphoma

Los tumores cardíacos primarios son raros. Tienen una incidencia que varía entre 1,38 y 30 por 100.000 personas al año, un aproximado de 75-80 por ciento son benignos. El linfoma cardíaco primario representa el 1,3 por ciento de los tumores cardíacos primarios y el 0,5 por ciento de los linfomas extranodales. Se define como un linfoma no Hodgkin (LNH) que afecta solo al corazón y/o el pericardio. Su complejo sintomático puede incluir disnea, dolor torácico, fatiga progresiva, sudoración nocturna, pérdida ponderal, arritmias, síndrome de vena cava superior y un aproximado del 20 por ciento de los pacientes pueden desarrollar insuficiencia cardíaca aguda como primera manifestación. Sin embargo, la mayoría de ellos cursan con sintomatología inespecífica y son detectados de manera incidental. El linfoma no Hodgkin difuso de células B grandes es la variante histológica más frecuente. Presentamos un caso que inició con síntomas de insuficiencia cardiaca derecha, evolucionó desfavorable hasta su fallecimiento y se realizó el diagnóstico en la autopsia(AU)

Primary cardiac tumors are rare. They have an incidence that varies between 1.38 and 30 per 100,000 people per year, 75-80 percent are benign, approximately. Primary cardiac lymphoma represents 1.3 percent of primary cardiac tumors and 0.5 percent of extranodal lymphomas. It is defined as a non-Hodgkin's lymphoma (NHL) affecting only the heart and/or the pericardium. Its symptoms may include dyspnea, chest pain, progressive fatigue, night sweats, weight loss, arrhythmias, and superior vena cava syndrome. Approximately 20 percent of patients may develop acute heart failure as the first manifestation. However, most of them have nonspecific symptoms and are detected incidentally. Diffuse large B-cell non-Hodgkin's lymphoma is the most frequent histological variant. We report a case that began with symptoms of right heart failure, progressed unfavorably until death, and the diagnosis was made at autopsy(AU)

Tumores cardiacos fetales: diagnóstico ecográfico, evolución y tratamiento / Fetal cardiac tumors: diagnosis, clinical course and management

Objetivos: Analizar el diagnóstico ecográfico, la evolución y el tratamiento de los tumores cardiacos fetales diagnosticados en el Hospital La Paz de Madrid, entre los años 1995 y 2010. Método: Se realizó un estudio retrospectivo descriptivo recogiendo los principales datos ecográficos, de la historia clínica materna, neonatal y pediátrica. Resultados: En el periodo de estudio se diagnosticaron ecográficamente 28 tumores cardiacos fetales, 21 casos fueron catalogados como rabdomiomas. Once de ellos tuvieron una regresión prácticamente completa, con ocho casos diagnosticados hasta el momento de esclerosis tuberosa. Hubo 2 muertes intraútero, 1 interrupción legal del embarazo y 4 diagnósticos ecográficos de rabdomiomas, no se confirmaron al nacimiento. Se diagnosticaron prenatalmente dos fibromas; de ellos una gestante optó por la interrupción legal del embarazo y en el otro caso se produjo la muerte neonatal. Dos neonatos fueron sometidos a cirugía con una resección completa del tumor, con resultado anatomopatológico de heman-gioma capilar en uno y teratoma en el otro. En un caso se realizó una biopsia que confirmó la presencia de un hemangiopericitoma auricular que se redujo posteriormente con quimioterapia. Conclusiones: Los tumores cardiacos son una patología poco frecuente. La mayor parte de ellos son rabdomiomas, cuya sin-tomatología y evolución depende de su localización. Estos suelen regresar espontáneamente, pero pueden asociarse al diagnóstico de esclerosis tuberosa, lo que empeora su pronóstico.

