Complejo de Carney: reporte de un caso y revisión de la literatura / Carney complex: a case report and literature review

Resumen El complejo de Carney es una enfermedad caracterizada por lesiones en la piel, tumores endocrinos, cardiacos, gonadales y en otros órganos, que está asociada con mutaciones del gen PRKAR1A. Presentamos el caso clínico de una paciente con varias de las manifestaciones más características de este síndrome. Finalmente, se hace una revisión de la literatura.

Abstract Carney complex is a disease characterized by skin lesions, endocrine, cardiac, gonadal and other organ tumors, associated with mutations of the PRKAR1A gene. We present the clinical case of a patient with several of the most characteristic manifestations of this syndrome. Finally, there will be a review of the literature.

Cardiac myxoma with cartilaginous differentiation-An uncommon variant presented as mitral stenosis

The estimated incidence of primary cardiac tumors is extremely rare. Among it, cardiac myxoma represents the most common benign cardiac tumor constituting about 80% of cases. We are presenting a 30–year-old female with large left atrial myxoma. She was presented with severe dyspnea, palpitations, and systolic murmurs. On 2D echocardiography, left atrial mass obstructing mitral flow was noted. On cardiac magnetic resonance imaging, a single, large, mobile pedunculated mass lesion in left atrium attached to inferior interatrial septum with heterogeneous enhancement was noted, which was likely represented to be myxoma. The mass was surgically excised and valve repairing was done. We received large, solid, lobulated, gray white, soft-to-firm mass measuring 4.5 × 4.1 × 2.5 cm, and on microscopy showed cardiac myxoma with cartilaginous differentiation. We are presenting this case for its clinical, imaging, and uncommon histological features.

Resultados quirúrgicos y seguimiento postoperatorio de mixomas auriculares / Surgical results and monitoring of postoperative atrial myxomas

Resumen: Objetivo: Analizar la casuística del mixoma auricular izquierdo, haciendo énfasis en los resultados y seguimiento. Método: Se revisaron los expedientes clínicos de los pacientes operados de mixomas cardiacos en el Hospital Central Sur de Alta Especialidad (HCSAE) de PEMEX en los últimos 7 años, haciendo hincapié en los resultados y seguimiento. Resultados: El análisis mostró 10 pacientes, de los cuales el 60% fueron mujeres y el 40% varones; las edades fueron de los 12 a los 76 años, con una edad media de 50 años. En las características clínicas de los pacientes predominó la disnea en un 90%, seguido de la fatiga (80%) y dolor torácico (60%). La incidencia fue del 90% para la aurícula izquierda y del 10% en aurícula derecha, hubo tumores de menos de 3 cm hasta mayores de 10 cm (media de 6-7 cm). El informe de patología fue de mixoma en el 100% de los casos, La morbimortalidad temprana fue del 0%, con una estancia hospitalaria promedio de 6 días; una paciente de 12 años tuvo una recidiva y se sometió otra vez a cirugía 5 meses más tarde. La supervivencia a 5 años es del 100%. Conclusiones: Se sabe que la presentación de Mixomas es muy poco frecuente, la experiencia en este Centro de Concentración Nacional, es de un paciente por cada 350 cirugías, en promedio un caso por año con una nula mortalidad y una excelente supervivencia.

Abstract: Objective: To analyze the casuistics of left atrial myxoma with emphasis on results and follow-up. Method: We reviewed the clinical records of patients operated in the Hospital Cardiac Myxomas South Central High Specialty (HCSAE) of PEMEX in the last 7 years, with an emphasis on results and follow-up. Results: The analysis showed 10 patients, of whom 60% were female and 40% male, with ages from 12 to 76 years, with a mean age of 50 years. In the clinical characteristics of patients predominated dyspnea in 90%, followed by fatigue (80%) and chest pain (60%). The incidence was 90% for the left atrium and 10% for the right atrium, had tumors less than 3 cm to up to over 10 cm (average of 6 to 7 cm). The pathology report was myxoma in the 100% of cases, the morbidity and early mortality was 0%, with a mean hospital stay of 6 days, and a patient of 12 years of age recurred and underwent surgery five months later. The 5-year survival was 100%. Conclusions: The incidence of myxomas is well known for rare presentation, the experience in this national medical center is one patient for every 350 operations, one case per year with no mortality and excellent survival.

Multiple Cardiac Myxomas-Unusual Locations- Flowers in the Cardia.

