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.

Tratamento cirúrgico do mixoma odontogênico sem ressecção maxilar: relato de caso / Surgical Treatment od Odontogenic Mixoma Without Maxillary Resection: Case Report / Tratamiento Quirúrgico del Mixoma Odontogénico Sin Resección Maxilar: Informe de Caso

Mixoma Odontogênico é um tumor de origem mesenquimal raro, de desenvolvimento lento e agressivo que acomete indivíduos entre os 10 e 40 anos de idade e principalmente, do gênero feminino. Este estudo teve como principal objetivo descrever um caso clinico de tratamento cirúrgico do mixoma odontogênico sem ressecção maxilar em uma paciente do gênero feminino que compareceu ao Ambulatório de Patologia Oral e Maxilo Facial, da Faculdade de Odontologia da UNIRG, na cidade de Gurupi-TO - Brasil. A paciente foi submetida ao tratamento cirúrgico conservador, através da curetagem e enucleação total do tumor. A proservação foi realizada em períodos de 12 meses, 24 meses e 48 meses aonde pode-se observar a sequencial e completam reparação óssea, inclusive a permanência dos dentes envolvidos que foram submetidos a tratamento endodôntico com total remodelação da lâmina dura e do ligamento periodontal... (AU)

Odontogenic Myxoma (OM) is a rare tumor of mesenchymal origin, of slow and aggressive development that affects individuals between 10 and 40 years of age and mainly female. This study aimed to describe a clinical case of surgical treatment of odontogenic myxoma with out maxillary resection in a female patient who attended the Outpa tient Clinic of Oral Pathology and Facial Maxillo, of UNIRG Dental School, in the city of Gurupi-TO - Brazil. The patient underwent con servative surgical treatment through curettage and total enucleation of the tumor. Proservation was carried out in periods of 12 months, 24 months and 48 months where it was possible to observe the sequential and complete bone repair including the permanence of the involved teeth that underwent endodontic treatment with total remodeling of hard blade and of the periodontal ligament... (AU)

El mixoma odontogénico es un tumor de origen mesenquimal poco frecuente, de desarrollo lento y agresivo que afecta a individuos entre 10 y 40 años de edad y principalmente mujeres. El objetivo principal de este estudio fue describir un caso clínico de tratamiento quirúrgico de mixoma odontogénico sin resección maxilar en una paciente femenina que asistió a la Clínica Ambulatoria de Patología Oral y Maxilo Facial, de la Facultad de Odontología de UNIRG, en la ciudad de Gurupi-TO - Brasil. El paciente se sometió a tratamiento quirúrgico conservador mediante legrado y enucleación tumoral total. La conservación se realizó en periodos de 12 meses, 24 meses y 48 meses donde es posible observar reparación ósea secuencial y completa, incluyendo la permanencia de los dientes implicados que fueron sometidos a tratamiento endodóntico con remodelación total de la durancia y ligamento periodontal... (AU)

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

Mixoma auricular gigante como diagnóstico diferencial de disnea: a propósito de un caso y revisión de la literatura / Giant atrial myxoma as differential diagnosis of dyspnea: a case report and literature review

Cardiac myxomas are the most common benign primary tumors and are most often located in the left atrium at the level of the interatrial septum, with the characteristic of being pedunculated and highly mobile, which is why they sometimes interfere with the functioning of the mitral valve, generating variable degrees of stenosis and mitral insufficiency. Diagnosis is by echocardiography and treatment is surgical resection. We present the case of a patient with a large atrial myxoma and severe double mitral lesion.

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.

A novel mutation in PRKAR1A gene in a patient with Carney complex presenting with pituitary macroadenoma, acromegaly, Cushing's syndrome and recurrent atrial myxoma

SUMMARY Carney complex (CNC) is a rare syndrome of multiple endocrine and non-endocrine tumors. In this paper we present a 23-year-old Iranian woman with CNC who harbored a novel mutation (c.642dupT) in PRKAR1A gene. This patient presented with pituitary macroadenoma, acromegaly, recurrent atrial myxoma, Cushing's syndrome secondary to primary pigmented nodular adrenocortical disease and pigmented schwanoma of the skin. PRKAR1A gene was PCR amplified using genomic DNA and analyzed for sequence variants which revealed the novel mutation resulting in substitution of amino acid cysteine instead of the naturally occurring valine in the peptide chain and a premature stop codon at position 18 (V215CfsX18). This change leads to development of tumors in different organs due to lack of tumor suppressive activity secondary to failure of synthesis of the related protein.

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.

Angiomixoma pélvico asociado a endometriosis infiltrativa profunda: reporte de un caso / Pelvic angiomyxoma associated to deep infiltrative endometriosis: case report

El angiomixoma de la pelvis es un tumor mesenquimal inusual; que dado su alto potencial de recidiva es relevante que la exéresis quirúrgica sea lo más radical posible. Por su parte, la endometriosis infiltrativa profunda, es otra entidad poco frecuente que requiere de tratamientos complejos. La co-existencia de ambas patologías representa una situación absolutamente infrecuente; de los que hay muy pocos casos reportado en la literatura occidental. Se presenta el caso de una mujer de 41 años de edad que desarrolló ambas entidades nosológicas de forma concomitante y que fue tratada quirúrgicamente con buenos resultados.

Angiomyxoma of the pelvis is an unusual mesenchymal tumor; that given its high potential for recurrence, it is relevant that the surgical resection be as radical as possible. For its part, to deep infiltrative endometriosis is another rare entity that requires complex treatments. The co-existence of both pathologies represents an absolutely infrequent situation; of which there are very few cases reported in western literature. We present the case of a 41-year-old woman who developed both clinical entities concomitantly and who was treated surgically with good results.

