| Popis: |
Asymptomatic 2 month-old infant referred for evaluation of a hard abdominal mass on the left side. The ultrasound examination showed a solid-cystic tumour above the left kidney. The alpha-fetoprotein level was 2000ng/ml. The meta-iodobenzylguanidine (123-I-MIBG) showed no tumour uptake. At 48h, she showed signs of hypovolemic shock. The chest X-ray showed cardiomegaly with a cardiothoracic ratio of 0.7 and pulmonary congestion. The echocardiogram showed a dilated left ventricle with ejection fraction 35-40%. Anaemia, hypertension, hypervolemia and hyper-secretion of catecholamines were ruled out. The virology and metabolic screens were normal. The highly vascularised retroperitoneal tumour was resected without incident and confirmed the diagnosis of an immature Norris grade 2 teratoma grade. At 3 months the outcome was satisfactory. Teratomas are rare tumours in childhood that generally have a benign course. Dilated cardiomyopathy (DCM) secondary to, chromaffin cell tumours (phaeochromocytoma, neuroblastoma, ganglioneuroblastoma), leukaemia infiltrates, and treatment with anthracyclines have been described, but there is no case reported in the literature regarding a teratoma with dilated cardiomyopathy. Various cytokines, such as INF-α, IL-1, IL-6 may be secreted by tumour, promoting fibroblast activity in the heart and inducing apoptosis and myocardial fibrosis. Thus, in the case presented resection of the tumour mass responsible for this production, enables the heart to return to normal. |
