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肾上腺囊性病变:聚焦儿科人群(综述)

Cystic adrenal lesions: focus on pediatric population (a review).

作者信息

Carsote Mara, Ghemigian Adina, Terzea Dana, Gheorghisan-Galateanu Ancuta Augustina, Valea Ana

机构信息

Endocrinology Department, Carol Davila University of Medicine and Pharmacy & C.I. Parhon National Institute of Endocrinology, Bucharest, Romania.

Endocrinology Department, Monza Oncoteam Hospital & C.I. Parhon National Institute of Endocrinology, Bucharest, Romania.

出版信息

Clujul Med. 2017;90(1):5-12. doi: 10.15386/cjmed-677. Epub 2017 Jan 15.

Abstract

BACKGROUND AND AIM

The cysts may potentially affect any organ; adrenals cysts are rare. This is a review of the literature regarding adrenal cysts, focusing on children and young adults.

GENERAL DATA

Three major types have been described: pure cysts (endothelial, epithelial, and hemorrhagic or pseudocyst), parasitic (as hydatid) cysts and cystic part of a tumour (most frequent are neuroblastoma, ganglioneuroma, pheocromocytoma, and teratoma). The complications are: bleeding, local pressure effects; infection; rupture (including post-traumatic); arterial hypertension due to renal vessels compression. Adrenal hemorrhage represents a particular condition associating precipitating factors such as: coagulation defects as Factor IX or X deficiency, von Willebrand disease, thrombocytopenia; antiphospholipid syndrome; previous therapy with clopidogrel or corticosteroids; the rupture of a prior tumour. At birth, the most suggestive features are abdominal palpable mass, anemia, and persistent jaundice. Adrenal insufficiency may be found especially in premature delivery. The hemorrhage is mostly self-limiting. Antenatal ultrasound diagnosis of a cyst does not always predict the exact pathology result. The most important differential diagnosis of adrenal hemorrhage/hemorrhagic cyst is cystic neuroblastoma which is highly suggestive in the presence of distant metastases and abnormal catecholamine profile. The major clue to differentiate the two conditions is the fact that the tumor is stable or increases over time while the adrenal hemorrhage is expected to remit within one to two weeks.

CONCLUSION

Pediatric adrenal cysts vary from simple cysts with a benign behavior to neoplasia- related lesions displaying severe prognosis as seen in cystic neuroblastoma. A multidisciplinary team is required for their management which is conservative as close follow-up or it makes necessary different surgical procedures in cases with large masses or if a malignancy suspicion is presented. Recently, laparoscopic approach is regarded as a safe procedure by some authors but generally, open surgery is more frequent used compare to adults; in most cases the preservation of normal gland is advisable.

摘要

背景与目的

囊肿可能累及任何器官;肾上腺囊肿较为罕见。本文是一篇关于肾上腺囊肿的文献综述,重点关注儿童和青少年。

一般资料

肾上腺囊肿主要有三种类型:单纯囊肿(内皮性、上皮性、出血性或假性囊肿)、寄生性(如包虫)囊肿以及肿瘤的囊性部分(最常见的是神经母细胞瘤、神经节神经瘤、嗜铬细胞瘤和畸胎瘤)。其并发症包括:出血、局部压迫效应、感染、破裂(包括创伤后)、肾血管受压导致的动脉高血压。肾上腺出血是一种特殊情况,与多种促发因素相关,如:凝血因子IX或X缺乏、血管性血友病、血小板减少等凝血缺陷;抗磷脂综合征;先前使用氯吡格雷或皮质类固醇治疗;先前肿瘤破裂。出生时,最具提示性的特征是腹部可触及肿块、贫血和持续性黄疸。肾上腺功能不全尤其在早产时可能出现。出血大多为自限性。产前超声诊断囊肿并不总能预测确切的病理结果。肾上腺出血/出血性囊肿最重要的鉴别诊断是囊性神经母细胞瘤,远处转移和异常儿茶酚胺谱的存在强烈提示该病。区分这两种情况的主要线索是肿瘤随时间稳定或增大,而肾上腺出血预计在一到两周内消退。

结论

小儿肾上腺囊肿从行为良性的单纯囊肿到如囊性神经母细胞瘤等预后严重的肿瘤相关病变不等。其治疗需要多学科团队参与,治疗方式较为保守,密切随访即可,但若出现大肿块或怀疑恶性,则需采取不同的手术方法。近来,一些作者认为腹腔镜手术是一种安全的术式,但总体而言,与成人相比,开放手术更为常用;大多数情况下,保留正常腺体是可取的。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8762/5305088/e185f4e642c2/cm-90-5f1.jpg

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