Importance of GLUT1 in differential diagnosis of vascular anomalies / Importância da GLUT1 no diagnóstico diferencial das anomalias vasculares

Vascular anomalies (VAs) include a group of distinct lesions, such as vascular system congenital malformations, as well as benign and malignant vascular tumors. These lesions may present similar clinical and histopathological features, leading to mistaken diagnoses and incorrect treatment choices. It is important that professionals responsible for monitoring the development of VAs conduct precise investigations and use the appropriate terminology. The human glucose transporter protein isoform 1 (GLUT1) has been proposed as a tool to aid in differential diagnosis between different VAs, given that it is a sensitive and specific marker for identification of infantile hemangiomas (HIs) in any organ. This article presents a review of the literature on this protein as an effective tool for identification and possible differential diagnosis between several VAs.

As anomalias vasculares (AVs) incluem um grupo de lesões distintas, como as más formações congênitas e os tumores vasculares benignos ou malignos. Estas lesões podem apresentar características clínicas e histopatológicas semelhantes, ocasionando equívocos diagnósticos e terapêuticos. Uma investigação precisa e o uso de terminologia adequada são fundamentais para as decisões do profissional responsável pelo acompanhamento da evolução de uma AV. A isoforma 1 da proteína humana transportadora de glicose (GLUT1) tem sido proposta como uma ferramenta auxiliar para o estabelecimento de diagnóstico diferencial entre AVs, uma vez que representa um marcador sensível e específico para a identificação de hemangiomas da infância de qualquer órgão. Este estudo objetiva fazer uma revisão da literatura acerca desta proteína como ferramenta eficaz na identificação e no possível diagnóstico diferencial entre as diversas AVs.

Clinical Experiences of Cardiac Myxoma

Although cardiac myxoma is rare, it is the most common primary cardiac tumor. Seventy-four cases of cardiac myxoma that were surgically treated in our center between August 1980 and February 2005 were retrospectively reviewed. The mean patient age was 50.4+/-15.0 (range 7-80) years, and 53 patients (71.6%) were female. The most common preoperative symptom, occurring in 44 patients, was dyspnea. The interval from onset of symptoms to surgery was 9 months. Seventy cases were located in the left atrium, 3 in the right atrium and 1 in the right ventricle. The myxoma in the right ventricle could not be resected completely, due to severe infiltration. Cardiopulmonary bypass and aortic cross clamp times were 100.4+/-37.1 and 64.8+/-29.8 minutes, respectively. There were no hospital deaths, and 7 patients suffered from postoperative complications including atrial fibrillation in 2 cases. During the follow up period (mean 105.7+/-73.6 months), there was no tumor recurrence and 6 late deaths that were not related to the underlying tumor. There was no evidence of tumor growth in the cases with incomplete resection during the 14-month follow-up. In conclusion, in this study there was no recurrence of tumors after complete resection and surgical resection is considered to be the curative method of treatment for cardiac myxoma.

Clinical Experiences of Cardiac Myxoma

Although cardiac myxoma is rare, it is the most common primary cardiac tumor. Seventy-four cases of cardiac myxoma that were surgically treated in our center between August 1980 and February 2005 were retrospectively reviewed. The mean patient age was 50.4+/-15.0 (range 7-80) years, and 53 patients (71.6%) were female. The most common preoperative symptom, occurring in 44 patients, was dyspnea. The interval from onset of symptoms to surgery was 9 months. Seventy cases were located in the left atrium, 3 in the right atrium and 1 in the right ventricle. The myxoma in the right ventricle could not be resected completely, due to severe infiltration. Cardiopulmonary bypass and aortic cross clamp times were 100.4+/-37.1 and 64.8+/-29.8 minutes, respectively. There were no hospital deaths, and 7 patients suffered from postoperative complications including atrial fibrillation in 2 cases. During the follow up period (mean 105.7+/-73.6 months), there was no tumor recurrence and 6 late deaths that were not related to the underlying tumor. There was no evidence of tumor growth in the cases with incomplete resection during the 14-month follow-up. In conclusion, in this study there was no recurrence of tumors after complete resection and surgical resection is considered to be the curative method of treatment for cardiac myxoma.

