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International Journal of Cardiology Mar 2013Cardiac myxomas are rare benign and slowly proliferating neoplasms of uncertain histogenesis with heterogeneous histomorphology and variable and sometimes clinically... (Review)
Review
Cardiac myxomas are rare benign and slowly proliferating neoplasms of uncertain histogenesis with heterogeneous histomorphology and variable and sometimes clinically quite malignant pathological manifestations. Majority of cardiac myxoma occur sporadically while a relatively small proportion of diagnosed cases develop as a part of Carney complex syndrome with established familial pattern of inheritance. Although histologically indistinguishable these two forms of cardiac myxoma exhibit distinct cytogenetic make-up and apparent pathological differences important for their clinical presentation and prognosis. Additional problem is presented with secondary lesions with more aggressive histology and significantly faster cell proliferation suggesting their successive malignant alteration. Surgical resection of cardiac myxoma is currently the only treatment of choice. However, to avoid potentially hazardous operating procedures and possible postoperative complications and to prevent recurrence of the neoplastic lesions it is necessary to develop alternative approaches and identify a possible drug targets for their successful pharmacological treatment. Due to the rarity of the disease, a small number of cases in one institution and lack of comprehensive experimental data particularly concerning the cases of metastatic dissemination and secondary lesions with malignant nature, a comprehensive multi-institutional approach is required for better understanding of their molecular pathology and illumination of key molecular, genetic as well as epigenetic markers and regulatory pathways responsible for their development. In this article we provide comprehensive pathohistological, molecular and cytogenetic overview of sporadic cardiac myxoma cases restating the major hypothesis concerning their histogenesis and emphasizing potential approaches for their further reexamination.
Topics: Diagnosis, Differential; Heart Neoplasms; Humans; Microarray Analysis; Molecular Diagnostic Techniques; Myxoma; Neoplasm Recurrence, Local
PubMed: 22243936
DOI: 10.1016/j.ijcard.2011.12.052 -
BMJ Case Reports Apr 2022Cardiac myxomas are scarce and their clinical manifestations can often be misdiagnosed or confused with other medical conditions. However, early diagnosis and surgical...
Cardiac myxomas are scarce and their clinical manifestations can often be misdiagnosed or confused with other medical conditions. However, early diagnosis and surgical resection can prevent devastating complications of myxomas.We herein describe a case of a huge left atrial myxoma of a female patient in her late 30s. What makes our case unique, apart from the massive size of the myxoma, is the unusual clinical presentation with incessant cough and haemoptysis for more than 6 months. The diagnosis was made by echocardiography and cardiac MR and successful surgical resection was performed with good long-term outcome.
Topics: Echocardiography; Female; Heart Atria; Heart Neoplasms; Hemoptysis; Humans; Myxoma
PubMed: 35487639
DOI: 10.1136/bcr-2021-245938 -
The Annals of Thoracic Surgery May 1980Ten patients with cardiac myxoma were reviewed. The ranged from 23 months to 60 years old. Echocardiography was the most helpful noninvasive diagnostic technique. The...
Ten patients with cardiac myxoma were reviewed. The ranged from 23 months to 60 years old. Echocardiography was the most helpful noninvasive diagnostic technique. The tumor was demonstrated by angiocardiography, left atrial myxomas frequently migrating to the left ventricle in diastole. Hemodynamically, left atrial myxomas were associated with moderately severe pulmonary hypertension and simulated mitral stenosis or insufficiency and right atrial myxomas, with right atrial hypertension. There were 7 myxomas in the left atrium, 2 in the right atrium, and 1 in the right ventricle. Eight patients underwent open-heart operation with removal of the myxoma, 1 had concomitant tricuspid valve replacement, and 1 had biopsy of the right ventricle only. The other patient was a Jehovah's Witness and refused operation. One patient died of cardiac arrest intraoperatively, and another died of a bilateral cerebral infarct. One patient had recurrence requiring reoperation. Postoperative hemodynamic and clinical improvement was more striking in patients with a left atrial myxoma presumably due to a normal mitral valve in contradistinction to the tricuspid valve.
