Angiomixoma agresivo pélvico: reporte de caso y revisión / Aggressive pelvic angiomyxoma: case report and revision / Angiomixoma agressivo: relato de caso e revisão

El angiomixoma agresivo pélvico es un tumor de partes blandas extremadamente raro, del cual se han reportado alrededor de 350 casos hasta la fecha. A continuación se reporta el caso clínico de una paciente que presentó dicho tumor y se describe el proceso diagnóstico y su tratamiento quirúrgico. Por otro lado, se realiza una breve revisión de la literatura disponible hasta el momento sobre el tema.

Aggressive pelvic angiomyxoma is an extremely rare soft tissue tumor, of which around 350 cases have been reported to date. This article aims at reporting the case of a patient presenting said tumor, as well as describing its diagnostic approach and its surgical treatment. On the other hand, it aims at briefly reviewing the available literature on the subject.

O angiomixoma pélvico agressivo é um tumor extremamente raro dos tecidos moles, tendo sido descritos até à data cerca de 350 casos. Relatamos o caso de uma doente que apresentava este tumor e descrevemos o processo de diagnóstico e tratamento cirúrgico. É também feita uma breve revisão da literatura disponível até à data sobre o assunto.

Síncope por mixoma auricular: a propósito de un caso clínico / Syncope due to atrial myxoma: presentation of a clinical case / Síncope por mixoma atrial: a propósito de um caso clínico

Las neoplasias cardíacas se dividen en tumores primarios y secundarios; estos últimos son 30 veces más frecuentes. La mayoría de las neoplasias cardíacas son benignas y dentro de este grupo destaca el mixoma auricular. La presentación clínica es muy variable en relación con su ubicación, tamaño y movilidad, y los hallazgos a nivel de las pruebas complementarias son inespecíficos. Dado que su historia natural muchas veces pasa inadvertida, el mixoma puede culminar en la obstrucción auriculoventricular, obstrucción del tracto de salida ventricular o incluso embolias sistémicas; de esta forma, es el causante de sintomatología severa como síncope, insuficiencia cardíaca y muerte súbita cardíaca. Su manifestación clínica como un síncope es bastante frecuente. Se expone el caso de un paciente de sexo masculino de 81 años que consultó en el servicio de urgencias por un síncope. Durante su valoración, se identificó el mixoma auricular como etiología de este.

Cardiac neoplasms are divided into primary and secondary tumors, the latter are 30 times more frequent. Most are benign and within this group the atrial myxoma stands out. The clinical presentation is very variable in relation to its location, size and mobility, and the findings in the complementary tests are nonspecific. Since its natural history often goes unnoticed, the myxoma can culminate in atrial ventricular obstruction, ventricular outflow tract obstruction or even systemic embolism, causing severe symptoms such as syncope, heart failure and sudden cardiac death. Its clinical manifestation as syncope is quite common. The case of an 81-year-old male patient who consulted at the emergency department for syncope is presented, in which the atrial myxoma was subsequently identified as the etiology of the event during the further evaluation.

As neoplasias cardíacas dividem-se em tumores primários e secundários, estes últimos são 30 vezes mais frequentes. A maioria são benignas e dentro deste grupo destaca-se o mixoma auricular. A apresentação clínica é muito variável em relação com sua localização, tamanho e mobilidade, e os achados a nível dos testes complementares são inespecíficos. Dado que a sua história natural muitas vezes passa despercebida, o mixoma pode culminar na obstrução aurículo-ventricular, obstrução do tracto de saída ventricular ou mesmo embolias sistémicas, sendo assim o causador de sintomatologia severa como síncope, insuficiência cardíaca e morte súbita cardíaca. Sua manifestação clínica como síncope é bastante comum. Apresenta-se o caso de um paciente de sexo masculino de 81 anos que consultou no serviço de urgências para um síncope. Durante a sua avaliação, se identificou mixoma auricular como etiologia deste

Fast growth rate of a right atrial myxoma

ABSTRACT Primary cardiac tumors are rare, with an incidence between 0.0017 and 0.19%, and are asymptomatic in up to 72% of cases. Approximately 75% of tumors are benign, and nearly 50% of these are myxomas. Concerning location, 75% of myxomas are in the left atrium, 15 to 20% in the right atrium, and more rarely in the ventricles. The finding of cardiac myxomas usually implies immediate surgical excision to prevent embolic events and sudden cardiac death. Reports with documented growth rate are rare, and the actual growth rate remains a controversial issue. We report the rapid growth rate of a right atrial myxoma in an oligosymptomatic 69-year-old patient, with negative previous echocardiographic history in the last two years, who refused surgery upon diagnosis, enabling monitoring of myxoma growth.

