Progressive Acute Lower Extremity Ischemia Resulting From Cardiac Myxoma Embolization: A Case Report.

Cardiac myxomas are the most common primary benign tumors of the heart. The occlusion of peripheral arteries and complete obstruction of the abdominal aorta by a tumor embolus presents with distinct clinical manifestations. Herein, we present the case of a 38-year-old male with acute paresthesia, muscle weakness, erythematous, and violaceous changes in skin color localized to the dorsum of the left forefoot initially treated as cutaneous vasculitis. Further studies revealed the total occlusion of the terminal abdominal aorta by a saddle embolus from a cardiac myxoma. A multidisciplinary team consisting of cardiothoracic and vascular surgeons were involved in treating the patient, which resulted in full resolution of the case. This paper details the progression of acute bilateral limb ischemia to chronic limb threatening ischemia resulting from the total occlusion of the terminal abdominal aorta by a saddle embolus.

Clinicopathologic and imaging features of odontogenic myxomas: a multi-institutional study.

PURPOSE: This study aims to report clinicopathologic and imaging features of odontogenic myxomas (OM), highlighting uncommon findings. METHODS: Clinicopathologic and imaging data of OMs diagnosed in the five Brazilian diagnostic pathology centers were collected and analyzed. RESULTS: The series comprised 42 females (68.9%) and 19 males (31.1%), with a 2.2:1 female-to-male ratio and a mean age of 34.5±15.4 years (range: 4-80). Clinically, most OMs presented as painless intraoral swelling (n = 36; 70.6%) in the mandible (n=37; 59.7%). Multilocular lesions (n=30; 83.3%) were more common than unilocular lesions (n=6; 16.7%). There was no statistically significant difference between the average size of unilocular and multilocular OMs (p=0.2431). The borders of OMs were mainly well-defined (n=24; 66.7%) with different degrees of cortication. Only seven tumors caused tooth resorption (15.9%), while 24 (54.5%) caused tooth displacement. Cortical bone perforation was observed in 12 (38.7%) cases. Morphologically, OMs were characterized mainly by stellate or spindle-shaped cells in a myxoid background (n=53; 85.5%). Surgical resection was the most common treatment modality (n=15; 65.2%), followed by conservative surgery (n=8; 34.8%). Outcomes were available in 20 cases (32.3%). Seven of these patients had local recurrence (35%). Enucleation was the treatment with the highest recurrence rate (4/7; 57.1%). CONCLUSIONS: OM has a predilection for the posterior region of the jaws of female adults. Despite their bland morphological appearance, they displayed diverse imaging features. Clinicians must include the OM in the differential diagnosis of osteolytic lesions of the jaws. A long follow-up is needed to monitor possible recurrences.

Prenatal Diagnosis of Umbilical Cord Angiomyxoma: Case Studies and Literature Review of 45 Cases.

We describe two cases of umbilical cord (UC) angiomyxoma diagnosed prenatally by sonography in the second trimester of pregnancy. In both cases, a complex mass was detected at the placental insertion site, characterized by an echoic nodule surrounding the umbilical vessels and distal edematous Wharton's jelly. Follow-up scans showed that the mass grew mainly at the expense of its edematous component, with normal uteroplacental Dopplers throughout the remaining of the pregnancy. However, late-onset fetal growth restriction complicated the progress of pregnancy, requiring delivery by Cesarean section at 37 weeks' gestation in both cases. Neonatal courses were unremarkable. An extensive review of the English literature was also performed, collecting 45 similar cases including ours. Our experience as well as the review of the literature confirms that UC angiomyxoma is an uncommon, sporadic condition that is usually detected incidentally during prenatal sonography and presents as an isolated finding. Nevertheless, it represents a high-risk condition for pregnancy complications including prematurity, fetal growth restriction, and fetal demise.

Acute ST-Elevation Myocardial Infarction in a Young Adult: Rare Presentation of Giant Atrial Myxoma. / Infarto com Supra-ST em Adulto Jovem: Rara Apresentação de Mixoma Atrial Gigante.

