Vascular tangle in the inter-atrial septum - Is it the source of cardiac myxoma?

BACKGROUND: Cardiac myxomas (CMs) are the most common primary tumors of the heart, said to be derived from pluripotent cardiac stem cells. They are most often attached to the left side of the inter-atrial septum (IAS) and a feature noted at the site of attachment is a conglomeration of thick-walled vessels that has been noted to precede the development of myxomas. AIMS: The present study was conducted to compare histology of the normal inter-atrial septa to the septal flap excised with the myxomas and to evaluate the significance of this 'vascular tangle' in the histogenesis of these tumors. MATERIALS AND METHODS: In a 10-year retrospective analysis of all surgically excised CMs, tumors with attached septal sleeves were selected. This histology was compared to the serial sections of 25 normal age-matched septa obtained from normal hearts at autopsy. RESULTS: Of the 56 myxomas seen in 10 years, 38 tumors (all left atrial in location) were received with a flap of the IAS. All of these cases, irrespective of the sizes of the tumor, showed the presence of conglomeration of thick-walled blood vessels, many of which showed abrupt myxoid change in their walls. Another noteworthy feature in many vessels in all flaps was migration of the myxoid tissue through the vascular walls and the endocardium to mushroom out into the atrial lumen. Such vascular channels were not seen in any of the normal IAS. CONCLUSIONS: Based on these findings, we propose a two-step hypothesis: an initial step that stimulates the pluripotent cells toward vasculogenesis with endothelial and smooth muscle differentiation, and a subsequent step leads to production of abundant mucopolysaccharides that splay apart the smooth muscle cells, which would explain rings, cords or nests of myxoma cells around endothelial lined spaces.

Superficial angiomyxoma in a pregnant cow.

A 3-year-old, pregnant, Alpine Brown cow showed a rapidly growing, pedunculated, skin mass located at the umbilical region, reaching 8 kg in weight over a 3-month period after its initial detection. Six days after parturition, the mass was completely surgically excised. During the follow-up period, the cow remained in good health, without signs of recurrence, and showed increased milk production. Histological examination of the mass revealed a loose proliferation of spindle-shaped or stellate cells, immersed in an abundant myxoid matrix with admixed numerous thin-walled blood vessels. Immunohistochemically, the tumour cells were positive for vimentin, α-smooth muscle actin, and desmin. Gross and histopathological features were compatible with superficial angiomyxoma, a subtype of angiomyxoma rarely described in humans, but not in the veterinary literature. The tumour did not infiltrate into the surrounding tissues, and there was no post-excision recurrence after 3 months. The possibility of hormonal dependence of the tumour during pregnancy is discussed based on such findings in some human cases.

Cardiac Myxoma Caused by Fumarate Hydratase Gene Deletion in Patient With Cortisol-Secreting Adrenocortical Adenoma.

CONTEXT: Germline mutations in fumarate hydratase (FH) gene are known to cause hereditary leiomyomatosis and renal cell carcinoma (HLRCC) and are occasionally accompanied with cutaneous and uterine leiomyoma or cortisol-producing adrenocortical hyperplasia. However, the association between FH mutations and cardiac or adrenocortical tumors has remained unknown. Here, we identified a novel deletion in FH, exhibiting cardiac myxoma and subclinical Cushing syndrome due to adrenocortical tumor. CASE DESCRIPTION: A 44-year-old man was referred to our hospital for cardiac and adrenal tumor evaluation. He had a history of multiple painful, dermal papules and nodules diagnosed as cutaneous leiomyoma. The surgically resected cardiac tumor was diagnosed as myxoma. The adrenal tumor was clinically diagnosed as subclinical Cushing syndrome. Laparoscopically resected adrenal tumor was pathologically diagnosed as adrenocortical adenoma harboring unique histological findings similar to primary pigmented nodular adrenocortical disease (PPNAD). DNA analysis revealed a germline deletion in FH c0.737delT (p. Phe225Leufs*31) and loss of heterozygosity (LOH) in cardiac myxoma. As a functional analysis of FH in cardiac myxoma, low FH protein expression with elevated 2-succinocysteine (2SC), a marker of FH dysfunction, was immunohistochemically detected. However, in adrenocortical tumor, LOH of FH was not detected, and FH or 2SC expression was not altered. CONCLUSIONS: This is the first case of HLRCC complicated by cardiac myxoma. LOH of FH deletion and its dysfunction were identified in cardiac myxoma. The association between FH deletion and adrenocortical lesion, however, needs to be further clarified.

Heart myxoma develops oncogenic and metastatic phenotype.