Topics: Adult; Angiocardiography; Child, Preschool; Echocardiography; Female; Heart Atria; Heart Neoplasms; Heart Ventricles; Humans; Male; Middle Aged; Myxoma; Neoplasm Recurrence, Local; Postoperative Complications
PubMed: 7377880
DOI: 10.1016/s0003-4975(10)61667-0 -
Indian Journal of Ophthalmology Sep 2017Myxomas are rare neoplasms of mesenchymal origin. Cases of conjunctival, corneal, and orbital myxomas have been reported in the literature; however, to the best of our...
Myxomas are rare neoplasms of mesenchymal origin. Cases of conjunctival, corneal, and orbital myxomas have been reported in the literature; however, to the best of our knowledge, there is no report of a lacrimal gland myxoma. We report a case of an orbital myxoma involving the lacrimal gland and its management.
Topics: Aged; Biopsy; Diagnosis, Differential; Eye Neoplasms; Humans; Lacrimal Apparatus; Male; Myxoma; Ophthalmologic Surgical Procedures; Tomography, X-Ray Computed
PubMed: 28905841
DOI: 10.4103/ijo.IJO_226_17 -
European Journal of Internal Medicine Dec 2012Atrial myxomas are the most common benign tumors of the heart and may present with a wide variety of symptoms. Although 45% of patients present with neurological... (Review)
Review
BACKGROUND
Atrial myxomas are the most common benign tumors of the heart and may present with a wide variety of symptoms. Although 45% of patients present with neurological symptoms, a diverse range of systemic symptoms also occur.
METHODS
A systemic review of the literature related to the diagnosis, treatment, pathogenesis, and symptoms associated with atrial myxomas was performed.
RESULTS
Here we summarize the current state of understanding about myxoma pathogenesis and treatments are described. We review the common and rare local and systemic effects of myxomas. Additionally, we review the paraneoplastic and metastatic potential of myxomas.
CONCLUSIONS
A better understanding of the diverse disease presentations, paraneoplastic syndromes, and side effects of cytokine abnormalities stemming from myxomas will aid the physician in earlier detection and monitoring of disease recurrence.
Topics: Heart Atria; Heart Neoplasms; Humans; Myxoma; Paraneoplastic Syndromes
PubMed: 23122392
DOI: 10.1016/j.ejim.2012.05.015 -
Annals of Diagnostic Pathology Apr 2000Myxoid lesions can be subdivided into (1) mainstream myxomas of soft tissues, (2) mainstream myxomas located outside the soft tissue, (3) inadequately substantiated... (Review)
Review
Myxoid lesions can be subdivided into (1) mainstream myxomas of soft tissues, (2) mainstream myxomas located outside the soft tissue, (3) inadequately substantiated myxomas, (4) myxoid soft tissue tumors or lesions not regarded as myxomas, (5) myxoid fatty conditions, (6) other soft tissue lesions and tumors that are sometimes markedly myxoid, (7) other soft tissue tumors in which myxoid foci may be seen, and (8) nonneoplastic myxoid conditions of soft tissue. More than 60 such conditions are listed and the five entities regarded as mainstream soft tissue myxomas (namely, intramuscular myxoma, juxta-articular myxoma, superficial angiomyxoma, aggressive angiomyxoma, and myxoid neurothekeoma [myxoma of nerve sheath]) are reviewed in detail. Intramuscular myxoma is exclusively intramuscular, usually affects middle-aged women, is most commonly located in the thigh, and does not recur after simple excision. Multiple intramuscular myxomas are rare and are usually associated with monostotic or polyostotic fibrous dysplasia and Albright's syndrome. Juxta-articular myxoma histologically resembles an intramuscular myxoma, but involves periarticular tendons, ligaments, joint capsules, muscles, and even the subcutis of adults. It may be associated with osteoarthritis of the adjacent joint. Some 30% recur locally. Superficial angiomyxoma also has been called cutaneous myxoma. It affects all ages, with a peak incidence in the third and fourth decades; arises in the trunk, lower limb, head, and neck regions; and usually measures less than 5 cm in diameter. Epithelial components are present in approximately 25% of tumors. Approximately one third recur locally, but there have been no metastases. Patients with multiple lesions may have the Carney complex. Aggressive angiomyxoma usually arises in the pelvic and perineal regions and affects females seven times as often as males. Tumors usually measure 10 cm or more in diameter, invade surrounding tissues, and recur in approximately 50% of cases. None have metastasized. Myxoma of nerve sheath (the myxoid variant of neurothekeoma) preferentially affects the dermis and subcutis of the cervicofacial areas and shoulders of young women. Most patients are younger than 40 years; one third of them are in the second decade of life. The majority of tumors measure between 0.5 and 1.5 cm. Only three of 102 cases compiled from the two largest published series recurred; none metastasized. The different clinicopathologic features and behavior of these five mainstream myxomas indicate that myxoma is not a single entity.
