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产前检测到的与贝克威思-维德曼综合征相关的肾上腺囊性肿块。

Prenatally detected cystic adrenal mass associated with Beckwith-Wiedemann syndrome.

作者信息

Merrot Thierry, Walz Jochen, Anastasescu Robert, Chaumoître Kathia, D'Ercole Claude

机构信息

Service de Chirurgie Infantile, AP-HM Nord, Université de la Méditerranée, Marseille, France.

出版信息

Fetal Diagn Ther. 2004 Nov-Dec;19(6):465-9. doi: 10.1159/000080155.

Abstract

We report a case of a right-sided cystic adrenal mass, detected after the 21st week of gestation, associated with fetal macrosomia. The diagnosis of Beckwith-Wiedemann syndrome was evoked. Prenatal sonography and magnetic resonance imaging did not allow establishing the origin of the suprarenal mass. The differential diagnosis of cystic neuroblastoma, pseudocystic adrenal haemorrhage, and adrenocortical macrocysts was discussed. A laparotomy was performed 2 weeks after birth due to the increasing size of the tumour and due to its possible malignant origin. Histological findings were haemorrhagic lesions without evidence of malignancy and adrenal cortical cytomegaly. The diagnosis of an adrenocortical macrocyst component of Beckwith-Wiedemann syndrome was established.

摘要

我们报告一例妊娠21周后发现的右侧肾上腺囊性肿块,伴有胎儿巨大症。引发了贝克威思-维德曼综合征的诊断。产前超声和磁共振成像未能确定肾上腺肿块的起源。讨论了囊性神经母细胞瘤、假性肾上腺出血和肾上腺皮质大囊肿的鉴别诊断。由于肿瘤体积增大及其可能的恶性起源,出生后2周进行了剖腹手术。组织学检查结果为出血性病变,无恶性证据及肾上腺皮质细胞肥大。确诊为贝克威思-维德曼综合征的肾上腺皮质大囊肿成分。

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