Recurrent Cardiac Myxoma Research Articles

Abstract 15843: Biatrial and Recurrent Cardiac Myxoma in a Rare Case of Carney Complex

Primary cardiac tumors (CTs) are exceptionally rare, and are predominantly benign masses. Myxomas make up more than 60% of these lesions and most frequently develop in the left atrium (LA), followed by the right atrium (RA). Carney complex (CNC) is a rare, typically autosomal dominant, multiple endocrine neoplasia characterized by cutaneous findings, myxomas, endocrine and/or nonendocrine neoplasms. We report a rare case of CNC involving biatrial and recurrent myxomas. A 36-year-old female presented to Cardiology Clinic at our center for management of CNC. Three years prior, she was evaluated at a local hospital for acute stroke where TTE showed a myxoma (2 x 2 x 2.5cm) attached to the left atrial septum, prompting resection. On followup TTE 2 years later, she had a mass in the LA, prolapsing through the mitral valve into the left ventricle. She underwent resection of the myxoma (2.9 x 3.9 cm) and left atrial appendage, which was complicated by recurrent stroke. She was found to have a PRKAR1A gene mutation (c.97 deletion) suggestive of CNC and was referred to our center. At Cardiology Clinic, she denied chest pain, dyspnea, presyncope, and syncope. Her father and children did not have the gene mutation. Exam revealed lentigines on her lower lip and pigmentation of an inner canthus. She was diagnosed with primary pigmented nodular adrenal disease by Endocrinology. On TEE, she was found to have a recurrent LA mass (1 x 1 x 0.8 cm) attached to the interatrial septum, but no right-to-left atrial shunt. This prompted surgery consultation. Numerous intracardiac myxomas were removed during surgery; one in the LA attached to the septum, 3-4 smaller ones, and one 2 cm myxoma located in the RA. The interatrial septum was also resected given recurrent myxoma in this region. She underwent cryoablation to prevent recurrence and did well post-surgery. CTs, which includes primary CTs and secondary metastatic disease, are uncommon where the former has an incidence of 0.001-0.3%. We presented a case of recurrent and biatrial cardiac myxomas in the setting of CNC, which is seldomly reported in the literature. Taken together, given the rarity of primary CTs, genetic predispositions or syndromes should be considered especially in the setting of recurrence and/or multiple masses.

Recurrent multi-cameral cardiac myxomas in a child with Carney complex.

Cardiac myxoma is a relatively rare tumour, usually solitary, that occurs primarily in the left atrium of adults, but comprises only 30% of cardiac tumours in children. We recently treated a 12-year-old girl with multiple recurrent myxomas in three cardiac chambers(following surgical resection 3 years earlier). Genomic analysis showed the PKAR1A mutation typical for Carney complex.

Recurrent Cardiac Myxoma: A Puzzle to be Solved.

Recurrent Cardiac Myxoma: A Puzzle to be Solved.

Diagnosis of Carney complex following multiple recurrent cardiac myxomas

Carney complex is a rare syndrome caused by a genetic mutation leading to multiple endocrine abnormalities and a variety of tumors. Here, we report a case of Carney complex diagnosed due to recurrent multiple myxomas in the right atrium of a patient 16 years after the resection of the primary left atrial myxoma. Surgical excision was performed for the multiple recurrent right atrial tumors under cardiopulmonary bypass. The patient remained complication-free after surgery and was discharged on the 14th day. He was scheduled to continue echocardiographic follow-up and periodic systemic review by an endocrinologist. This case emphasizes the fact that if cardiac myxomas tend to be multiple and recurrent at a relatively young age, the possibility of Carney complex should be considered, even in the absence of any other related feature other than cardiac tumors.

