Epithelioid hemangioma of the ascending aorta: A rare case

Cardiac epithelioid hemangioma is extremely rare. Currently, there are only a few described cases of intraluminal hemangiomas of the aorta and the aortic valve and no described cases with extraluminal epithelioid hemangiomas of the ascending aorta. We now present a case of epithelioid hemangioma of the ascending aorta that was an incidental finding during the coronary artery bypass and successfully resected.

Supradesnivel del segmento ST como forma de presentación atípica de un tumor cardíaco: rol diagnóstico de la multimodalidad de imagen / ST segment elevation as an atypical presentation of a cardiac tumor: diagnostic role of a multimodal imaging approach / Elevação do segmento ST como apresentação de tumor cardíaco: papel diagnóstico da multimodalidade de imagem

Los tumores cardíacos malignos son neoplasias poco frecuentes que pueden presentarse de diversas formas, lo que dificulta su diagnóstico. La ecocardiografía y la resonancia magnética cardíaca son técnicas fundamentales para el diagnóstico, la caracterización y la evaluación de su extensión tumoral. La identificación de la línea tumoral es esencial al iniciar un tratamiento oncológico dirigido. Si bien el "estándar de oro" para este fin es el estudio anatomopatológico (obtenido por biopsia o resección quirúrgica), en los casos en que esto no es posible, la resonancia magnética cardíaca es la técnica no invasiva que proporciona un mejor abordaje diagnóstico. El tratamiento de elección es la resección quirúrgica y el pronóstico, en general, es malo. Presentamos el caso de una paciente con un tumor cardíaco de presentación clínica atípica, en la que un abordaje imagenológico multimodal aporta información clave y complementaria para el diagnóstico y la caracterización tisular.

Malignant cardiac tumors are rare neoplasms that can present in various forms, making their diagnosis difficult. Echocardiography and cardiac magnetic resonance imaging are fundamental techniques for the diagnosis, characterization, and evaluation of tumor extension. Identification of the tumor line is essential when initiating targeted cancer therapy. Although the "gold standard" for this purpose is the pathological study (obtained by biopsy or surgical resection), in cases where this is not possible, cardiac resonance is the non-invasive technique that provides a better diagnostic approach. The treatment of choice is surgical resection and the prognosis is generally poor. We present the case of a patient with an atypical clinical presentation, in which a multimodal approach provides key and complementary information for tumor diagnosis and tissue characterization.

Os tumores cardíacos malignos são neoplasias raras que podem se apresentar de várias formas, dificultando seu diagnóstico. A ecocardiografia e a ressonância magnética cardíaca são técnicas fundamentais para o diagnóstico, caracterização e avaliação da extensão tumoral. A identificação da linha do tumor é essencial ao iniciar a terapia direcionada do câncer. Embora o "padrão ouro" para esse fim seja o estudo patológico (obtido por biópsia ou ressecção cirúrgica), nos casos em que isso não seja possível, a ressonância cardíaca é a técnica não invasiva que proporciona melhor abordagem diagnóstica. O tratamento de escolha é a ressecção cirúrgica e o pronóstico geralmente é ruim. Apresentamos o caso de uma paciente com apresentação clínica atípica, em que a abordagem multimodal fornece informações essenciais e complementares para o diagnóstico do tumor e caracterização do tecido.

Cardiac papillary fibroelastoma; when, how, why.

We would like to present an interesting case operated on in our department and discuss the international bibliography about this issue. We also present some interesting images of this case. Our material is composed from a 68‑year‑old woman treated by the authors. She presented with a small murmur in the auscultation while she was asymptomatic and then she diagnosed with a tumor on of the left coronary cusp of the aortic valve with the characteristics of papillary fibroelastoma. On the basis of the potential embolic risk either of the mass itself or of associated thrombus and the possibility of further enlargement, the patient although asymptomatic at the time of diagnosis was referred for elective surgical excision of the mass. She underwent on median sternotomy and through extracorporeal circulation the mass has been excised with the preservation of the well‑functioning valve. Through this case, we would like to discuss the bibliography for the decision making in these cases. Hence, the aim of our study is that we have to keep in mind that this kind of friable mass may be the cause of embolism, stroke or coronary artery occlusion and must be excided in a conservative setting, sparing the aortic valve.

Uterine Intravenous Leiomyomatosis with Intracardiac Extension and Pulmonary Benign Metastases on FDG PET/CT: A Case Report

A 48-year-old woman presented with a 50-day history of irregular vaginal bleeding and lower abdominal pain. Ultrasound indicated an extremely large occupying lesion in the pelvic cavity that was highly suggestive of malignancy. Fluorodeoxyglucose (FDG) positron emission tomography/computed tomography (PET/CT) was performed to further assess the nature of pelvic abnormality. PET/CT images demonstrated a diffusely lobulated mass ranging from cervix up to the inferior pole of kidneys with mild FDG uptake. Simultaneously, multiple nodules in bilateral lungs and a hypodense lesion in the right ventricle were shown without FDG-avidity. Based on the imaging results, the presumptive diagnosis was uterine intravenous leiomyomatosis with intracardiac extension and pulmonary benign metastases, which was subsequently confirmed by MRI and the lesion biopsy.