Ultrasonographic features of cutaneous myxomas in a patient with Carney complex

Abstract Carney complex is a rare genodermatosis characterized by cardiac and cutaneous myxomas, among other tumors. In the majority of cases, cutaneous myxomas precede the diagnosis of cardiac myxomas, which are the main cause of death in these patients. Despite the fact that the diagnosis of cutaneous myxomas is histopathological, high-frequency ultrasonography plays an essential role in the differential diagnosis with other cutaneous and subcutaneous tumors. The authors of the present study describe, for the first time in the literature, the ultrasonographic features of both variants of cutaneous myxomas, superficial and subcutaneous, in a patient with a Carney complex.

Multidisciplinary approach to right ventricular myxoma

Abstract Right ventricular (RV) myxoma that obstructs the RV outflow tract is rare. Multimodality imaging is crucial due to the curved and triangular shape of the RV anatomy. Incomplete resection by the right atrial approach in cardiac myxomas may be prevented by preoperative imaging with echocardiography, computed tomography and magnetic resonance imaging to provide detailed visualization. Right ventriculotomy may be an alternative approach to the isolated atrial approach to get complete resection of RV myxoma in suitable patients. The preferred surgical treatment is not well defined for ventricular myxomas and careful preoperative planning is essential. Surgical resection should be performed as soon as possible to avoid outflow tract obstruction, which might result in sudden death. The collaboration between cardiologist and heart surgeon and the effective use of imaging tools are essential for successful treatment. In this article, diagnosis and treatment and the heart team approach to RV myxoma are discussed with a demonstrative patient.

Mixoma auricular izquierdo gigante que causa obstrucción valvular mitral, hipertensión pulmonar y tromboembolia pulmonar / Giant atrial myxoma causing mitral valve obstruction, pulmonary hypertension and pulmonary thromboembolism

Resumen: Los tumores cardíacos pueden ser primarios o, más frecuentemente secundarios o metastásicos. Entre los tumores primarios es más frecuente el mixoma, cuya ubicación más común es en la aurícula izquierda. Las manifestaciones clínicas son diversas, producidas principalmente por obstrucción mecánica, embolizaciones, y manifestaciones constitucionales. Se comunica el caso de un paciente de 32 años, con cuadro clínico de insuficiencia cardíaca, hipertensión pulmonar severa y tromboembolismo pulmonar bilateral. Se hizo el diagnóstico de mixoma auricular izquierdo. Se resecó el tumor y se manejó la hipertensión pulmonar desde el ingreso al hospital con inhibidores de la fosfodiesterasa asociado a anticoagulación. Se discute el tema dando énfasis a aspectos fisiopatológicos involucrados tanto en la hipertensión pulmonar como en la presencia de tromboembolia pulmonar.

Abstract: Cardiac tumors may be primary or, more frequently secondary or associated to metastasis. Atril myxoma es the most frequent primary tumor, usually located in the left atrium. Clinical manifestations include those due to mitral valve occlusión, emboli and general non spedific symptoms and signs. Herein we report the clinical case of a 32 year old patient with severe pulmonary hypertension and bilateral pulmonary embolism. The tumor was extirpated, and he received phosphoro-diesterase inhiborts and anticoagulants. Subsequent clinical course was satisfactory. A brief discussion of this condicion is included.

Variabilidad de síntomas del mixoma cardíaco / Variability of Symptoms of Cardiac Myxoma