Recurrent Cardiac Myxoma Treated by Heart Transplantation

Recurrent Cardiac Myxoma Treated by Heart Transplantation

À propos d’un cas de myxome cardiaque avec éléments glandulaires récidivant chez un enfant

Primary cardiac tumors are extremely rare and mainly benign. The majority of these are myxomas (40%). Myxoma are generally sporadic tumors which occur most commonly in adult females between 30 and 40 years, and are seldom found in the paediatric population (5%). Seven percent are associated with igenetic diseases. We report the case of an eight-year-old boy presenting a recurrent glandular cardiac myxoma. In 2011, he presented a deterioration of the general state. An echocardiography highlighted a left atrial mass on the interatrial septum, with a pedicular insertion. On the microscope, it consisted of a proliferation of stellate cells isolated or clustered in rudimentary vessels in a myxoid stroma presenting haemorrhage changes. These cells expressed CD34 and calretinine. Glandular elements without atypia were clustered within the myxomatous proliferation. They expressed cytokeratin (CK) 7. Surgical resection was macroscopically complete. In 2014, the boy had a sudden neurological deficit during a football match. An echocardiography revealed a recurrence at the same location. The lesion was excised and addressed in several fragments. Classical myxoma was associated with glands without atypia. This last component expressed CKAE1/AE3 and CK7. Ki67 index of proliferation was low. The surgical reintervention was macroscopically complete. The final diagnosis was glandular cardiac myxoma. A genetic survey was conducted, showing the presence of Carney complex. This is the first description in the litterature of a recurrent glandular cardiac myxoma occuring in a child.

Recurrent cardiac myxoma in a 25 year old male: a DNA study

We present a 25 year old Caucasian male patient with multiple recurrences of cardiac myxomas after surgical removal of the original tumor. His mother was operated on for right ventricular myxoma. The genetic analyses disclosed an aneuploid DNA content by flow cytometry analysis. The familial form of the cardiac myxomas must be distinguished from Carney complex syndrome. A long- term echocardiographic follow up is recommended to patients and their first degree relatives with cardiac myxomas.

Sudden Death in a Patient with Carney's Complex

Carney’s complex is a rare autosomal dominantly inherited multiple endocrine neoplasia syndrome that involves spotty skin pigmentations, recurrent cardiac myxomas, endocrine hyperactivity, pituitary adenomas, peripheral nerve tumors, testicular tumors, and ovarian lesions. We present a case of sudden cardiac death in a 40 year old female with a history of Carney’s complex with recurrent cardiac myxomas presenting for exploratory laparotomy and enblock adnexal resection of a slowly enlarging right sided ovarian mass. This case highlights the risk for sudden death in these patients as well as the preoperative assessment that should be undertaken by the anesthesiologist as it relates to Carney’s complex.

Resection of Multiple Recurrent Cardiac Myxomas in an Adult Man With Carney's Complex

