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儿童和成人心脏肿瘤:来自一个外科中心的59例患者的临床病理研究

Heart tumors in children and adults: clinicopathological study of 59 patients from a surgical center.

作者信息

Thomas-de-Montpréville Vincent, Nottin Rémi, Dulmet Elisabeth, Serraf Alain

机构信息

Department of Pathology, Marie Lannelongue Surgical Center, 92350 Le Plessis Robinson, France.

出版信息

Cardiovasc Pathol. 2007 Jan-Feb;16(1):22-8. doi: 10.1016/j.carpath.2006.05.008.

Abstract

BACKGROUND

Heart tumors are rare lesions with variegated histological types. Their clinicopathological features could be more comprehensively categorized.

METHODS

This is a 19-year retrospective study of 17 infants/toddlers (<2 years of age) and 42 patients aged between 14 and 79 years (mean = 51.5) in a surgical center.

RESULTS

Congenital tumors (n = 17; 29%), including rhabdomyomas (n = 9), ventricular fibromas (n = 6), and hemangiomas (n = 1), required surgery mainly because of mass effect. Familial myofibromatosis was the only embolic congenital lesion. Acquired benign tumors (n = 28; 47%) included myxomas (n = 21), fibroelastomas (n = 3), myofibroblastic inflammatory tumors (n = 2), and lipomas (n = 2). Eight (29%) were revealed by systemic embolization. These benign noncongenital tumors were all treated by complete resection, except for an incompletely resected lipoma of the mitral valve. Postoperative arrhythmia (n = 1) and pericardial effusion (n = 3) were the only complications. Primary sarcomas (n = 8; 14%) were mostly vascular tumors (five of eight), and patients with high-grade tumors had a mean survival of 15 months (n = 5). Cardiac metastases (n = 6; 10%) were from carcinomas (n = 3) or sarcomas (n = 3); apart from a necrotic metastasis, all patients died (mean survival of 6 months).

CONCLUSIONS

This study shows that, regardless of patients' age, heart tumors can be classified as: (a) congenital lesions, which are spontaneously nonprogressive or regressive lesions possibly requiring surgery mainly because of mass effect; (b) acquired benign tumors, which are lesions requiring surgery often because of embolization risk; and (c) primary and secondary malignant tumors, which are lesions with globally poor prognosis but with some indications for resection.

摘要

背景

心脏肿瘤是组织学类型多样的罕见病变。其临床病理特征可得到更全面的分类。

方法

这是一项在一个外科中心进行的为期19年的回顾性研究,研究对象为17名婴儿/幼儿(<2岁)和42名年龄在14至79岁之间(平均 = 51.5岁)的患者。

结果

先天性肿瘤(n = 17;29%),包括横纹肌瘤(n = 9)、心室纤维瘤(n = 6)和血管瘤(n = 1),主要因占位效应而需要手术。家族性肌纤维瘤病是唯一的栓塞性先天性病变。后天性良性肿瘤(n = 28;47%)包括黏液瘤(n = 21)、纤维弹性瘤(n = 3)、肌纤维母细胞性炎性肿瘤(n = 2)和脂肪瘤(n = 2)。其中8例(29%)通过全身栓塞发现。这些良性非先天性肿瘤均通过完整切除进行治疗,但二尖瓣的1例脂肪瘤切除不完全。术后心律失常(n = 1)和心包积液(n = 3)是仅有的并发症。原发性肉瘤(n = 8;14%)大多为血管性肿瘤(8例中的5例),高级别肿瘤患者的平均生存期为15个月(n = 5)。心脏转移瘤(n = 6;10%)来自癌(n = 3)或肉瘤(n = 3);除1例坏死性转移外,所有患者均死亡(平均生存期6个月)。

结论

本研究表明,无论患者年龄如何,心脏肿瘤可分为:(a)先天性病变,为自发非进行性或退行性病变,可能主要因占位效应而需要手术;(b)后天性良性肿瘤,为常因栓塞风险而需要手术的病变;(c)原发性和继发性恶性肿瘤,为总体预后较差但有一些切除指征的病变。

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