Introducción: De los tumores cardiacos primarios, más del 50 por ciento son mixomas. La variabilidad sintomática del mixoma cardiaco, puede llevar a confusiones diagnósticas. Objetivo: Presentar un caso donde se destaca la variabilidad de síntomas del mixoma cardíaco. Caso clínico: Paciente masculino de 51 años, atendido con manifestaciones de insuficiencia cardíaca, trastornos del ritmo cardíaco, micro embolias cerebrales, convulsiones tónico clónicas, hemoptisis, trastornos psiquiátricos, síndrome general con astenia, anorexia y pérdida de peso. Durante dos años, fue atendido en varias instituciones, con múltiples estudios y tratamientos. En el Servicio de Cardiología de Morón, Ciego de Ávila, mediante el estudio clínico y ecocardiográfico, se diagnosticó un tumor cardíaco. Se traslada al Cardiocentro de Santa Clara y allí se le extirpó el tumor. Histológicamente era un mixoma. Evolucionó muy bien, con excelente calidad de vida. Conclusiones: El mixoma cardíaco ocasiona síntomas obstructivos, embólicos y constitucionales, con cuadro clínico muy equívoco(AU)

Introduction: Of the primary cardiac tumors, more than 50 percent are myxomas. Symptomatic variability of cardiac myxoma can lead to diagnostic confusion. Objective: To present a case where the variability of cardiac myxoma symptoms is highlighted. Clinical case: A 51-year-old male patient, attended with manifestations of heart failure, heart rhythm disorders, micro cerebral embolisms, clonic tonic seizures, hemoptysis, psychiatric disorders, general syndrome with asthenia, anorexia and weight loss. For two years, he was treated in several institutions, with multiple analyses and treatments. In the Cardiology Department of Morón, Ciego de Ávila, through a clinical and echocardiographic study, a cardiac tumor was diagnosed. He moved to the Cardiocentro of Santa Clara and there, the tumor was removed. Histologically it was a myxoma. It evolved very well, with excellent quality of life. Conclusions: Cardiac myxoma causes obstructive, embolic and constitutional symptoms, with a very equivocal clinical picture(AU)

Tumores cardiacos primarios fetales: revisión de la literatura / Primary fetal cardiac tumors: review of the literature

Los tumores cardiacos primarios fetales representan una entidad de baja incidencia en la práctica clínica. La gran mayoría corresponde a lesiones benignas, siendo el rabdomioma el tipo más común. Si bien, es frecuente la regresión espontánea de este tipo de lesiones durante el embarazo, los tumores cardiacos fetales pueden asociarse a complicaciones como obstrucción del flujo cardíaco, insuficiencia valvular, arritmias, insuficiencia cardíaca e hidrops fetal, pudiendo conducir incluso a la muerte fetal. El mayor desarrollo de técnicas imagenológicas ha permitido un aumento en el número y precisión de los diagnósticos prenatales de tumores cardiacos, generando al mismo tiempo, nuevos desafíos y alternativas en relación al abordaje terapéutico. El objetivo del presente artículo de revisión es exponer la evidencia actual en relación al diagnóstico prenatal, manejo, complicaciones y condiciones asociadas de los tumores cardiacos fetales más frecuentes.

Fetal primary cardiac tumors represent a low incidence entity in clinical practice. The vast majority corresponds to benign lesions, with rhabdomyoma being the most common type. Although spontaneous regression of this type of lesions during pregnancy is frequent, fetal cardiac tumors can be associated with complications such as obstruction of cardiac flow, valvular insufficiency, arrhythmias, heart failure and fetal hydrops, which can even lead to fetal death. The greater development of imaging techniques has allowed an increase in the number and precision of prenatal diagnoses of cardiac tumors, generating at the same time, new challenges and alternatives in relation to the therapeutic approach. The objective of this review article is to present the current evidence regarding the prenatal diagnosis, management, complications and associated condition s of the most frequent fetal cardiac tumors.

Upper vena cava syndrome secondary to giant atrial myxoma

SUMMARY Cardiac myxoma is a benign neoplasm, which corresponds to the most common primary heart tumour, responsible for about 50% of the cases. In general, 75-80% of myxomas are located in the left atrium, 18% in the right atrium, and more rarely in the ventricles or multicentric. Right atrial myxoma, in particular, can obstruct the tricuspid valve, causing symptoms of right heart failure, peripheral oedema, hepatic congestion, and syncope. Systemic embolization occurs in 30% of cases, by either tumour fragmentation or total tumour detachment. In the present report, we present a case of a symptomatic patient, who showed a large right intra-atrial lesion, with consequent superior vena cava syndrome, and then underwent surgical resection at admission.