Neoplasias primárias do coraçäo: apresentaçäo clínica e histológica de 50 casos / Primary neoplasms of the heart: clinical and histological presentation of 50 cases

OBJECTIVE: To analyze clinical and histologic findings of 50 patients with primary neoplams of the heart in a tertiary referral center. METHODS: From 1980 to 1998, we retrospectively analyzed 50 patients, 32 of whom were females, whose ages ranged from 9 to 73 years (mean age = 44.16 + or -18 years). RESULTS: Most tumors were located in the left side of the heart (72 percent), myxoma being the most common (84 percent) histologic type. The other histologic types found were as follows: fibroma (4 percent), lipoma (2 percent), rhabdomyosarcoma (2 percent), hemangioma (2 percent), sarcoma (2 percent), angiosarcoma (2 percent), and lymphoma (2 percent). Diagnosis was established by echocardiography in 94 percent of the cases. Clinical findings were as follows: dyspnea (36 percent), weight loss (20 percent), palpitations (18 percent), chest pain (16 percent), fever (8 percent), and arthralgia (6 percent). All patients with thromboembolic phenomena (10 percent) had left atrial myxoma. Approximately 20 percent of the patients were asymptomatic at the initial clinical assessment. CONCLUSION: Primary cardiac tumors are a rare entity with diverse clinical and histologic findings, requiring, therefore, a high level of clinical suspicion

El sarcoma cardiaco ¿simula lupus eritematoso y vasculitis? Informe de 2 casos / Heart sarcoma mimicks systemic lupus erythematosus? Report of 2 cases

Los tumores primarios del corazón son raros. El más común es el mixoma, que es capaz de sintetizar IL-6 y con frecuencia tiene manifestaciones sistémicas, que confunden el diagnóstico. Los sarcomas cardiacos primarios, aún más raros, nunca han sido asociados a enfermedad sistémica. Informamos dos casos, en donde el diagnóstico presuntivo de lupus eritematoso sistémico fue modificado ante el hallazgo de sarcomas cardiacos primarios de estirpe muscular

Mixoma Cardíaco

Se presentan los aspectos clínicos patológicos de dos casos de Mixoma cardíaco, todos localizados en la aurícula izquierda. Los dos casos se presentaron en adultos de 39 y 22 años de edad. Uno produjo insuficiencia cardíaca descompensada y el otro produjo complicación embólica cerebral

Tumores cardiacos / Cardiac neoplasms

En el pesente trabajo se analiza la bibliografía pertinenete, haciendo incapié en los aspectos clínicos, de laboratorio y gabinete de cada uno de los tumores cardiacos. Los tumores de corazón y pericardio se clasifican en primarios y secundarios, a su vez los primarios en benignos y malignos. Los tumores primarios son raros y representan entre 0.001 y 0.28 por ciento de los tumores globales del cuerpo. Los mixomas son los más comunes y constituyen alrededor de 50 por ciento de todos los tumores histológicamente benignos del corazón. Los tumores secundarios son hasta 40 veces más frecuentes que los primarios. Los que más metastatizan son los mielomas, melanomas, leucemias, carcinomas, lifomas y sarcomas. Cabe señalar que aunque los tumores cardiacos contituyen una patología poco común, ésta debe ser del conocimiento del médico en general y del internista y cardiólogo en particular, sobre todo porque son grandes simuladores de padecimientos cardiovasculares, pricipalmente los mixomas; lo que ha hecho que el cínico piense con mayor frecuencia en ellos y gracias a estudios no invasivos son diagnosticados con mayor facilidad y veracidad. El ecocardiograma modo M, doppler color y transesofágico, así como la resonancia magnética nuclear son los métodos de elección para el diagnóstico. Su tratamiento es puramente quirúrgico y debido a que algunos tienen una alta incidencia de complicaciones que varían desde fenómenos embólicos hasta muerte súbita, paciente diagnosticado, paciente que debe de ser valorado integralmente para ser intervenido quirúrgicamente. El pronóstico de estos tumores dependerá de su grado de malignidad, de las estructuras cardiacas afectadas, si es primario o secundario y de la presencia de metástasis

Cardiopatia neoplásica / Neoplasic cardiopathy

Mesmo diante do avanço propedêutico observado na Cardiologia moderna, devido à sua freqüência clínica realmente incomum, a chamada Cardiopatia Neoplásica exibe reduzido reconhecimento diagnóstico na prática médica. Os tumores cardíacos primários benignos sao menos raros do que os malignos, com predominância, neste aspecto, dos mixomas, ao passo que, em termos de câncer metastático, as neoplasias broncogênicas e de mama sao as que mais envolvem o coraçao durante seu curso evolutivo.

Sarcoma fusocelular de pericardio en la infancia / Spindle-cell sarcoma of the pericardium in a child

En este trabajo se presenta el caso de un sarcoma fusocelular de pericardio reconocido en un niño de 12 años, que se presentó como un tumor mediastinal de topografía incierta y que sólo en la necropsia fue posible determinar su ubicación pericárdica. El tumor se mantuvo limitado al pericardio sin dar metástasis . La inmunomarcación, positiva sólo para vimentina, favorece la interpretación de que el tumor no es un mesotelioma fusocelular.