Leiomiosarcoma atrial izquierdo como causa de insuficiencia cardíaca / Left atrial leiomyosarcoma as cause of heart failure

Se presenta el caso clínico de una paciente de 26 años de edad con diagnóstico de insuficiencia cardíaca y presencia de una masa ecogénica no calcificada y móvil, adherida a la región posterolateral de la aurícula izquierda. La joven fue operada y el examen hístico evidenció un leiomiosarcoma en el corazón, con alto grado de malignidad. La evolución posoperatoria resultó satisfactoria y se vigiló rigurosamente su estado clínico por parte de los especialistas de cardiología y oncología en la institución.

The case of a 26 year-old patient with diagnosis of heart failure and presence of a mobile echogenic mass without calcification attached to the posterolateral area of the left atrium was reported. She underwent surgery and tissue examination evidenced a high-grade heart leiomyosarcoma. Postoperative course was satisfactory and her clinical state was rigorously monitored by oncology and heart specialists in the institution.

Rabdomioma cardiaco tratado quirúrgicamente con éxito y revisión de la literatura / Cardiac rhabdomyoma surgically treated with success. Review of literature

Los tumores cardiacos primarios son raros, con incidencia variable en todas las edades del 0.005 al 0.05%. En pacientes pediátricos, la incidencia es del 0.27%. Los tumores más frecuentes durante la infancia son los rabdomiomas cardiacos, considerados como benignos. Aunque la expresión clínica es amplia, en la mayoría de los casos son asintomáticos y se detectan por la presencia de soplos. En la etapa prenatal se manifiestan con arritmias o hydrops fetalis. En algunos neonatos y lactantes se encuentran arritmias, datos de bajo gasto cardíaco o muerte súbita. La asociación con esclerosis tuberosa se ha observado hasta en un 81%. Se presenta el caso de paciente masculino neonato, con diagnóstico de rabdomioma cardiaco que inicialmente estaba asintomático, sin embargo en el seguimiento requirió de tratamiento quirúrgico a los 5 meses de edad, por datos de insuficiencia cardiaca secundaria a obstrucción del tracto de salida del ventrículo derecho. A 5 meses de la cirugía, el paciente está asintomático.

The primary cardiac tumors are inusual, the incidence varies in all the ages between 0.005 to 0.05%. In pediatrics patients the incidence is 0.27%. The more frequent tumors during the childhood are the cardiac rhabdomyomas. These tumors are considered benigns. The clinical expression is wide, in the most the cases, the patients are asymptomatic and are detected by murmurs. In the prenatal age are manifested by arrhythmias or hydrops fetalis. The neonates and children may be show cardiac arrhythmias, low cardiac index and sudden cardiac death. The association with tuberous sclerosis had been reported in 81%.We presenta neonate with cardiac rhabdomyoma diagnosed in the newborn period when he was asymptomatic, however in the follow-up he developed cardiac failure by obstruction in the out flow tract of the right ventricle. He underwent open cardiac surgery to resect the obstruction. Five months after surgery, the patient remain asymptomatic.

Rara ubicación de un mixoma gigante / Rare location of a giant mixoma

Se presentó el caso de una joven de 25 años que a las 24 sem de embarazo le fue diagnosticado un tumor cardíaco de gran tamaño en la cavidad ventricular derecha, por la negación de la paciente, no se operó y parió estando asintomática. Cuatro meses después se ingresó en este centro por insuficiencia cardíaca y el ecocardiograma realizado mostró un crecimiento acelerado del tumor, se resecó el mismo, se sustituyó la válvula tricúspide con una bioprótesis y en el estudio de anatomía patológica se diagnosticó mixoma del ventrículo derecho, se dio de alta asintomática.

The case of a 25-year-old woman that on the 24th week of pregnancy was diagnosed a heart tumor of large size in the right ventricular cavity, denied to be operated on and gave birth being asymptomatic, was presented. Four months later, she was admitted in this center due to heart failure and the echocardiogram showed an accelerated growth of the tumor. It was resected and the tricuspid valve was replaced with a bioprothesis. A mixoma of the right ventricle was diagnosed in the pathological anatomy study. She was asymptomatic at the time of discharge.

Fatty Hamartoma of Epicardium

The fatty hamartoma of pericardium is characterized by abnormal accumulations of adipose tissue forming a recognizable mass. We reported a case that could best be put into "fatty hamartoma" group. This 66 year old male presented with atrial fibrillation came in for a anterior mediastinal mass. He received an operation for adenocarcinoma of ascending colon several months ago. The chest CT revealed an inhomogeneous mass in the right anterior mediastinal area, which was not clearly demarcated from the right cardiac border. The heart was deviated to the left side due to the mass. Surgical excision was performed under the impression of cardiac teratoma. The large mass was well encapsulated, and was bright yellow and partly pink, and measured 12x8 cm. Microscopically, the mass consisted predominantly of mature fat cells and abundant fibrous tissue with scattered nests of primitive cardiac muscle cells. Reviewing the literature we found the term "fatty hamartoma" could also refer to rhabdomyolipoma or fibrolipoma. Since it contains entrapped cardiac muscle cells, abundant fibrous tissue, multiple blood vessels and fetal fat cells, it could best the categorized into "hamartoma". The term "fatty" represents it's main component. Therefore we propose the term "fatty hamartoma" that could to be used further.