RESUMO O mixoma cardíaco é uma neoplasia benigna, que corresponde ao tumor primário mais comum do coração, responsável por cerca de 50% dos casos. De modo geral, 75 a 80% dos mixomas estão localizados no átrio esquerdo, 18% no átrio direito, e mais raramente, nos ventrículos ou multicêntricos. O mixoma atrial direito, em particular, pode obstruir a válvula tricúspide, causando sintomas de insuficiência cardíaca direita, edema periférico, congestão hepática e síncope. A embolização sistêmica ocorre em 30% dos casos, quer pela fragmentação do tumor ou pelo desprendimento total do mesmo. No presente relato, apresentamos um caso de uma paciente sintomática, que evidenciou grande lesão intra-atrial direita, com consequente síndrome da veia cava superior, sendo, então, submetida a ressecção cirúrgica na internação.

Atrial myxoma in a patient with patent foramen ovale: evaluation with multimodal imaging. Case report / Mixoma auricular en un paciente con foramen oval permeable: evaluación con imágenes multimodales. Reporte de un caso

Abstract: Primary cardiac tumors in adults are rare and mostly benign, approximately 75%, being the myxoma the most frequent in half of that percentage. One-fourth of the primary cardiac tumors are malignant and 95% of the cases are sarcomas. Metastases are more frequent than primary tumors. Cardiac surgery is indicated specially in benign masses and therapies such as chemotherapy and/or radiotherapy should be reserved for unresectable or metastatic malignancies. Hence the importance of a diagnostic approach, which leads to the best therapeutic conduct and in many cases, a multimodal image approach is necessary, as it is exposed in our case.(AU)

Resumen: Los tumores cardiacos primarios en adultos son raros y en su mayoría benignos, aproximadamente el 75%, siendo el mixoma el más frecuente en la mitad de ese porcentaje. Un cuarto de los tumores cardiacos primarios son malignos y el 95% de los casos son sarcomas. Las metástasis son más frecuentes que los tumores primarios. La cirugía cardiaca está indicada especialmente en masas benignas y las terapias como la quimioterapia y/o la radioterapia deben reservarse para tumores malignos o metástasis irresecables. De ahí la importancia de un enfoque de diagnóstico, que conduzca a la mejor conducta terapéutica y, en muchos casos, es necesario un enfoque de imagen multimodal, como está expuesto en nuestro caso.(AU)

Mixoma auricular izquierdo: manifestación por lipotimia y episodios sincopales de corta duración / Left atrial myxoma: manifestation due to lipotimia and syncopal episodes of short duration

Se presenta un caso de mixoma auricular izquierdo que se manifiesta clínicamente por episodios sincopales de corta duración relacionados con la posición postural. El diagnóstico fue confirmado con la ecocardiografía transtorácica y transesofágica y complementado con un cateterismo cardiaco en donde se descubrió una lesión severa de la arteria descendente anterior. La cirugía cardiaca con circulación extracorpórea fue el tratamiento lográndose exéresis completa del tumor en el sitio de implante de su pedículo.

We present a case of left atrial myxoma that is manifested clinically by syncopal episodes of short duration related to the postural position. The diagnosis was confirmed by transthoracic and transesophageal echocardiography and complemented by cardiac catheterization in which a severe lesion of the anterior descending artery was discovered. Cardiac surgery with extracorporeal circulation was the treatment achieving complete excision of the tumor at the site of implantation of its pedicle.

Evaluación de tumores cardiacos por tomografía computada multidetector y resonancia magnética cardiaca / Evaluation of cardiac tumors by multidetector computed tomography and magnetic resonance imaging

Resumen Los tumores cardiacos constituyen una patología poco frecuente (0.002-0.3%) en todos los grupos de edad, sin embargo tienen importancia clínica por el órgano que comprometen. Se clasifican en primarios (benignos o malignos) y secundarios (metástasis). De los primarios el mixoma es el tumor benigno más común, y el sarcoma representa la mayoría de las lesiones malignas. Las metástasis cardiacas son más frecuentes que los tumores primarios. Las manifestaciones clínicas de los tumores cardiacos son poco específicas y varían de acuerdo a su localización, tamaño y agresividad. El uso de tomografía computada multidetector (TCMD) y resonancia magnética (RM) nos ayuda a tener conocimiento de la localización, el tamaño, las relaciones anatómicas y el compromiso de las estructuras adyacentes; además, la RM ayuda a la caracterización tisular del tumor. Es por eso que los estudios en imagen cardiovascular no invasiva tienen un papel importante en la caracterización de estas lesiones y su diagnóstico diferencial entre ellas.

