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曾接受实体瘤治疗的儿童肝局灶性结节性增生。发病率、危险因素及预后。

Hepatic focal nodular hyperplasia in children previously treated for a solid tumor. Incidence, risk factors, and outcome.

作者信息

Bouyn Celine Icher-De, Leclere Jerome, Raimondo Graziella, Le Pointe Hubert Ducou, Couanet Dominique, Valteau-Couanet Dominique, Hartmann Olivier

机构信息

Pediatric Department, Institut Gustave-Roussy, Villejuif, France.

出版信息

Cancer. 2003 Jun 15;97(12):3107-13. doi: 10.1002/cncr.11452.

Abstract

BACKGROUND

The detection of hepatic nodules, particularly in patients treated for a previous malignancy, raises a diagnostic dilemma. Focal nodular hyperplasia (FNH) of the liver is an uncommon, benign tumor in children and must be differentiated from malignant hepatic lesions. The etiology of FNH is obscure, and its pathogenesis is poorly understood. FNH may be a reaction to localized vascular abnormalities and circulatory disturbances. The goal of the current study was to identify risk factors for the occurrence of FNH in children who had received prior treatment for a malignant tumor.

METHODS

The current retrospective study examined 14 cases of FNH in pediatric patients who previously had been treated for a malignancy. Diagnosis was based on clinical and radiologic findings and was proven histologically in four cases.

RESULTS

FNH lesions were discovered by chance during routine examination in 78% of patients. The incidence of FNH was particularly high in the current series (0.45%) compared with the incidence in the general pediatric population. High doses of alkylating agents (e.g., busulfan or melphalan), venoocclusive disease, and liver radiotherapy may be responsible for injury to the vascular endothelium and subsequent localized circulatory disturbances. FNH is characterized by the absence of complications after its detection; therefore, only close follow-up is recommended.

CONCLUSIONS

FNH appears to be a late complication of an iatrogenic vascular disease in children with a history of malignancy.

摘要

背景

肝脏结节的检测,尤其是在既往有恶性肿瘤治疗史的患者中,引发了诊断难题。肝脏局灶性结节性增生(FNH)在儿童中是一种罕见的良性肿瘤,必须与恶性肝脏病变相鉴别。FNH的病因尚不明确,其发病机制也了解甚少。FNH可能是对局部血管异常和循环障碍的一种反应。本研究的目的是确定曾接受过恶性肿瘤治疗的儿童发生FNH的危险因素。

方法

本回顾性研究检查了14例既往有恶性肿瘤治疗史的儿科患者的FNH病例。诊断基于临床和影像学表现,4例经组织学证实。

结果

78%的患者在常规检查中偶然发现FNH病变。与普通儿科人群的发病率相比,本系列中FNH的发病率特别高(0.45%)。高剂量烷化剂(如白消安或美法仑)、肝静脉闭塞病和肝脏放疗可能导致血管内皮损伤及随后的局部循环障碍。FNH的特点是发现后无并发症;因此,建议仅密切随访。

结论

FNH似乎是有恶性肿瘤病史儿童医源性血管疾病的晚期并发症。

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