Topics: Adult; Female; Humans; Male; Myxoma
PubMed: 10760324
DOI: 10.1016/s1092-9134(00)90019-4 -
The American Journal of the Medical... Aug 2009Myxomas are the most common primary cardiac neoplasms. They are generally benign and most commonly arise from the left atrium. The clinical course of the left atrial...
Myxomas are the most common primary cardiac neoplasms. They are generally benign and most commonly arise from the left atrium. The clinical course of the left atrial myxoma is characterized by symptoms resulting from obstructive, embolic, or "constitutional" effects of the tumor (Goodwin, Lancet. 1963;1:464; Selzer et al, Am J Med. 1972;52:9; Nasser et al, Am Heart J. 1972;83:694). Obstructive symptoms are most common. Generally symptoms of the obstructive presentation are represented by dyspnea, pulmonary edema, or syncope. Embolic ischemic symptom manifestations are typically cerebral, although they could be peripheral (Greenwood, Am Heart J. 1972;83:694). Constitutional symptoms are seen in 50% of patients. Weight loss, low-grade fever, myalgia, arthralgia, and rash are typical nonspecific constitutional manifestations. Here, we describe an unusual clinical presentation of left atrial myxoma in a young boy, which initially was assessed as allergic dermatitis and later as vasculitis.
Topics: Adolescent; Heart Neoplasms; Humans; Male; Myxoma
PubMed: 19680024
DOI: 10.1097/MAJ.0b013e3181a3c2b1 -
Heart (British Cardiac Society) May 2022Cardiac myxomas (CM) are the most common type of primary cardiac tumours in adults, which have an approximate incidence of up to 0.2% in some autopsy series. The purpose... (Review)
Review
Cardiac myxomas (CM) are the most common type of primary cardiac tumours in adults, which have an approximate incidence of up to 0.2% in some autopsy series. The purpose of this review is to summarise the literature on CM, including clinical presentation, differential diagnosis, work-up including imaging modalities and histopathology, management, and prognosis. CM are benign neoplasms developed from multipotent mesenchyme and usually present as an undifferentiated atrial mass. They are typically pedunculated and attached at the fossa ovalis, on the left side of the atrial septum. Potentially life-threatening, the presence of CM calls for prompt diagnosis and surgical resection. Infrequently asymptomatic, patients with CM exhibit various manifestations, ranging from influenza-like symptoms, heart failure and stroke, to sudden death. Although non-specific, a classic triad for CM involves constitutional, embolic, and obstructive or cardiac symptoms. CM may be purposefully characterised or incidentally diagnosed on an echocardiogram, CT scan or cardiac MRI, all of which can help to differentiate CM from other differentials. Echocardiogram is the first-line imaging technique; however, it is fallible, potentially resulting in uncommonly situated CM being overlooked. The diagnosis of CM can often be established based on clinical, imaging and histopathology features. Definitive diagnosis requires macroscopic and histopathological assessment, including positivity for endothelial cell markers such as CD31 and CD34. Their prognosis is excellent when treated with prompt surgical resection, with postsurgical survival rates analogous to overall survival in the age-matched general population.
