Benign intracardiac thyroid mass causing right ventricular outflow tract obstruction.
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A 62 years old man with Child B liver cirrhosis, prostate cancer and a recent colon carcinoma resection was referred to our cardiology department for trans-thoracic-echocardiography (TTE) in order to establish left ventricular function before starting chemotherapy. TTE revealed a mobile mass (16 x 8 mm) attached to the anterior-medial left ventricular wall, protruding and swinging within the left ventricle cavity. At follow-up TTE showed growing of the intra-cardiac tumor up to 27 x 10 mm, corresponding to a size increase of 1 mm/month. Among different pathologies a rapid growing benign tumor with a high risk of systemic embolisation or an endocardial blood cyst were retained as possible diagnoses. Given the progression of the cardiac finding and the patient's improved general condition, surgical resection of the cardiac mass was performed. Histological examination revealed a mixed capillary/cavernous hemangioma. This case shows the unusual concomitant appearance of a rapid growing cavernous hemangioma which rarely located at ventricular level and the feasibility of cardiac resection without further sequelae in a poly-morbid patient.
We operated on a 34-year-old man with a metastatic tumor that extended from the tricuspid valve to the pulmonary valve and obstructed the right ventricle inflow and outflow tracts. The tumor was removed with preservation of the tricuspid valve. Additional chemotherapy was carried out according to the BEPO (etoposid, eisplatin, bleomycin, vincritin) scheme. Histology revealed metastasis of a choriocarcinoma originating from the right testis. Computed tomography performed after 6 months detected no metastases in the lungs. Magnetic resonance imaging showed a thickened right ventricle free wall and apex. The patient is doing well 18 months postoperatively.
We report an unusual study of a 72-year-old woman who presented with a symptomatic space-occupying lesion in the left ventricle. The lesion was surgically excised with a good long-term result. It was histologicaly diagnosed as an inflammatory myofibroblastic tumor. Also known as an inflammatory pseudotumor, these tumors affecting the heart are extremely rare and occur mainly in children and adolescents. We review the literature and highlight the unique nature of the lesion.
BACKGROUND: Intravenous leiomyomatosis is a rare variant of leiomyoma. CASE: The patient was a 49-year-old gravida 3, para 3 woman with menopause at age 46. She presented with a history of syncope. Vaginal examination revealed an enlarged and elastic-soft mass of the uterus. A pelvic ultrasound, computed tomography scan, and magnetic resonance imaging showed a heterogeneous, irregularly shaped 8- to 10-cm tumor. In addition, the inferior vena cava was almost completely occluded. Cardiac ultrasound demonstrated a mobile mass in the right atrium. The serum estradiol was 208 pg/mL (normal 0-59). Intravenous leiomyomatosis with cardiac extension was diagnosed preoperatively. A resection of the intracardiac and intracaval mass and a subtotal hysterectomy with bilateral salpingo-oophorectomy were performed. The uterine tumor weighed 600 g, and the cordlike intravascular tumor extending from the internal iliac vein into the right ventricle was 40 cm long and weighed 60 g. Pathologic examination confirmed intravenous leiomyomatosis with no evidence of atypia. The level of estrogen receptor in the tissue was 140 fmol/mg protein. The postoperative course was uneventful, and she has been in good health for 17 months after the operation. CONCLUSION: We report a case of intravenous leiomyomatosis extending into the right ventricle treated with a one-stage operation. It is possible that a high concentration of serum estradiol and high level of tissue estrogen receptor are related to the intravenous leiomyomatosis.
