Recurrent cardiac myxoma: A case report.
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ABSTRACT:
SUBMITTER: Mahavar RK
PROVIDER: S-EPMC8617394 | biostudies-literature |
REPOSITORIES: biostudies-literature
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Project description:INTRODUCTION: Carney complex is a multiple neoplasia syndrome involving cardiac, endocrine, neural and cutaneous tumors with a variety of pigmented skin lesions. It has an autosomal dominant mode of inheritance. Approximately 7% of cardiac myxomas are related to the Carney complex. Myxomas that occur as part of the Carney complex affect both sexes with equal frequency. Cardiac myxomas with Carney complex are reported mostly in the left side of the heart and are less common on the right side. As per our review, this is the first reported case of Carney complex with right ventricle cardiac myxoma. CASE PRESENTATION: We present a rare case of recurrent cardiac myxoma in a patient later diagnosed to have Carney complex. A 46-year-old Caucasian man with a history of thyroid hyperplasia came to out-patient cardiology department with new onset atrial fibrillation. A transthoracic echocardiogram revealed a right ventricular mass attached to his interventricular septum, which was later seen on a transesophageal echocardiogram and cardiac magnetic resonance imaging. He underwent resection of the ventricular mass which on pathology revealed myxoma. He later developed skin lesions, pituitary adenoma and Sertoli cell tumor suggesting Carney complex. Two years later he developed a new mass within his right atrium which was later resected. CONCLUSIONS: Carney complex is a rare autosomal dominant disease with variable penetrance. Since it involves multiple organs, patients diagnosed with Carney complex should undergo serial endocrine workup, neural assessments, echocardiograms and testicular ultrasounds. Of the total number of cases of Carney complex, 65% are linked to PRKAR1A gene mutation. It is important for clinicians to be cognizant of a link between cardiac myxoma and Carney complex. The use of multi-imaging modalities allows better delineation of the mass before planned resection. Carney complex-related cardiac myxoma comprises 7% of all cardiac myxomas. Right ventricular cardiac myxomas are rare. This case report is the first to describe right ventricular myxoma with Carney complex.
| S-EPMC4038109 | biostudies-literature
Project description:BackgroundCardiac myxoma is a rare neoplasm with female preponderance. It is the commonest benign cardiac neoplasm. We report a case entailing the perioperative anaesthetic challenges in managing a young morbidly obese man with a huge left atrial myxoma, who presented to us with acute symptoms of heart failure.Case presentationA young morbidly obese man with a body mass index (BMI) of 43.3 kg/m2 presented to us with acute symptoms of heart failure. An urgent transthoracic echocardiogram showed a huge pedunculated mass which arise from the left atrium, which occasionally completely occludes the mitral valve during each diastole. He underwent a resection of cardiac myxoma, which was complicated with intraoperative hypotension. Fortunately, it was successfully managed by placing the patient in Trendelenburg position for optimum blood pressure control.DiscussionTumours of the heart are very rare. Common histopathological findings are myxomas, followed by uncommon types of rhabdomyosarcomas and angiosarcomas. Pedunculated cardiac tumours can cause partial to complete obstructions which may lead to devastating conditions. Anaesthesia for morbidly obese patients with pedunculated cardiac myxoma are extremely challenging. Cardiac anaesthetists should be vigilant in managing these patients as they posed many life-threatening complications.ConclusionCardiac myxoma are uncommon benign tumor of the heart with higher preponderances on the left atrium. Pedunculated mass can cause obstruction by limiting left ventricular filling, mimicking severe mitral stenosis. Positioning the patient Trendelenburg can transiently reduce intracardiac obstruction, improve cardiac output and venous return to the heart. Optimum patient positioning and management will lead to excellent outcomes.