Predictors of normalization of circulating interleukin-6 after cardiac myxoma resection

Abstract Objective: To disclose the relationships between the anatomic features of cardiac myxomas and plasma interleukin (IL)-6 levels. Methods: Twelve patients undergoing cardiac myxoma resection at The First Hospital of Putian, Teaching Hospital, Fujian Medical University were enrolled into this study. Pre- and postoperative IL-6 levels were determined by an enzyme-linked immunosorbent assay method, and correlations between cardiac myxoma dimension or volume and plasma IL-6 levels were analyzed. C-reactive protein (CRP) levels were also evaluated. Results: IL-6 and CRP levels were significantly decreased one month after cardiac myxoma resection in comparison to preoperative values. IL-6 and CRP levels did not differ between patients with a cardiac myxoma of irregular appearance and those with a myxoma of regular gross appearance, or between patients with a pedicled or a sessile myxoma. Decrement of IL-6 of patients with irregular cardiac myxomas was much higher than that of patients with regular ones, while no intergroup difference was noted in decrement of CRP. A close direct correlation was noted between IL-6 levels and maximal dimension (length) or volume of cardiac myxomas, whereas CRP levels only correlated with maximal dimension of cardiac myxomas. Conclusion: Anatomic features of cardiac myxomas (sessile, irregular appearance, maximal dimension, and volume) could be determinants of the patients' circulating IL-6 levels. IL-6 was likely to be a more sensitive biomarker than CRP in predicting the inflammatory status of patients with cardiac myxoma. Sessile and irregular cardiac myxomas might predict more severe inflammatory conditions for their more abundant endothelial cells and IL-6 overproduction.

Angiomixoma agresivo postrasplante renal / Aggresive angiomyxoma after renal transplantation

La condición de inmunosuprimido aumenta el riesgo de cáncer en trasplantados renales, en comparación a la población general. La mejor supervivencia de esta población en los últimos años ha convertido a las neoplasias y a la enfermedad cardiovascular en las principales causas de morbi-mortalidad. Presentamos el caso de un paciente trasplantado renal que desarrolló cuatro años después del trasplante una forma inusual de tumor mesenquimatoso, el angiomixoma agresivo, que requirió resección quirúrgica amplia.

The condition of immunosuppressed increases the risk of cancer in kidney transplant patients, as compared to the general population. The best survival of inmunosupressed patients in recent years has turned both neoplasms and cardiovascular diseases into the main causes of morbidity and mortality. We present the case of a renal transplanted patient who developed an unusual form of mesenchymal tumor such as the aggressive angiomyxoma, four years after the implant and requiring wide surgical resection.

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.

Resection of left atrial myxoma in a patient with retrosternal gastric tube: a case report

Abstract The median sternotomy remains the standard approach in cardiovascular surgery but, in some conditions, it can be considered difficult to perform, especially in patients with history of esophagectomy. This case report describes a successful resection of a left atrial myxoma through a right anterolateral thoracotomy approach in a patient with a previous retrosternal gastric tube reconstruction. The decision for the best surgical approach was made after a heart surgery team discussion. Through this surgical access, a safe and excellent exposure of the left atrium was possible, and a complete resection of the myxoma was performed without any injury to the gastric tube.

Left atrial myxoma following coronary artery bypass grafting with patient coronary arterial grafts: a rarity

Abstract The development of left atrial myxoma after coronary artery bypass graft surgery is a rare entity. A 60-year-old man with previous off-pump coronary artery bypass grafting four years ago with patent coronary grafts was diagnosed with left atrial mass. The patient underwent successful resection of the same through minimally invasive right anterolateral thoracotomy. Histopathology of the atrial mass confirmed the diagnosis of atrial myxoma.

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.

Biatrial approach provides better outcomes in the surgical treatment of cardiac myxomas

Abstract Objective: We aimed to present clinical features, surgical approaches, importance of surgical technique and long-term outcomes of our patients with cardiac myxoma who underwent surgery. Methods: We retrospectively collected data of patients with cardiac myxoma who underwent surgical resection between February 1990 and November 2014. Biatrial approach is the preferred surgical method in a large proportion of patients that are operated due to left atrial myxoma because it provides wider exposure than the uniatrial approach. To prevent recurrence during surgical resection, a large excision is made so as to include at least 5 mm of normal area from clean tissue around the tumor. Moreover, special attention is paid to the excision that is made as a whole, without digesting the fragment of tumor with gentle dissections. Results: Forty-three patients (20 males, mean age of 51.7±8.8 years) were included. Most common symptom was dyspnea (48.8%). Tumor was located in the left atrium in 37 (86%) patients. Resections were achieved via biatrial approach in 34 patients, uniatrial approach in 8 patients, and right atriotomy with right ventriculotomy in 1 patient. One patient died due to low cardiac output syndrome in the early postoperative period. Mean follow-up time was 102.3±66.5 months. Actuarial survival rates were 95%, 92% and 78% at five, 10 and 15 years, respectively. Recurrence was observed in none of the patients during follow-up. Conclusion: Although myxomas are benign tumors, due to embolic complications and obstructive signs, they should be treated surgically as soon as possible after diagnosis. To prevent recurrence, especially in cardiac myxomas which are located in left atrium, preferred biatrial approach is suggested for wide resection of the tumor and to avoid residual tumor.