OBJECTIVE: Primary cardiac tumours are rare. The literature predominantly contains series on myxomas in adults and only a few long-term series that involve the very different primary cardiac tumours in early childhood. As foetal ultrasonography has continued to improve, cardiac tumours are increasingly detected early before significant symptoms develop. It is a challenge for paediatric cardiologists and surgeons to ascertain which patients need surgery and which will benefit from conservative follow-up. METHODS: A retrospective review of a 10-year period revealed 51 tumours in 26 children (median age: 1 month). Analysis was by presentation, location, associated findings, interventions, histological findings, and clinical course. RESULTS: The most common tumours were rhabdomyomas (29), fibromas (nine), teratomas (two), and haemangiomas (two). The tumour location was the right ventricle in 24 and the left ventricle in 22 patients. The symptoms varied between abnormal heart murmur (20), arrhythmia and conduction abnormalities (ten), obstruction of the outflow tract >30 mmHg (nine), severe cyanosis (three) and congestive heart failure (two). Fourteen children with haemodynamic compromises underwent surgery. There was one post-operative death and one heart transplantation after bridging with an assist device. There was no tumour recurrence even when resection was incomplete. Nine of 13 children with rhabdomyomas had spontaneous tumour regression without intervention. CONCLUSIONS: Most of the cardiac tumours in children are benign. Spontaneous regression is possible not only in rhabdomyoma. Surgical intervention is only required for children who develop relevant clinical symptoms. Total resection of the tumour is not the only therapeutic aim; more important is the restoration of the best possible heart function.
In a 55-year-old woman (10 years after subtotal thyroidectomy for follicular adenoma) echocardiography revealed a 25 x 23-mm tumour in the right ventricular outflow tract. The successfully removed tumour appeared to be a follicular carcinoma. Subsequently, there has been no clinical and laboratory evidence for another site of metastasis or ectopic thyroid. The whole body 131I scan showed only correct radioiodine uptake in the place of cervical residual thyroid gland. We believe this is the first description of follicular carcinoma in cardiac ectopic thyroid.
Summary Wilms' tumour (nephroblastoma) is known to invade the inferior vena cava and extend to the intracardiac chambers. We describe the treatment and surgical removal of the intracardiac extension of a Wilms' tumour using cardiopulmonary bypass in a 4-year-old girl. Techniques to avoid paradoxical embolism in the presence of a patent foramen ovale and to deal with excessive hepatic venous blood flow using the Pringle manoevre (hepatic inflour occlusion) are described. Good communication between anaesthesiologists, surgeons and perfusionists was indispensable. The anaesthesiologist is an important member of the team during performance of a complicated procedure.
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A rare case of metastatic soft tissue sarcoma (STS) involving the endocardium of the left ventricle of the heart is described. A 57-year-old man with a previously resected synovial sarcoma of the anterior abdominal wall presented 5 years later with an acute ischaemic arm resulting from tumour embolus. The treatment and outcome of the patient are outlined. Metastatic STS cardiac involvement and management of this complication are reviewed.
Six cases of primary cardiac tumour have been operated upon in a 7 year period from 1 June 1979 until 1 June 1986. All patients were under 6 months of age at the time of operation and two of the patients were in their first week of life. The principal indication for surgery was obstruction mainly at the right or left ventricular outflow tract level. More recently echo evaluation alone has been adequate to define the problem prior to surgery. Surgical excision has been performed without mortality or significant complications. In most cases resection has been complete, although in one case residual tumour has been left because of attachment of the tumour to vital structures. Follow-up of this case has not resulted in further surgery being required because of regrowth of the tumour. In one case, with co-existent congenital heart disease, the tumour was brought to notice after palliative systemic to pulmonary artery shunt had been performed. From the cardiac view point gratifying results have been obtained both in the short and long term following surgical resection. However, for patients with rhabdomyoma, later development of symptomatic tuberosclerosis should be anticipated in 50% of cases.
We describe the case of a patient with the Carney syndrome and several resections for recurrent left atrial myxomas who underwent autotransplantation of the heart with resection of the left and right atria and reconstruction of both atria with bovine pericardium. She subsequently presented with severe shortness of breath, ascites, and peripheral edema. She was found to have stenosis of all four pulmonary veins and severe pulmonary hypertension. We describe the echocardiographic findings and review the literature on assessment of acquired pulmonary vein stenosis.
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The association of fibrin and tumour cells on a sclerosed mitral valve in a 62-year-old woman is described. This was the first indication of malignant disease but bilateral ovarian cancer was proved two months later. ino further tumour deposits have been found in fifteen months. The tumour deposit on the valve was most likely a metastasis but primary heart valve sarcoma has not been positively excluded. If the lesion was a secondary deposit this has possible implications for the role of fibrin in metastasis in humans.
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