Topics: Adult; Echocardiography; Embolism; Heart Neoplasms; Humans; Myxoma; Prognosis
PubMed: 34493547
DOI: 10.1136/heartjnl-2021-319479 -
Annals of Vascular Surgery Nov 2018Myxomas are the most common primary cardiac neoplasms. Multimodality imaging is essential for proper diagnosis and treatment. The cardiovascular manifestations depend on... (Review)
Review
Myxomas are the most common primary cardiac neoplasms. Multimodality imaging is essential for proper diagnosis and treatment. The cardiovascular manifestations depend on myxomas location and size. Intracardiac obstruction, constitutional symptoms, and infected myxomas have been described. Though uncommon, myxomas should also be included in the diagnosis of peripheral embolization to the extremities or visceral aorta. We hereby present a rare case of left atrial myxoma with multiple systemic emboli and a review of the associated literature.
Topics: Biopsy; Computed Tomography Angiography; Echocardiography, Transesophageal; Embolism; Heart Atria; Heart Neoplasms; Humans; Male; Middle Aged; Myxoma; Neoplastic Cells, Circulating; Treatment Outcome
PubMed: 30012450
DOI: 10.1016/j.avsg.2018.04.024 -
World Journal of Surgical Oncology Mar 2023Cardiac myxoma is the most common type of primary cardiac tumor, with the majority located in the atrial wall. The tumor is attached to valvular structures in a few... (Review)
Review
BACKGROUND
Cardiac myxoma is the most common type of primary cardiac tumor, with the majority located in the atrial wall. The tumor is attached to valvular structures in a few cases, of which the pulmonary valve is the least affected. Pulmonary valve myxoma may have different clinical manifestations from the more common cardiac myxomas because of its vital position. A misdiagnosis of these types of cardiac myxoma may be detrimental to the care and well-being of patients. Therefore, this systematic review aims to define the clinical characteristics of pulmonary valve myxoma and how this differs from a more common cardiac myxoma.
METHODS
Employed literature was obtained from PubMed, ScienceDirect, Scopus, Springer, and ProQuest without a publication year limit on August 23, 2022. The keyword was "pulmonary valve myxoma." Inclusion criteria were as follows: (1) case report or series, (2) available individual patient data, and (3) myxoma that is attached to pulmonary valve structures with no evidence of metastasis. Non-English language or nonhuman subject studies were excluded. Johanna Briggs Institute checklists were used for the risk of bias assessment. Data are presented descriptively.
RESULTS
This review included 9 case reports from 2237 articles. All cases show a low risk of bias. Pulmonary valve myxoma is dominated by males (5:4), and the patient's median age is 57 years with a bimodal distribution in pediatric and geriatric populations. The clinical manifestation of pulmonary valve myxoma is often unspecified or asymptomatic. However, systolic murmur in the pulmonary valve area is heard in 67% of cases. Echocardiography remains the diagnostic modality of choice in the majority of cases. Tumor attached to the pulmonary cusps or annulus and extended to adjacent tissues in all cases. Therefore, valve replacement or adjacent tissue reconstructions are required in 77% of cases. The recurrence and mortality are considerably high, with 33% and 22% cases, respectively.
CONCLUSIONS
Pulmonary valve myxoma is more common in males with a bimodal age distribution, and its outcomes seem worse than usual cardiac myxomas. Increasing awareness of its clinical symptoms, early diagnosis, and complete myxoma resection before the presence of congestive heart failure symptoms are important in achieving excellent outcomes. A firm embolization blockade is needed to prevent myxoma recurrence.
Topics: Male; Humans; Child; Aged; Middle Aged; Pulmonary Valve; Echocardiography; Heart Neoplasms; Myxoma; Heart Atria
PubMed: 36941612
DOI: 10.1186/s12957-023-02984-0