• Journal of Chest Surgery
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• v.54 no.5
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• pp.422-424
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• 2021

Malignant peripheral nerve sheath tumors are rare sarcomas of the heart. Herein, we report the case of a 24-year-old man who complained of dyspnea, cough, and upper left back pain. He was found to have multiple primary heart tumors obstructing the right superior pulmonary vein in the left atrium, which were diagnosed as malignant peripheral nerve sheath tumors. The patient underwent successful resection of the tumors and immunohistochemistry was utilized for diagnosis.

• Journal of Chest Surgery
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• v.42 no.6
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• pp.770-773
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• 2009

Rhabdomyoma has been reported to be the most common type of cardiac tumors in fetuses and children, and multiple cardiac rhabdomyomas almost certainly signify the association with tuberous sclerosis. We report here on a case of Tetralogy of Fallot (ToF) that was associated with multiple rhabdomyomas and tuberous sclerosis. A two-year-old boy, who had undergone systemic-pulmonary shunt during the neonatal period, received total correction of his ToF after the complete regression of the cardiac tumor. The postoperative course was uneventful, and he has been follow-up for 2 months. The boy is currently in an excellent condition.

• Journal of Chest Surgery
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• v.41 no.4
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• pp.496-498
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• 2008

Cardiac papillary fibroelastomas are the second most common primary cardiac tumor. This tumor is usually benign and it involves the cardiac valve. However, most cardiac papillary fibroelastomas originate from a single site, and the incidence of cardiac papillary fibroelastomas originating from multiple sites is very rare (5%). A 55-year-old woman who presented with momentary dizziness and syncope was evaluated by performing echocardiography. Multiple tumors attached to the aortic valve were noted. The mass was removed freely without leaving any defect on the aortic valve leaflet. After the recovery period, the patient is currently being followed up at the outpatient department.

• Clinical and Experimental Pediatrics
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• v.51 no.7
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• pp.766-770
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• 2008

Primary tumors of the heart are uncommon among pediatric patients. Rhabdomyoma is the most frequent cardiac tumor in infants and children, which is commonly associated with tuberous sclerosis. Tuberous sclerosis is a neurocutaneous syndrome affecting the brain, heart, skin, and other organs. Cardiac rhadomyomas are reported in 50-64% of infants with tuberous sclerosis. Tuberous sclerosis involves multiple locations in the atrium, ventricle and septum, and may induce mechanical obstruction of the outflow tract and heart failure depending on the location, number, size, and degree of invasion of tumors. Arrhythmias may also develop in infants with cardiac rhadomyomas, but only a few of these patients require prolonged anti-arrhythmic therapy because arrhythmia often disappears with spontaneous regression of the tumors, and the ultimate prognosis may be decided by the cerebral manifestations.

• Journal of the Korean Society of Radiology
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• v.82 no.5
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• pp.1321-1327
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• 2021

Neurofibromatosis type 1 (NF1) is a relatively common inherited disorder characterized by the formation of neurofibromas, pigmentary abnormalities of the skin, Lisch nodules of the iris, and skeletal abnormalities. Multiple cutaneous neurofibromas are benign nerve sheath tumors and the main manifestation of NF1. Cardiac neurofibroma associated with NF1 is very rare, and few cases have been reported in the literature. Herein, we present the CT and MRI findings of a surgically confirmed left ventricular neurofibroma in a 32-year-old female with NF1.

• Journal of Chest Surgery
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• v.35 no.10
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• pp.750-754
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• 2002

Primary cardiac tumors are rare and about 20~25% of primary cardiac tumors are malignant. Moreover, primary malignant mesenchymoma of the heart is extremely rare. Recently, we have experienced two cases of cardiac malignant mesenchymoma. In the first case, malignant mesenchymoma which was originated from the posterior wall of the left atrium obstructing the mitral orifice was revealed pathologically in a 61-year-old woman with mitral regurgitation. The mass, which was 2.7$\times$3.7cm in size on the posterior wall of left atrium, was extended to the posteromedial commissure and annulus of the mitral valve. The mass was resected partially without excision of the left atrial free wall. She was discharged after 30 days without any problems and she received chemotherapy and followed up for 19months. The second case was a 4$\times$5cm in size, friable, yellow-whitish multilobulated mass in the left atrium which was originated from the left lower pulmonary vein. Multiple minor tumor nodules were found in the wall of the left atrium and the posterior leaflet of mitral valve. Partial mass excision and mitral valve replacement were performed.

• Journal of Chest Surgery
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• v.43 no.6
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• pp.725-728
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• 2010

Rhabdomyoma is the most common benign cardiac neoplasm in neonates. Most patients with rhabdomyoma experienced spontaneous regression. Yet some of them need surgical therapy because of hemodynamic problems of the heart such as arrhythmia, outflow tract obstruction and valvular dysfunction. We found multiple masses in both ventricles on the patient's fetal echocardiogram. Heart failure caused by severe left ventricular outflow tract obstruction quickly presented after birth. The mass interfering with the outflow tract was resected via the transaortic approach at the first day of birth. Postoperative echocardiography showed complete release of the outflow tract obstruction. He was discharged on the postoperative day 8. During the 3 years of follow up, we found that the sizes of the remnant masses had gradually decreased.

• Clinical and Experimental Pediatrics
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• v.50 no.10
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• pp.1024-1029
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• 2007

Costello syndrome (CS) is a rare multiple congenital abnormality syndrome characterized by a typical coarse face, developmental delay, psychomotor and growth retardation, neurologic abnormalities, cardiac and cutaneous anomalies, severe feeding difficulties with postnatal growth failure, and increased risk of tumors. Since Costello first described it in 1971 and again in 1977, over 100 cases have been reported worldwide. It was recently shown that CS is a congenital condition caused by heterozygous de novo missense mutations affecting the codon for glycine 12 or 13 of the HRAS gene. We experienced three unrelated cases with coarse faces, developmental delays, short statures, macrocephaly, and redundant skin with deep palmar and plantar creases, hypertrophic cardiomyopathy and atrial tachycardia, which are characteristic of CS.