PURPOSE: Heart myxomas have been frequently considered as benign lesions associated with Carney's complex. However, after surgical removal, myxomas re-emerge causing dysfunctional heart. METHODS: To identify whether cardiac myxomas may develop a metastatic phenotype as occurs in malignant cancers, a profile of several proteins involved in malignancy such as oncogenes (c-MYC, K-RAS and H-RAS), cancer-associated metabolic transcriptional factors (HIF-1α, p53 and PPAR-γ) and epithelial-mesenchymal transition proteins (fibronectin, vimentin, ß-catenin, SNAIL and MMP-9) were evaluated in seven samples from a cohort of patients with atrial and ventricular myxomas. The analysis was also performed in: (1) cardiac tissue surrounding the area where myxoma was removed; (2) non-cancer heart tissue (NCHT); and (3) malignant triple negative breast cancer biopsies for comparative purposes. RESULTS: Statistical analysis applying univariate (Kruskal-Wallis and Dunn's tests) and multivariate analyses (PCA, principal component analysis) revealed that heart myxomas (7-15 times) and myxoma surrounding tissue (22-99 times) vs. NCHT showed high content of c-MYC, p53, vimentin, and HIF-1α, indicating that both myxoma and its surrounding area express oncogenes and malignancy-related proteins as occurs in triple negative breast cancer. CONCLUSIONS: Based on ROC (receiver operating characteristics) statistical analysis, c-MYC, HIF-1α, p53, and vimentin may be considered potential biomarkers for malignancy detection in myxoma.

[Aggresive angiomyxoma after renal transplantation]. / Angiomixoma agresivo postrasplante renal.

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.

A rare cause of ischemic stroke: cardiac myxoma. Case report and review of literature.

A 46-year-old female diagnosed several years ago with arterial hypertension and an ischemic stroke with significant recovery was admitted for dyspnea on usual physical activity and fatigue. Physical examination revealed signs of heart failure with crackles on both lung bases, distented jugular veins, accentuated pulmonic valve closure (P2) and tricuspid regurgitation murmur. Echocardiography identified a large tumor in the left atrium, suggestive of atrial myxoma, which caused a severe functional mitral stenosis and produced severe pulmonary hypertension. A cardiac embolic source should always be checked in young patients with stroke. Atrial myxoma can mimic a variety of diseases: rheumatic mitral stenosis, infective endocarditis or autoimmune disease. A review on myxoma's histology, immunohistochemistry and genetics together with clinical aspects is presented.

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.

Mixoma auricular: uma causa rara de acidente vascular cerebral isquêmico / Atrial myxoma: a rare cause of ischemic stroke

O mixoma auricular é uma causa rara de acidente vascular cerebral isquêmico, sendo mais frequente em mulheres jovens. Sua etiologia é desconhecida, e as manifestações clínicas ocorrem por obstrução valvular, embolismo ou sintomas constitucionais. Descrevemos o caso de uma mulher de 62 anos com hemiparesia esquerda e ataxia da marcha, com evidência de lesões isquêmicas bilaterais na ressonância magnética craniencefálica. No ecocardiograma, identificou-se massa auricular esquerda sugestiva de mixoma, submetida à ressecção cirúrgica e confirmação histológica. Posteriormente, foi diagnosticada fibrilação auricular, sendo esta a complicação pós-operatória mais comum. Na suspeita de causa cardiogênica, o ecocardiograma deve ser prontamente realizado, pois o mixoma é potencialmente tratável e tem baixo risco de recorrência após a ressecção cirúrgica. Este caso realça a importância do estudo complementar no acidente vascular cerebral isquêmico e a necessidade de excluir uma fonte embólica proximal na presença de isquemia cerebral em territórios vasculares diferentes.(AU)

The atrial myxoma is a rare cause of ischemic stroke, and is more common in young women. Its etiology is unknown, and clinical manifestations occur due to valve obstruction, embolism or constitutional symptoms. We describe the case of a 62-year-old woman with left hemiparesis and gait ataxia, with evidence of bilateral ischemic lesions on brain magnetic resonance imaging. On the echocardiogram, a left atrial mass suggestive of myxoma was identified; it was resected, and histologically confirmed. Later, an atrial fibrillation was diagnosed, which is the most common postoperative complication. On suspicion of a cardiogenic cause, the echocardiogram should be promptly performed because the myxoma is potentially treatable with a low risk of recurrence after surgical resection. This case highlights the importance of complementary study in ischemic stroke, and the need to rule out a proximal embolic source in the presence of cerebral ischemia in different vascular territories.(AU)

[Asymptomatic Cardiac Myxoma Complicated with Carney Complex;Report of a Case].