Cardiac myxomas have many manifestations, including an association with endocrine overreactivity, unusual testicular tumors, and manifestations in Carney's complex [1McCarthy P.M. Piehler J.M. Schaff H.V. et al.The significance of multiple, recurrent, and “complex” cardiac myxomas.J Thorac Cardiovasc Surg. 1986; 91: 389-396PubMed Google Scholar]. We recently treated a 40-year-old man who presented with an embolic stroke. He had a family history of cardiac myxoma and underwent resection of a right atrial myxoma 7 years earlier.Echocardiography revealed multiple intracardiac masses in the left atrium (Fig 1A , arrow), right atrium, and right ventricle (Fig 1B, arrow). An ultrasound examination of the scrotum showed multiple bilateral testicular calcifications (Figs 2A, 2B), thought to be calcifying Sertoli cell tumors. A computed tomography scan of the abdomen showed no abnormalities, and the 24-hour urine cortisol level was within the normal reference range. Genetic evaluation revealed a heterozygous mutation in the PRKAR1A gene.Fig 2View Large Image Figure ViewerDownload (PPT)The patient underwent excision of 4 cardiac myxomas (left atrium, right atrium, and right ventricle; Fig 3) using cardiopulmonary bypass and standard aortic and bicaval cannulation. His postoperative course was unremarkable, and he was dismissed on the fourth postoperative day.Fig 3View Large Image Figure ViewerDownload (PPT) Cardiac myxomas have many manifestations, including an association with endocrine overreactivity, unusual testicular tumors, and manifestations in Carney's complex [1McCarthy P.M. Piehler J.M. Schaff H.V. et al.The significance of multiple, recurrent, and “complex” cardiac myxomas.J Thorac Cardiovasc Surg. 1986; 91: 389-396PubMed Google Scholar]. We recently treated a 40-year-old man who presented with an embolic stroke. He had a family history of cardiac myxoma and underwent resection of a right atrial myxoma 7 years earlier. Echocardiography revealed multiple intracardiac masses in the left atrium (Fig 1A , arrow), right atrium, and right ventricle (Fig 1B, arrow). An ultrasound examination of the scrotum showed multiple bilateral testicular calcifications (Figs 2A, 2B), thought to be calcifying Sertoli cell tumors. A computed tomography scan of the abdomen showed no abnormalities, and the 24-hour urine cortisol level was within the normal reference range. Genetic evaluation revealed a heterozygous mutation in the PRKAR1A gene. The patient underwent excision of 4 cardiac myxomas (left atrium, right atrium, and right ventricle; Fig 3) using cardiopulmonary bypass and standard aortic and bicaval cannulation. His postoperative course was unremarkable, and he was dismissed on the fourth postoperative day.

Familial cardiac myxoma with multifocal recurrences: a case report and review of the literature

We report a case of myxoma with multiple recurrences in both the atrium and ventricle in a 26-year-old woman five years after the surgical removal of left atrial myxoma. Her 52-year-old mother had a similar medical history. To our knowledge, this was the first familial case who suffered multifocal cardiac myxoma recurrences without any sign of the myxoma complex. Based on our understanding of the mechanism of recurrence, the approaches to prevent the recurrence, and markers to predict recurrence, we propose that multifocal recurrences, as reported herein, may result from a combination of familial predisposition and multifocal onset. The bi-atrial surgical approach and transesophageal echocardiography are preferred for patients with recurrent cardiac myxomas, especially for those with multiple recurrences and familial myxoma. Immunological and genetic screenings may help to identify family members at risk for developing this disease.

Cardiac Myxomas: Experience Over One Decade

This single-center study reviews our experience with cardiac myxomas over the past decade. Sixty-two patients (23 male) with median age 38 years (range: 8 to 69 years) underwent excision of primary or recurrent cardiac myxomas between 2000 and 2009. Patients were evaluated with echocardiography preoperatively and annually postoperatively. Follow-up is current for all survivors (range 13 months to 10 years). Fifty-two patients had left atrial myxomas, seven right atrial, two biatrial, and one right ventricular. Three cases were familial. Maximum number of myxomas in a single patient was four. Symptom duration ranged from two to eight months. Two early deaths were due to low cardiac output and embolic cerebrovascular accident; one late death was due to a noncardiac cause. Actuarial survival was 96.8 ± 1.8% at 10 years. Most patients were asymptomatic following surgery. No sporadic, multiple, or biatrial myxomas recurred. Recurrence occurred in two familial cases, both with single, left atrial myxoma. Freedom from reoperation was 98.4 ± 1.3% at five years and 96.8 ± 1.8% at 10 years. Biatrial involvement or multiplicity of myxomas does not mandate recurrence. Surgical excision has excellent overall survival and freedom from reoperation rates, but annual follow-up including echocardiographic surveillance is recommended as familial cases tend to recur.

Carney-Complex: Multiple resections of recurrent cardiac myxoma

We report a case of a female patient who was operated at the third relapse of an atrial myxoma caused by Carney complex. The difficult operation was performed without any complications despite extensive adhesions caused by the previous operations. The further inpatient course went without complications and the patient was discharged to the consecutive treatment on the 9th postoperative day. The echocardiographic finding postoperative showed no abnormalities.