Aims: To analize the diagnosis, clinical course and management of fetal cardiac tumors diagnosed at La Paz Hospital (Madrid) between 1995 and 2010. Methods: We performed a retrospective descriptive study collecting the main ultrasound dates of the maternal, newborn and pediatric history. Results: During the study period, 28 fetal cardiac tumors were dignosed. Rhabdomyomas were diagnosed in 21 fetuses; 11 rhabdom-yomas returned almost completely. Eight of them were diagnosed of tuberous sclerosis up to the moment. Other two cases died in utero. One pregnant decided to be practised a miscarriage and four rhabdomyomas which were diagnosed by ultrasound, were not found in the newborns. Two fibroms were diagnosed by ultrasound; one of the pregnant woman decided to be practised a miscarriage and the other fetus died when he was born. Two newborns were operated, with the pathological anatomy result of a hemangioma and a teratoma. A biopsy was made that confirmed the presence of an atrial hemangiopericitoma which was treated by quimiotherapy. Conclusion: Fetal cardiac tumors are a rare disease. Most of them are rhabdomyomas which syntomatology and clinical course depend on its location. They usually regret spontaneously, but they can be associated with tuberous sclerosis, and this aggravates their prognosis.

Malignant primary fibrous histiocytoma of the right ventricle

We report a rare case of primary cardiac malignant fibroushistiocytoma. A 65-year-oldmanpresentedwithepigastric tenderness. Echocardiography detected large pericardial effusion and right ventricular mass 5 x 4 cm. Tumor biopsy revealed malignant fibroushistiocytoma. Thorough investigation did not detect any extra-cardiac primary source or metastasis. Tumor resection was offered to the patient but he refused. Despite refusal the patient was still alive one year after diagnosis

Comportamiento clínico de los tumores cardíacos desde el feto hasta el adulto: serie multicéntrica de 38 pacientes / Cardiac tumors in children and adults: A retrospective study

Background: Cardiac tumors are very uncommon at all ages. There are important clinical differences between children and adults in the behavior of these tumors. Aim: To compare the behavior of primary and secondary cardiac tumors, from fetal age to adults. Patients and Method: Multicentric retrospective analysis of 38 children and adults with cardiac tumors, evaluated with echocardiography between January 1995 and August 2001. Medical records, echocardiographic and radiological examinations, surgical protocols and pathologic examinations were reviewed. Follow-up was obtained through data on medical records or calling patients by telephone. Results: Tumors were diagnosed in 38 patients (13 children and 25 adults), from a total of 31.800 echocardiograms. In children the diagnosis was made by fetal, transthoracic or transesophageal echocardiography in 23.6 percent and 8 percent of cases, respectively. Eighty five percent were primary (10 benign and 1 malignant) and 15 percent, secondary tumors. Fifty four percent were rhabdomyomas and 75 percent regressed spontaneously. Seventy seven percent were symptomatic and 31 percent were treated with surgery. During a follow up of 44±35 months, 31 percent of patients died. In adults, 76 percent of tumors were diagnosed by transthoracic and 20 percent by transesophageal echocardiography. Seventy six percent were primary (18 benign and 1 malignant) and 24 percent secondary tumors. Fifty six percent were myxomas. Ninety two percent were symptomatic and 84 percent were treated surgically. Twenty percent of patients died in the early postoperative period. No adult patients had a follow-up. Conclusions: Rhabdomyomas were solely found in children. In adults, myxomas are the predominat cardiac tumors. Primary and metastasic malignant tumors are observed both in children and in adults.