Carney complex(CNC) is a rare genetic syndrome, characterized by spotty pigmentation of the skin, cardiac myxomas and multiple endocrine tumors. We present a case of asymptomatic cardiac myxoma associated with CNC. She was 49 year-old healthy woman whose son was known to have CNC. She was also diagnosed as CNC due to her family history, typical cutaneous findings and screening endocrine test. Screening ultrasound echocardiography resulted in discovering her asymptomatic left atrial myxoma of 30 mm size. Tumor was successfully resected via median sternotomy and no signs of recurrence were observed at 1 year follow up. Periodical follow up is mandatory because of its high recurrence rate.

Left Atrial Myxoma Following Coronary Artery Bypass Grafting with Patient Coronary Arterial Grafts: a Rarity.

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.

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.

Renal myxoma in a pediatric transplant recipient.

Renal myxoma is a very rare benign neoplasm seen almost exclusively in adults with only 16 reported cases in the literature. All of these cases have been reported in native kidneys with none being reported in a transplant kidney. We report the case of a renal myxoma in a 17-year-old boy's transplant kidney that was found as an incidental mass on ultrasonography and further evaluated with CT and PET scans. PET findings of a renal myxoma are reported here for the first time, and imaging findings from previous cases are briefly reviewed. This case report highlights the fact that adult-predominant tumors and pathology should always be a consideration in pediatric patients who receive organ transplants from adult donors.

Recurrent Maxillary Odontogenic Myxoma Following Partial Maxillary Resection and Consecutive Osseous Reconstruction Including Tooth Transplantation.

Odontogenic myxoma (OM) is a rare tumour arising in the jaws. The tumour is purported to be odontogenic in origin due to the frequent localisation of the tumour inside the jaws in close relation to teeth. The aim of this report was to detail the course of a patient who developed OM of the maxilla, underwent adequate ablative surgery and reconstruction, including tooth transplantation to the original tumour site, and subsequently developed a local recurrence in close proximity to the teeth transplanted to the reconstructed maxilla 6 years after the first diagnosis. Once again, a partial maxillary resection was performed, with no reconstruction. The patient has been free from tumour recurrence for over 20 years. We discuss the current hypothesis on OM pathogenesis and the possible impact of actively dividing cells on tumour re-growth.

Current diagnosis and management of cardiac myxomas.

Cardiac myxoma is the most common cardiac neoplasm. In the majority of cases, it is isolated (non-syndromic) and located in the left atrium. In up to 10% cases, it is seen in syndromic association with the Carney complex where it is encountered in younger patients, with atypical and multiple locations, such as the right atrium or ventricles, and carries a high risk of recurrence. Imaging is pivotal in the diagnosis, management guidance and surveillance. Surgical excision is the established definitive treatment. Further research should address management strategies in incidentally discovered small myxomas in asymptomatic patients and the role of genetic testing and screening in syndromic myxomas.

Carney complex: a rare cause of Cushing syndrome in pregnancy.

BACKGROUND: Carney complex is a rare, autosomal-dominant, multisystem disorder characterized by endocrine overactivity, spotty skin pigmentation, and myxomas. CASE: We present the case of a 24-year-old primigravid woman with a pregnancy complicated by Carney complex. At 18 weeks of gestation, severe hypertension developed. Medical history was significant for chronic hypertension, nephrolithiasis, and an atrial myxoma excised in 2011. She had Cushingoid features, an elevated 24-hour urine free cortisol, and a cutaneous myxoma. At 26 weeks of gestation, superimposed preeclampsia developed. She underwent a primary classical cesarean delivery, delivering a live female weighing 650 g. CONCLUSION: Carney complex is a rare cause of hypercortisolism and hypertension during pregnancy. It should be considered when features of Cushing syndrome and severe hypertension are present.

Rapid growth of left atrial myxoma after radiofrequency ablation.

Atrial myxoma is the most common benign tumor of the heart, but its appearance after radiofrequency ablation is very rare. We report a case in which an asymptomatic, rapidly growing cardiac myxoma arose in the left atrium after radiofrequency ablation. Two months after the procedure, cardiovascular magnetic resonance, performed to evaluate the right ventricular anatomy, revealed a 10 × 10-mm mass (assumed to be a thrombus) attached to the patient's left atrial septum. Three months later, transthoracic echocardiography revealed a larger mass, and the patient was diagnosed with myxoma. Two days later, a 20 × 20-mm myxoma weighing 37 g was excised. To our knowledge, the appearance of an atrial myxoma after radiofrequency ablation has been reported only once before. Whether tumor development is related to such ablation or is merely a coincidence is uncertain, but myxomas have developed after other instances of cardiac trauma.