Abstract Cardiac tumors, are a rare pathology (0.002-0.3%) in all age groups, however, they have a clinic importance, due the affected organ. They are classified in primary (benign or malignant) and secondary (metastasis) types. Among primary type, mixoma, is the most common benign tumor, and sarcoma represents most of the malignant injuries. Cardiac metastasis are more frequent than primary tumors. Clinic effects of cardiac tumors are unspecific and vary according their location, size and agresivity. The use of Multidetector Computed Tomography (MDCT) and Magnetic Resonance Imaging (MRI) assist on the location, sizing, anatomical relationships and the compromise of adyacents structures, besides, MRI is useful for tissue characterization of the tumor. Due to the previous reasons, studies based on noninvasive cardiovascular imaging, have an important role on the characterization of these lesions and the differential diagnosis among them.

Mixoma: manifestaciones neurológicas. a propósito de un caso / Mixoma: neurological manifestations. on purpose of a case

El objetivo del presente artículo es informar de un caso de mixoma cardíaco en aurícula izquierda (AI) en paciente de sexo femenino de 35 años, que presenta accidente vascular isquémico (AVC) manifestado neurológicamente como síndrome retiniano izquierdo y hemiparesia derecha. Se realizan estudios complementarios como TC e IRM de cerebro, ecocardiografías, resección tumoral y confirmación histopatológica. El tratamiento fue exitoso. Conclusiones: El mixoma auricular es una patología infrecuente, que puede no producir sintomatología cardíaca y presentarse con manifestaciones neurológicas, debiendo realizarse conducta quirúrgica una vez diagnosticada. Es importante realizar ecocardiografía en los casos en los que no se halla la causa de un AVC.

The objective of this article is to report one case of left atrial cardiac myxoma in female patient 35 years old that presents vascular ischemic accident with neurological manifestation and later sequels. We carried out complementary studies as TC and brain IRM, echocardiography, resection tumoral and pathologic confirmation. The treatment was successful. Conclusions: The atrial cardiac myxoma is an uncommon pathology that cannot produce heart sintomatology and to show up with neurological manifestations, should be carried out a surgical behavior once diagnosed. It is important to carry out echocardiography in the cases in whom is not clear the cause of a vascular cerebral event.

Mixoma odontogénico, un reto para el diagnóstico / Odontogenic myxoma, a challenge to diagnosis

Se presenta un paciente masculino, de 41 años de edad, piel blanca, con hábitos de fumar e ingerir bebidas alcohólicas que al examen físico manifiesta lesión tumoral de 3,3 cm. de diámetro, con aspecto de roseta, ocupando el área desdentada de reborde alveolar inferior derecho correspondiente a primer y segundo molares. En la radiografía panorámica se observó área radiolúcida multilocular que respeta el borde de la mandíbula, con una cortical irregular en algunas áreas. El diagnóstico histopatológico utilizando la coloración de hematoxilina y eosina fue de mixoma odontogénico. El paciente fue tratado quirúrgicamente y se confirmó el diagnóstico utilizando la coloración de azul alciano, que fue positiva. El mixoma odontogénico por ser un tumor que no tiene características clínicas ni radiográficas patognomónicas, síntomas imprecisos, comportamiento agresivo local y recidivas frecuentes debe ser siempre tenido en cuenta en los diagnósticos diferenciales de los tumores del maxilar y la mandíbula(AU)

The case of a 41-year-old male white patient who smokes and drinks alcoholic beverages and that on the physical examination manifests a rosette-like tumoral lesion of 3.3 cm of diameter that ocuppies the edentulous area of the inferior right alveolar crest of the mandible corresponding to the first and second molars is presented. In the panoramic radiography, it was observed a radiolucid multiocular area that respects the border of the mandible with an irregular cortical in some areas. An odontogenic myxoma was histopathologically diagnosed by using hematoxylin and eosin staining. The patient was surgically treated and the diagnosis was confirmed by alcian blue staining, which yielded positive. As the odontogenic myxoma is a tumor with neither clinical nor pathognomic radiographic characteristics, inaccurate symptoms, local aggresive behavior and frequent relapses, it should always be taken into account in the differential diagnoses of the tumors of the maxilla and the mandible(AU)