Cardiac tumors are rare entities, among which atrial myxoma (AM) stands as the most frequent, accounting for approximately half of all reported cases. The incidence of AM is estimated to range from 0.001% to 0.3% within the general population, yet only about 0.06% of these cases present with coronary embolic events. We report on a 33-year-old male smoker who experienced acute, severe precordial pain radiating to the left upper limb, lasting for one hour. The electrocardiographic evaluation demonstrated ST-segment elevation in leads D2, D3, and aVF, alongside significantly elevated serum troponin levels, confirming a diagnosis of ST-segment elevation myocardial infarction (STEMI). Subsequent coronary angiography revealed proximal occlusion of the right coronary artery due to thrombus. An initial attempt of thrombus aspiration was unsuccessful, followed by primary angioplasty with balloon inflation without stent placement. Further diagnostic exploration through transthoracic echocardiography identified a homogenous, smooth-surfaced mass measuring 5.2 cm x 2.3 cm attached to the interatrial septum. This mass, characterized by lobulations, prolapsed into the mitral valve and left ventricle during diastole, consistent with AM. Surgical resection of the mass was successfully performed, with the patient being discharged asymptomatic. In the reported case, the patient's profile, notably his age, and gender, diverges from the typical epidemiological characteristics associated with AM. This case adds to the limited number of reports where the inferior wall is affected by the right coronary artery being occluded. This report emphasizes the significance of differential diagnoses in younger patients presenting with STEMI.

Neoplasias cardíacas são raras, tendo como principal representante o mixoma atrial (MA), que corresponde a cerca de metade de todos os casos. O MA tem incidência estimada entre 0.001% e 0.3% na população em geral, no entanto apenas aproximadamente 0,06% desses cursam com eventos embólicos coronarianos. Homem de 33 anos, tabagista, admitido com quadro de precordialgia intensa e irradiação para membro superior esquerdo com duração de uma hora. O eletrocardiograma evidenciou elevação de segmento ST nas derivações D2, D3 e aVF troponina sérica elevada, confirmando infarto com supra desnivelamento do segmento ST (IAMCSST). Foi realizada cineangiocoronariografia, a qual revelou oclusão em terço proximal de artéria coronária direita por trombo. Realizada tentativa de aspiração do trombo, sem sucesso, seguido por angioplastia primária com balão sem colocação de stent. Durante a investigação do quadro, paciente realizou ecocardiograma transtorácico o qual demonstrou massa homogênea de superfície regular, de 5.2 cm x 2.3 cm, aderida ao septo interatrial, com lobulações de características emboligênicas prolapsando para valva mitral e ventrículo esquerdo na diástole, compatível com MA. Foi realizada ressecção cirúrgica com paciente evoluindo assintomático, recebendo alta para seguimento ambulatorial. O caso relatado difere em idade e sexo do perfil epidemiológico típico sendo um dos poucos descritos com acometimento da parede inferior apresentando a artéria coronária direita como culpada. Este relato ratifica a importância do diagnóstico diferencial frente às apresentações de IAMCSST em jovens.

Our 10-Year Experience with Atrial Myxomas: Is Concurrent Valve Intervention Really Warranted?

INTRODUCTION: Primary cardiac myxomas are rare tumors. Concurrent valvular lesion is a common finding on evaluation which is thought to be due to annular dilatation secondary to tumor movement across the valve, functional obstruction across the valve, and severe pulmonary hypertension secondary to chronic obstruction. A common belief among surgeons is that excision of myxoma leads to abatement of symptoms, and further valve intervention may not be warranted. METHODS: A 10-year retrospective descriptive study was designed to analyze patients who underwent excision of cardiac myxoma at our center. Data was analyzed regarding presenting features, echocardiographic findings of myxoma and valve morphology, intraoperative assessment, and postoperative outcome with/without valve repair/replacement in all patients. RESULTS: A total of 22 patients underwent surgery for myxoma. Six patients underwent successful mitral valve repair with ring annuloplasty, two had moderate mitral regurgitation, three had severe mitral regurgitation, and one patient had no mitral regurgitation on preoperative assessment, but moderate mitral regurgitation was found intraoperatively. Four of these patients had no residual mitral regurgitation in follow-up period while two had mild residual mitral regurgitation. One patient had severe mitral stenosis of concurrent rheumatic etiology and successfully underwent mitral valve replacement. CONCLUSION: Cardiac myxomas are rare benign tumors commonly associated with mitral valve insufficiency. Mitral valve should be assessed intraoperatively after excision of mass as preoperative assessment might often be insufficient. Concomitant mitral valve intervention might be needed with a case-specific tailored approach, and mitral valve repair with ring annuloplasty offers best surgical outcome in such cases.