Tumores cardíacos malignos / Malignant cardiac tumours

Os tumores do sistema cardiovascular são afecções raras. Manifestam-se por sinais e sintomas inespecíficos, na maioria das vezes. O tratamento de alterações de ritmo cardíaco, das doenças que acarretam disfunção no sistema de condução Hiss-Purkinje e das síndromes que podem acometer o coração (isquêmica, particularmente) devem levar em consideração a possibilidade de neoplasias cardíacas. Os tumores do músculo cardíaco são os rabdomiossarcomas, que se desenvolvem com maior freqüência nas cavidades cardíacas esquerdas, principalmente no ventrículo esquerdo. Dentre os tumores endovasculares, o mais freqüente é o mixoma de átrio esquerdo e suas possíveis variantes. Os tumores metastáticos do coração, entre eles o melanoma maligno, são descritos com maior freqüência acometendo o ventrículo direito (parede anterior e septal, com invasão do ventrículo esquerdo), nas formas de doença não disseminada. O diagnóstico tardio implica em altas taxas de morbidade e mortalidade, associada ou não ao tratamento cirúrgico. Os autores descrevem caso de paciente com melanoma localizado no ventrículo direito, a abordagem diagnóstica e terapêutica realizada, comparando-as com os dados da literatura. A revisão da literatura é inconclusiva quanto ao tratamento de escolha dos tumores malignos do músculo cardíaco (primários ou metastáticos). Os autores sugerem a realização de estudos retrospectivos com metanálise na tentativa de estabelecer critérios diagnósticos e terapêuticos para os tumores malignos do coração, devido à pequena incidência desta doença.

Cardiac tumours: clinical, echocardiographic and pathological features

OBJETIVO: Caracterizaçäo clínica e ecocardiográfica dos pacientes com tumores cardíacos, avaliaçäo da terapêutica cirúrgica e da recidiva dos tumores. MÉTODO: Na primeira parte da pesquisa, o delineamento metodológico foi do tipo retrospectivo, através da identificaçäo de tumores cardíacos e coleta de dados dos prontuários do Hospital Universitário Walter Cantídio (UFCE) e Hospital de Messejana, no período de 1981 a 2001, onde foram encontrados 19 casos de tumores cardíacos. Os pacientes foram submetidos a uma reavaliaçäo clínica, eletro e ecocardiográfica durante o período de março a julho de 2002. RESULTADOS: Sintomas congestivos e dor torácica foram os sintomas mais freqüentes em nossa série. A localizaçäo preferencial dos tumores cardíacos em nossa série foi no átrio esquerdo. Entre os pacientes submetidos a operaçäo, todos tiveram o tumor ressecado dos átrios, predominando a localizaçäo em átrio esquerdo (79 por cento). O tipo histopatológico mais encontrado em nossa série foi o mixoma (78 por cento). A mortalidade cirúrgica foi de 14 por cento. Detectamos recidiva de tumor em um paciente. CONCLUSÕES: Tumores cardíacos foram mais encontrados no átrio esquerdo. Os tumores benignos foram mais freqüentes que os malignos. O tipo histopatológico mais encontrado foi o mixoma.

Surgical management of cardiac myxomas.

Cardiac myxomas are rare cardiac lesions, though they are the commonest tumours of the heart. Seventeen cases of cardiac myxomas have been operated during the last one decade. Exertional dyspnoea, palpitation and chest pain were the main presenting symptoms. Echocardiographic assessment was the only definitive diagnostic investigation required prior to surgery. Early surgical excision was planned in all the cases. Irrespective of the exposure techniques, removal of the tumour with wide excision of its base was practised. There was one early death due to low cardiac output in a patient brought in a shock like state. Follow up study has revealed 14 patients in NYHA class I and two patients are having class II symptoms. Periodic echocardiographic follow up study has not revealed any recurrence till date. It is concluded that an early diagnosis and surgery gives excellent long term results in these cases.

Fibroadenomas cardiacos en la infancia. Informe de un caso y revisión de la literatura / Cardiac fibroadenomas in childhood: Case report and review of literature

Se presenta un caso de fibroma cardíaco en una niña de cuatro meses con cuadro clínico final de choque cardiogénico. El diagnóstico se estableció en el estudio de autopsia. El tumor midió 4 X 6 cm. y se localizó en el septum interventricular con extensión amplia a ambos ventrículos, reduciendo la cavidad cardíaca en un 30% aproximadamente. Se analizan las características epidemiológicas, clínicas e histopatológicas de esta entidad