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儿童肾横纹肌样瘤:CT表现

Rhabdoid tumors of the kidney in children: CT findings.

作者信息

Chung C J, Lorenzo R, Rayder S, Schemankewitz E, Guy C D, Cutting J, Munden M

机构信息

Department of Radiology, Scottish Rite Children's Medical Center, Atlanta, GA 30342.

出版信息

AJR Am J Roentgenol. 1995 Mar;164(3):697-700. doi: 10.2214/ajr.164.3.7863897.

Abstract

OBJECTIVE

The purpose of this study was to identify CT characteristics of rhabdoid tumor of the kidney, a rare, aggressive, malignant neoplasm of unknown origin that occurs mainly in infants and young children.

MATERIALS AND METHODS

CT scans of eight children (newborn to 13 years old; mode, 4 months old; six boys and two girls) with histopathologic diagnosis of rhabdoid tumor of the kidney were evaluated retrospectively by two of the authors, who were not blinded to the diagnosis. All eight CT scans were performed with intravenous and oral contrast, and noncontrast intravenous CT scans were available in four cases. The following CT characteristics were evaluated: location of tumor within the kidney; presence of calcification, subcapsular hematoma, multiple tumor lobules, enlarged vessels, vascular invasion, or central tumor necrosis or hemorrhage; visibility of tumor margin; distant metastasis; and primary tumor size.

RESULTS

All eight primary tumors (five on the left, one of which had contralateral renal nodules) were central in location and involved the hilum. Calcification outlining the tumor lobule was present in two of the four tumors on noncontrast CT scans. Subcapsular hematoma was seen in five children. Tumor necrosis and hemorrhage were seen in seven children. Tumors were well defined from the renal cortex in four children. Lobules of tumor were seen in seven children.

CONCLUSION

CT findings of calcification, subcapsular hematoma, and lobular appearance in a large, centrally located, and heterogeneous renal mass in a child suggest a rhabdoid tumor of the kidney.

摘要

目的

本研究旨在确定肾横纹肌样瘤的CT特征,这是一种罕见的、侵袭性的、起源不明的恶性肿瘤,主要发生于婴幼儿。

材料与方法

对8例经组织病理学诊断为肾横纹肌样瘤的儿童(年龄从新生儿至13岁;平均年龄4个月;6名男孩和2名女孩)的CT扫描进行回顾性评估,评估者为两位作者,他们知晓诊断结果。所有8例CT扫描均进行了静脉内和口服对比剂增强扫描,4例还进行了非增强静脉CT扫描。评估了以下CT特征:肿瘤在肾内的位置;有无钙化、包膜下血肿、多个肿瘤小叶、血管增粗、血管侵犯、肿瘤中心坏死或出血;肿瘤边缘的清晰度;远处转移;以及原发肿瘤大小。

结果

所有8例原发肿瘤(5例位于左侧,其中1例伴有对侧肾结节)均位于肾脏中央并累及肾门。在4例非增强CT扫描的肿瘤中,有2例可见勾勒肿瘤小叶的钙化。5例儿童可见包膜下血肿。7例儿童可见肿瘤坏死和出血。4例儿童的肿瘤与肾皮质界限清晰。7例儿童可见肿瘤小叶。

结论

儿童肾脏内一个大的、位于中央且不均匀的肿块,若CT表现为钙化、包膜下血肿及小叶状外观,则提示为肾横纹肌样瘤。

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