Atrial myxoma and associated Cushing syndrome: Carney complex.

A 33-year-old woman with a history of high blood pressure since she was 8 years old, hypothyroidism, polycystic ovary syndrome, metabolic syndrome, multiple nevi, and a maternal family history of death at age 50 due to malignant high blood pressure and heart failure. Cushing's syndrome secondary to a secretory pituitary microadenoma was diagnosed, being the cause of secondary arterial hypertension, and ruling out other causes such as renal stenosis and coarctation of the aorta. A transthoracic and transesophageal echocardiogram was performed, which detected a left atrial myxoma. Given the presence of an atrial myxoma, Cushing's syndrome and polycystic ovary syndrome, a diagnosis of Carney Complex was made due to the presence of positive Stratakis criteria. The cardiac tumor was resected, and pathology confirmed that it was an atrial myxoma. She evolved clinically stable in outpatient controls in a 6-month follow-up. Resection of the pituitary microadenoma is planned as a curative treatment for Cushing's syndrome and arterial hypertension.

Mujer de 33 años, con antecedentes de hipertensión arterial desde los 8 años, hipotiroidismo, síndrome de ovario poliquístico, síndrome metabólico, nevos múltiples y antecedente familiar materno de muerte a los 50 años por hipertensión arterial maligna e insuficiencia cardiaca. Se diagnosticó síndrome de Cushing secundario a un microadenoma hipofisario secretor, siendo la causa de la hipertensión arterial secundaria, y descartándose otras causas como estenosis renal y coartación de aorta. Se realizó u n ecocardiograma transtorácico y transesofágico que detectaron un mixoma auricular izquierdo. Ante la presencia de un mixoma auricular, síndrome de Cushing y síndrome de ovario poliquístico se llegó al diagnóstico de Complejo de Carney por la presencia de criterios de Stratakis positivos. Se realizó la resección del tumor cardiaco, y la anatomía patológica confirmó que se trataba de un mixoma auricular. Evolucionó clínicamente estable en controles ambulatorios en un seguimiento de 6 meses, y se planifica la resección del microadenoma hipofisario como tratamiento curativo del síndrome de Cushing y la hipertensión arterial.

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.

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 Malignant Lymphoma Growing Inside a Cardiac Mixoma: A Case Report.

INTRODUCTION: Lymphomas arising from cardiac myxomas represent a particularly rare pathology, with only few cases reported in the literature.Case presentation: We report a complete excision of a malignant lymphoma arising from a cardiac myxoma in a 44-year-old female patient. The myxoma presented like a floating mass within the left atrium with a maximum diameter of 3.5 cm. The clinical post-operative period was uneventful and the patient was dismissed on the 6th post-operative day. CONCLUSION: This case reinforces the concept of radical excision of cardiac neoplasms.

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.

Ultrasonographic features of cutaneous myxomas in a patient with Carney complex.

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.

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.

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.

Multidisciplinary Approach to Right Ventricular Myxoma.

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.

Cerebral embolization associated with parenchymal seeding of the left atrial myxoma: Potential role of interleukin-6 and matrix metalloproteinases.

Systemic embolization has been reported in up to 40% of patients with left atrial myxoma, half of them with cerebral involvement. However, development of intracerebral embolization associated with parenchymal seeding of the myxoma emboli is an extremely rare complication, with only 36 histologically diagnosed cases reported in the published literature. We describe a 69-year-old woman who arrived at the emergency service with hemiparesis associated with drug-resistant epilepsy and a medical history of resection of a left atrial myxoma 10 months previously. Cranial computed tomography revealed multiple large lesions of heterogeneous density and cystic components in the occipital lobes and posterior fossa parenchyma. Histopathological analyses after stereotactic biopsy of the occipital lesion revealed infiltrative myxoma cells with benign histological findings and uniform expression of calretinin similar to that of the primary cardiac myxoma. Additional immunohistochemical studies confirmed brain parenchymal seeding of the myxoma cells with strong expression of interleukin-6 (IL-6) and focal expression of matrix metalloproteinases-2 (MMP-2). Here, we discuss the clinicopathological features of intracerebral embolization of left atrial myxomas associated with progressive parenchymal seeding of the tumor emboli and the potential pathogenic role of IL-6 and MMPs.