The ABCs of BHD: An In-Depth Review of Birt-Hogg-Dube Syndrome

被引：26
作者：
Gupta, Shiva [1 ]
Kang, Hyunseon C. [1 ]
Ganeshan, Dhakshinamoorthy [1 ]
Morani, Ajaykumar [1 ]
Gautam, Rabindra [2 ]
Choyke, Peter L. [3 ]
Kundra, Vikas [1 ,4 ]
机构：
[1] Univ Texas MD Anderson Canc, Dept Diagnost Radiol, 1515 Holcombe Blvd,Unit 1473, Houston, TX 77030 USA
[2] NCI, Harris Tech Serv Corp, NIH, Bethesda, MD 20892 USA
[3] NCI, Ctr Canc Res, NIH, Bethesda, MD 20892 USA
[4] Univ Texas MD Anderson Canc Ctr, Dept Canc Syst Imaging, Houston, TX 77030 USA
来源：
AMERICAN JOURNAL OF ROENTGENOLOGY | 2017年 / 209卷 / 06期
关键词：
Birt-Hogg-Dube syndrome; kidney; renal cell carcinoma; RENAL-CELL CARCINOMA; SPONTANEOUS PNEUMOTHORAX; PATHOLOGICAL CORRELATION; HISTOLOGIC SUBTYPES; DIAGNOSTIC-APPROACH; IMAGING SPECTRUM; GROWTH-RATES; KIDNEY; ONCOCYTOMA; DISEASE;
D O I：
10.2214/AJR.17.18071
中图分类号：
R8 [特种医学]; R445 [影像诊断学];
学科分类号：
1002 ; 100207 ; 1009 ;
摘要：
OBJECTIVE. Birt-Hogg-Dube (BHD) syndrome is an autosomal dominant inherited syndrome involving multiple organs. In young patients, renal neoplasms that are multiple, bilateral, or both, such as oncocytomas, chromophobe renal cell carcinoma (RCC), hybrid chromophobe RCC-oncocytomas, clear cell RCC, and papillary RCC, can suggest BHD syndrome. Extrarenal findings, including dermal lesions, pulmonary cysts, and spontaneous pneumothoraces, also aid in diagnosis. CONCLUSION. Radiologists may be one of the first medical specialists to suggest the diagnosis of BHD syndrome. Knowledge of pathogenesis and management, including the importance of the types of renal neoplasms in a given patient, is needed to properly recognize this rare condition.
引用
收藏
页码：1291 / 1296
页数：6
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