Cardiac Autotransplantation and Radical Bi-Atrial Resection for Recurrent Atrial Myxoma

There have been sporadic reports of cardiac autotransplantation for ex-vivo resection of anatomically difficult cardiac tumors. We describe the first case of cardiac explantation, ex-vivo total biatrial resection, and reconstruction with bovine pericardial for recurrent cardiac myxoma.

A Case of Carney Complex: Diagnosed 11 Years after Resection of Recurrent Cardiac Myxomas

Carney complex (CNC) is a rare disease characterized by myxoma, spotty skin pigmentation, and multiple neuroendo crine tumors. Here, we present a case of CNC, diagnosed 11 years after resection of recurrent cardiac myxomas. We had previously reported a 37-year-old woman in 1984 as biatrial myxomas and in 1994 as recurrent left atrial and ventricular myxomas, and at those times, she did not have any other myxomas and skin lesions. On her present admission, she had skin pigmentation and myxomatosis of breast, nostril and forearm. Upon the examination of her relatives, no characteristics of CNC were found on them. She was diagnosed as a sporadic form of CNC. If cardiac myxomas tend to be multiple and recurrent, we should consider the possibility of CNC, even other characteristics of CNC except cardiac myxomas are not present at diagnosis. (Korean Circulation J 2007;37:183-186)

The Carney Complex: Unusual Skin Findings and Recurrent Cardiac Myxoma

The Carney Complex: Unusual Skin Findings and Recurrent Cardiac Myxoma

Multicentric familial cardiac myxoma

Familial cardiac myxoma is a rare syndrome which constitutes approximately 10% or less of all myxomas. We describe a rare case of LA and LV mass simultaneously in a 35-year-old female presenting to our hospital for evaluation of recurrent cardiac myxoma. Echocardiography revealed both LA and LV mass. Surgery was done and histological findings confirmed the diagnosis of myxoma.

Genetic Analysis in a Patient With Recurrent Cardiac Myxoma and Endocrinopathy

A 60 year-old male was referred for treatment of a cardiac myxoma in the right atrium. He had a past history of left atrial cardiac myxoma at age 49 and pituitary microadenoma related to acromegaly at age 55. He did not have a family history of cardiac neoplasm or endocrinopathy. The intracardiac tumor was resected and its pathology was compatible with myxoma. A diagnosis of Carney complex (CNC) was made because the diagnostic criteria of this neoplastic syndrome were satisfied by the presence of recurrent cardiac myxoma, endocrine tumor and spotty skin pigmentation. In genetic analysis novel frame shift mutation was detected in exon 2 in a heterozygous fashion in the causative gene of CNC, protein kinase A regulatory subunit 1 alpha (PRKAR1A). This genetic mutation is thought to cause haplo-insufficiency of PRKAR1A resulting in tumorigenesis. Although it is the most common, usually benign, cardiac tumor, myxoma can cause a critical clinical situation and thus detecting the PRKAR1A mutation can assist with prognosis.

Multiple cerebral aneurysms in a patient with recurrent cardiac myxomas. A case report.

A 22 year old female presented in 1987 a cardiac myxoma, removed in 1991. In 1992 catheter angiography showed bilateral aneurysms. Conservative treatment was elected. In 2000 recurrence of the myxoma and subsequent removal, prompted new angiography. All aneurysms had decreased in size, some had spontaneously disappeared.

Metastatic cardiac myxoma

A 22-year-old woman who had a history of three cardiac operations and a bilateral femoral embolectomy for recurrent cardiac myxoma and myxoma embolism, respectively, was accepted to our clinic with multiple immobile peripheral masses. One of them was compressing the left common femoral artery. This mass was extirpated. Pathology examination revealed myxoma. Chemotherapy was given to the patient and regression of the masses was observed.

Interleukin-6 production and recurrent cardiac myxoma

Interleukin-6 production and recurrent cardiac myxoma