摘要
目的：分析结节性硬化多器官多系统损害的影像学表现，提高对结节性硬化全面认识。方法：回顾性分析10例临床确诊的结节性硬化伴多器官损害的临床及影像学资料，了解颅外器官损害及病理类型。结果：10例均有特征性颅内结节，1例伴室管膜下巨细胞星形细胞瘤；8例累及肾脏，均为双侧肾脏多发血管平滑肌脂肪瘤，其中1例伴双肾多发小囊肿，1例伴瘤内动脉瘤，1例伴肝脏多发血管平滑肌脂肪瘤，2例伴肺淋巴管平滑肌瘤病，2例伴心室腔内横纹肌瘤，其中1例伴先天性肛门畸形；1例同时累及肾脏、骨骼及大血管；1例伴胰腺神经内分泌肿瘤；1例膝关节皮下纤维瘤伴同侧关节腔积液。结论：结节性硬化除颅脑特征性结节外，身体其他系统器官也可出现受损害表现，了解其影像学表现，有助于提高结节性硬化的诊断。
Abstract
Objective: To analyze the imaging characteristics of tuberous sclerosis complex(TSC) with multiple organ impairment in multiple systems which might help improving the diagnostic efficacy． Methods: Clinical presentation and imaging data of 10 TSC with multiple organ impairment confirmed in clinic were retrospective analyzed, so as to study the extracranial manifestations and pathological types. Results: All the 10 cases were accompanied with typical subependymal nodules, one with subependymal giant cell astrocytoma. Bilateral multiple renal angiomyolipomas(AML) were proven in seven cases with one case of bilateral multiple cysts found concomitantly, intratumoral aneurysm in one case, multiple hepatic AML in one case, pulmonary lymphangioleiomyomatosis in two cases, cardiac rhabdomyosarcomas in two cases with one had congenital anal malformation, pancreatic endocrine neoplasm in one case and knee subcutaneous fibroma with joint effusion in one case. Conclusion: TSC involves multiple organs besides subependymal nodules. Hence, better understanding of these imaging manifestations permits a more specific and accurate diagnosis.
关键词
结节性硬化症 /
体层摄影术 /
X线计算机 /
磁共振成像
Key words
Tuberous sclerosis /
Tomography /
X-ray computed /
Magnetic resonance imaging
茅依玲;李玉华;李美蓉;崔艳芬.
结节性硬化症伴多系统损害的影像学表现[J]. 中国临床医学影像杂志. 2014, 25(11): 800-803
MAO Yi-ling;LI Yu-hua;LI Mei-rong;CUI Yan-fen.
Imaging characteristics of tuberous sclerosis complex with multiple system impairment[J]. Journal of China Clinic Medical Imaging. 2014, 25(11): 800-803
中图分类号：
Ｒ758.3
R814.42
Ｒ445.2
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