| S-EPMC8606837 | biostudies-literature
Project description:Cardiac myxomas are benign tumors that commonly arise within the left atria. Familial cardiac myxomas are a part of Carney Complex (CNC), an autosomal dominant multiple neoplasia syndrome caused by germline mutations in PRKAR1A. Seven percent of cardiac myxomas are associated with CNC. To date, the genetic basis of isolated cardiac myxomas (ICM), however, has not been fully elucidated.We investigated the genetic profile of ICM using whole exome sequencing (WES). Suspected mutations were confirmed using targeted sanger sequencing. To further examine the presence of PRKAR1A mutations in ICM, we performed targeted sequencing in an additional 61 ICM specimens.87.5% (7/8) of ICM harbored mutations in PRKAR1A. Three of the 8 ICM harbored biallelic somatic mutations of PRKAR1A, including c.607_610del:p.Leu203fs (pathogenic) + c.C896G:p.Ser299X (pathogenic), c.952delT:p.Leu318fs (pathogenic) + c.769-2 A>G (pathogenic) and c.178-1 G>C (pathogenic) + c. 550+1 G>C (pathogenic). Four of 8 tumors harbored monoallelic PRKAR1A mutations, including c.523_524insG:p.Tyr175_Val176delinsX (pathogenic), c.C920A:p.Ser307X (pathogenic), c.30delG:p.Glu10fs (pathogenic) and c.C289T:p.Arg97X (pathogenic). No identical variants were observed across the 8 ICM samples. Interestingly, none of these variants have been previously described in familial cardiac myxomas. In order to confirm our findings, directed sequencing of 61 ICM specimens was subsequently performed. Sixty-four percent (39/61) of ICMs tumors contained inactivating PRKAR1A mutations.Our findings suggest that loss-of-function mutations of PRKAR1A may play a vital role in the formation of isolated cardiac myxomas.
| S-EPMC5732779 | biostudies-literature
Project description:Introduction:Myxoma is the most common cardiac benign tumour. While a typical myxoma is generally a straightforward diagnosis, some myxomas have unusual features that can make the diagnosis challenging. Glandular myxomas and metastatic adenocarcinomas, the most common type of metastatic carcinoma to the heart, can have very similar features. Case presentation:We report a 60-year old man who presented with progressive shortness of breath on exertion. Echocardiography demonstrated a large heterogeneous, cystic left and right atrial mass. He was referred for surgery where a smooth and multilobulated left atrial mass was excised. Histopathological analysis with special stains revealed an unusual form of cardiac myxoma with extensive glandular differentiation. Discussion:Cardiac myxomas can present with diverse clinical, radiological, and pathological features. Echocardiography is a modality of choice for diagnosis but can also miss small or multiple masses. Cardiac myxoma with glandular features is a rare type of myxoma. In our case, there was extensive glandular differentiation and the echocardiographic appearance provided clues for the unusual features of the tumour, suggesting a potential role for echocardiography in the detection and recognition of this morphologic histologic variation.
| S-EPMC6177082 | biostudies-literature
Project description:A 73-year-old woman presented to the emergency department with a syncopal episode and a history of dizzy spells. A transthoracic echocardiogram demonstrated a large left atrial mass extending into the right upper pulmonary veins. Subsequently, cardiac magnetic resonance imaging and coronary computed tomography angiography with three-dimensional reconstruction and printing of the heart and mass were performed, which demonstrated a high index of suspicion for an atypical left atrial myxoma. The mass was excised robotically, and the pathology report confirmed a diagnosis of myxoma.
| S-EPMC10847281 | biostudies-literature
Project description:BackgroundPrimary pericardial mesothelioma is an extremely rare disease. Prognosis is poor, with little effects of chemo- or radio-therapy. The majority of cases is diagnosed at autopsy.Case summaryA 22-year-old man, who presented with recurrent pericarditis and large pericardial effusion 2 months after a second BNT162b2 COVID-19 vaccine, underwent pericardiocentesis and pericardial window. Pathology specimen of pericardium revealed benign mesothelial inflammation, consistent with acute pericarditis. Four months later, he presented with a large pericardial mass manifesting in heart failure and underwent urgent pericardiectomy. A new pathology specimen immunostaining and fluorescence in situ hybridization analysis revealed pericardial mesothelioma. Despite intensive care, the patient died 3 weeks later.DiscussionPrimary pericardial mesothelial should be considered in the differential diagnosis of refractory recurrent pericarditis, even with prior biopsy-proven pericarditis or when a putative trigger (COVID-19 mRNA prior vaccination) is suspected, as was the case in this patient. Tumour diagnosis and identification consist of multimodal imaging and laboratory tests. A multidisciplinary, individualized care approach should be performed.