Myxomas represent the most frequent benign tumor in adult population. It accounts for 25% of all cardiac tumors. Multiple myxomas within the cardia at unusual sites is very rare. We report a 60 year old lady who presented with myxomas in left atrium, right atrium, and pulmonary artery. Myxomas in the pulmonary artery is a very rare entity.

Características histopatológicas e inmunohistoquímicas de los mixomas cardiacos / Histopathological and immunohistochemical features of cardiac myxomas

Los mixomas son los tumores cardiacos primarios más frecuentes, con una incidencia estimada de 0,5-1 por 10(6) individuos por año. Estos tumores han generado interés debido a su peculiar localización (el lado izquierdo del septum auricular cerca de la fossa ovalis), su presentación clínica variable y su histogénesis que aún no ha sido definida. La mayoría de los mixomas cardiacos son esporádicos mientras que aproximadamente el 10% de los casos forman parte del complejo de Carney. Esta neoplasia es de histogénesis incierta, sin embargo, se ha propuesto diferenciación endotelial, neurogénica, fibroblástica, muscular lisa, muscular cardiaca y raramente puede presentar diferenciación glandular. Recientemente, por la expresión de algunos factores específicos cardiomiogénicos, se ha propuesto un origen en células progenitoras mesenquimatosas cardiomiocíticas. Histológicamente los mixomas cardiacos están compuestos por células estelares fusiformes y poligonales inmersas en una matriz mixoide amorfa. Por inmunohistoquímica algunos marcadores endoteliales están presentes como el CD31, CD34 y FVIIIAg. Ha sido también informada positividad a la proteína S-100, calretinina, vimentina, desmina, miosina de músculo liso, CD56, α1-antitripsina, y α1-antiquimiotripsina. La resección quirúrgica es actualmente el único tratamiento. Presentamos en este artículo una revisión histopatológica e inmunohistoquímica de los mixomas cardiacos.

Mixomas are the most common primary cardiac tumors with an estimate incidence of 0,5-1 per 10(6) individuals per year. These tumors have generated interest due to their unique location (left side of the atrial septum near the fossa ovalis), variable clinical presentation and undefined histogenesis. Most cardiac myxomas occur sporadically while approximately 10% of diagnosed cases develop as part of Carney complex. This neoplasm is of uncertain histogenesis, however, endothelial, neurogenic, fibroblastic, and cardiac and smooth muscle cells differentiation has been proposed, and rarely glandular differentiation has been observed. Recently, due to the expression of certain cardiomyocyte-specific factors, an origin of mesenchymal cardiomyocytes progenitor cells has been suggested. Histologically cardiac myxomas are mainly composed of stellated, fusiform and polygonal cells, immersed in an amorphous myxoid matrix. Immunohistochemically some endothelial markers, such as CD31, CD34, FVIIIAg, are present. Positive staining has also been reported for S-100 protein, calretinin, vimentin, desmin, smooth muscle myosin, CD56, α1 antitrypsin and α 1 antichymotrypsin. Surgical resection is currently the only treatment of choice. We present in this article a histopathological and immunohistochemical review of cardiac myxomas.

Right atrial myxoma: An unusual cause of clinical right heart failure

Myxomas are the most common type of primary cardiac tumour with 86% occurring in the left atrium. Right atrial myxomas is far less common. Patients usually present with progressive dyspnoea, fever, weight loss, right-sided heart failure, or pulmonary embolisation. We report the case of a 55-year-old man who presented with progressive dyspnoea and signs of right heart failure secondary to a large right atrial myxoma, which was diagnosed with a bedside transthoracic echocardiogram. This was successfully removed surgically.

