心脏肺动脉脂肪母细胞瘤：罕见的发生在不寻常部位的肿瘤。
Cardiac lipoblastoma of the pulmonary valve: A rare tumor in an unusual location.
机构信息
Department of Cardiac Surgery, Beijing Children's Hospital affiliated to Capital Medical University, National Center for Children's Health, Beijing, China.
出版信息
J Card Surg. 2021 Jul;36(7):2589-2591. doi: 10.1111/jocs.15580. Epub 2021 Apr 24.
BACKGROUD
Lipoblastoma is a rare, benign, fatty tissue tumour tumor that occurs in infancy and early childhood. Intrathoracic and mediastinal involvement of this tumour is rare, and an intracardiac location is even rarer.
AIMS
To report the first case of a lipoblastoma in the pulmonary valve.
MATERIALS
In this study, we report the case of a 15-month-old girl who presented with a tumour in the pulmonary valve.
RESULTS
The tumour was completely resected macroscopically. Histological examination of the lesion confirmed the diagnosis of lipoblastoma.
DISCUSSION
Most primary cardiac tumours are benign in the sense they are not invasive. However, benign tumours maintain the potential for serious illness related to significant haemodynamic compromise or life-threatening dysrhythmias. Due to the association of lipoblastoma with other heart malformations, preoperative diagnosis remains challenging.
CONCLUSION
Tumours on the heart valve are more likely to cause haemodynamic disturbances. Complete resection yields an excellent prognosis.
背景
脂肪母细胞瘤是一种罕见的良性脂肪组织肿瘤，发生于婴儿和幼儿期。这种肿瘤在胸腔和纵隔内的累及较为罕见，而发生于心内者则更为罕见。
目的
报告首例发生于肺动脉瓣的脂肪母细胞瘤。
材料
本研究报道了 1 例 15 个月大的女孩，其肺动脉瓣有一肿瘤。
结果
肿瘤大体上完全切除。病变的组织学检查证实了脂肪母细胞瘤的诊断。
讨论
大多数原发性心脏肿瘤在无侵袭性的意义上是良性的。然而，良性肿瘤仍有发生严重疾病的可能，与显著的血流动力学障碍或危及生命的心律失常有关。由于脂肪母细胞瘤与其他心脏畸形有关，术前诊断仍然具有挑战性。
结论
心脏瓣膜上的肿瘤更有可能引起血流动力学紊乱。完全切除可获得良好的预后。
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