| S-EPMC10019824 | biostudies-literature
Project description:BackgroundBenign cardiac tumours are infrequent in clinical practice and, of these, cardiac myxoma is the one with the highest incidence. Given that a left intraventricular presentation is rare, other differential diagnoses such as papillary fibroelastoma should be considered.Case summaryA 73-year-old woman patient with cardiac mass detected in transthoracic echocardiography (TTE) after a transient ischaemic attack. At TTE 2D-3D, a left intraventricular mass anchored at the level of the anterolateral papillary muscle was detected. Subsequently, cardiac magnetic resonance (CMR) was performed for mass characterization. This revealed behaviour in T1 (isointense with respect to myocardium), T2 (hyperintense), very prolonged T1-mapping (1848 msg), and T2-mapping (161 msg) values, without gadolinium uptake in the first-pass perfusion sequence, but with intense uptake in late enhancement sequences. Previous findings were compatible with a diagnosis of papillary fibroelastoma. The mass was resected intraoperatively and, although its macroscopic appearance pointed to a diagnosis of cardiac myxoma, it was finally confirmed to be a papillary fibroelastoma by pathological anatomy.DiscussionIn cases where the size of the mass and its mobility allow tissue characterization by CMR, a diagnosis of papillary fibroelastoma and its differentiation with cardiac myxoma are feasible by this cardiac imaging technique.
| S-EPMC9351726 | biostudies-literature
Project description:BackgroundAtrial myxomas are the most common benign cardiac tumours. Clinical manifestations vary from constitutional symptoms, to valvular stenosis and embolic events, and surgical removal is the only suggested treatment.Case summaryA 50-year-old female patient was referred to our centre for surgical excision of a reported right atrial mass. A transoesophageal echocardiographic exam revealed two right atrial masses and the surgical plan was total removal of both masses. However, during the operation the surgeons were not able to locate the larger of the two masses and embolization to the pulmonary trunk was considered as the most likely explanation in this setting. A control suction of the right ventricle and the proximal part of the pulmonary arteries was performed to check if the mass had embolized distally but this did not yield any mass. The patient was transferred to the intensive care unit and remained stable for 2 h, until she developed an abrupt cardiogenic shock with signs of right heart failure. An emergent pulmonary computed tomography angiography demonstrated the embolized mass to the left pulmonary artery and the patient was retransferred to the operating room for emergent surgical removal of the mass. The patient showed immediate clinical and haemodynamic improvement after the removal of this mass and had an uneventful further hospitalization.DiscussionMultiple right atrial myxomas are rarely reported and surgical excision requires experience, as in case of embolization immediate removal must be obtained to prevent from right ventricle distress and cardiogenic shock.
| S-EPMC7793044 | biostudies-literature
Project description:Aspergillary rhinopharyngitis in an equine patient
| PRJEB46193 | ENA
Project description:Primary heart neoplasms are rare occurring with an estimated incidence of 0.0017-0.19%. Myxoma is the most prevalent primary heart tumor. The right atrium is an unusual localization, occurring only in 15-20% of myxoma cases. We report a rare case of a massive right atrial myxoma causing tricuspid valve obstruction and presenting as syncope and exertional dyspnea. This case illustrates the influence of myxoma's size, position and mobility as well as patient's body posture and respiration to the development of signs and symptoms. Three-dimensional echocardiography proved useful in surgery planning, allowing a better definition of the tumor outline and attachment.
| S-EPMC2894750 | biostudies-literature