Mixomas cardíacos: estudio morfológico e inmunohistoquímico de 50 biopsias

Los mixomas son los tumores primarios más frecuentes del corazón. Se analizaron histopatológicamente 50 biopsias de mixomas cardíacos en 24 de las cuales, se hizo el estudio inmunohistoquímico. Los pacientes (M/F: 19/31) cuyas edades fueron entre la 2ª y 7ª década de vida, presentaron sintomatología variada, fueron operados con el diagnóstico de tumor y/o masa intracardíaca. La localización más frecuente de los mixomas fue en aurículas izquierda (86,0 por ciento) y derecha (8,0 por ciento). Macroscópicamente, presentaron aspecto gelatinoso y mixoide. Sólo un caso estaba petrificado. Midieron entre 3 y 10 cm y el 50,0 por ciento eran pediculados. Al microscopio de luz, se caracterizaron por la presencia de: células aisladas o formando estructuras vasculares (94,0 por ciento), seudopapilares (54,0 por ciento) y seudo glandulares (4,0 por ciento);abundante matriz mixoide con células inflamatorias (96,0 por ciento), hemorragia (94,0 por ciento), nódulos de Gamna-Gandi (30,0 por ciento) y calcificaciones (18,0 por ciento). El estudio inmunohistoquímico reveló: marcaje positivo en las células tumorales para: vimentina (100,0 por ciento) ,CD34 (83,0 por ciento), actina músculo liso (37,0 por ciento) y desmina (17,0 por ciento). El marcaje para S-100 fue negativo y la citoqueratina (A1/AE3) resultó positiva sólo en un caso con diferenciación glandular. Conclusiones: Basándonos en el estudio inmunohistoquímico consideramos que existen evidencias del origen mesenquimal del tumor con diferenciación predominante hacia la línea endotelial

Myxomas are the most frequent primary tumors of the heart. Fifty cardiac biopsies of myxomas were analyzed by histopathology, in 24 of which, the immunohistochemical was done. Patients (M/F: 19/31) whose ages were between second and seventh decade of life, presented varied sintomatology, were operated with the diagnosis of tumor and/or intracardiac mass. The most frequent location of myxomas was in left (86.0%) and right (8.0%) auricles. Macrocospically, they presented gelatinous and mixoide aspect, one single case was petrified. They measured between 3 and 10 cm and 50.0% were pediculate. To the light microscope, they were characterized by: isolated cells or forming vascular structures (94.0%), seudopapilars (54.0%) and pseudo glandulars (4.0%); abundant mixoide matrix with inflammatory cells (96.0%), hemorrhage (94.0%), nodules of Gamna-Gandi (30.0%) and calcifications (18.0%). The immunohistochemical study revealed: positive marker in the tumorlike cells: vimentine (100.0%), CD34 (83.0%), actine smooth muscle (37.0%) desmine (17.0%). The marker for S-100 were negative and the cytoqueratine (A1/AE3) was positive in a single case with glandular differentiation. Conclusions: on the base of the immunohistochemical study, we considered that evidences exist that support the mesenquimal origin of the tumor with predominant differentiation towards the endotelial line

Recurred Left Atrial and Left Ventricular Myxoma after Surgical Excision of Biatrial Atrial Myxoma

We report on a 28 years old female with recurred cardiac myxomas who presented with dizziness, headache, and blurred vision. She had an excision of biatrial atrial myxomas 10 years age. Varying sized multiple cerebral aneuysms and myxomas in left atrium and left ventricle were found on a cerebral angiogram and echocardiogram, respectively. After wide excision including interatrial septum and part of left ventricular septum, her symptoms were much improved.

Clinical diagnosis and treatment of 71 cases of cardiac myxoma / 解放军医学杂志

Objective To study the clinical manifestations of cardiac myxoma and the morbidity, mortality and recurrence rate following surgery in our institution. Methods From December 1989 to November 2004, 71 patients underwent complete excision of primary or recurrent intracardiac myxoma. Pre-operative diagnosis was established by echo-cardiography. All patients underwent operation soon after the diagnosis of myxoma was made. Combined cardiac disorder was also treated synchronously. The excised myxoma was histopathologically studied routinely. Results Myxoma most commonly occurred in the fourth decade of life. Its commonest location (92.9%) was the left atrium (LA), but 3 patients had myxoma in the right atrium (RA), one in the right ventricle (RV), and one in the left ventricle (LV). Patients with LA myxoma simulated mitral stenosis clinically, whereas patients with RA and RV myxoma presented features of right heart failure. Patients with LV myxoma had the symptoms of the left ventricular outlet tract obstruction. 6 patients had history of embolism. Combined cardiac diseases included coronary heart disease (2 cases), rheumatic mitral stenosis (2 cases), and severe mitral insufficiency (3 cases), and severe tricuspid insufficiency (4 cases). One patient had myxoma recurrence for three times after his first surgery, and died in the fourth operation. All the other patients survived the operation with the condition improved during the follow-up period. No late deaths were observed. Conclusions Echocardiography is the ideal diagnostic tool. Immediate surgical treatment is indicated in all patients. Cardiac myxoma can be excised with a low rate of mortality and morbidity. Close follow-up for detecting recurrence is necessary.