Hereditary leiomyomatosis and renal cell carcinoma (HLRCC) syndrome: Spectrum of imaging findings.
Hereditary leiomyomatosis and renal cell carcinoma (HLRCC) syndrome: Spectrum of imaging findings.
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DOI：
10.1016/j.clinimag.2020.06.010
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发表时间：
2020-12
期刊：
影响因子：
2.1
通讯作者：
Malayeri AA
中科院分区：
文献类型：
--
作者：
Paschall AK;Nikpanah M;Farhadi F;Jones EC;Wakim PG;Dwyer AJ;Gautam R;Merino MJ;Srinivasan R;Linehan WM;Malayeri AA
回顾性研究HLRCC相关肾肿瘤的影像学表现，以促进准确的病变特征，并将这些表现与文献报道的单纯囊肿和其他亚型肾细胞癌（RCC）的特征进行比较。对30例（男20例，女10例）经病理证实的HLRCC综合征患者的39个病灶的MRI和CT影像学特征进行了评价。患者诊断时的平均年龄为43.8 ± 13.1岁。记录病变特征，包括单侧性、均匀性、直径（cm）、结节性、分隔、T1和T2信号强度、强化和受限扩散。病变的影像学特征与同期手术切除的良性单纯性囊肿的特征进行进一步比较。受检病灶的平均直径为5.06 ± 3.80 cm，平均生长速率为2.91 × 10−3 cm/天，估计年生长速率为1.06 cm/年。50%的病变表现为结节性，65%大多为T2高信号，83%的病变实性部分表现为扩散受限，65%的病变边缘清晰。76%的患者表现为肾外表现，53%的淋巴结肿大，43%的远处转移。我们的分析证实，虽然HLRCC相关的肾脏病变表现出多样性的影像学表现，大多数是单侧和孤立的，T2高信号，不均匀，边界清晰，并经常表现出限制扩散和结节。
To retrospectively investigate the radiological presentations of HLRCC-associated renal tumors to facilitate accurate lesion characterization and compare these presentations with simple cysts and characteristics of other subtypes of renal cell carcinoma (RCC) as reported in the literature. The MRI and CT imaging characteristics of 39 pathologically confirmed lesions from 30 patients (20 male, 10 female) with HLRCC syndrome were evaluated by two radiologists. Patients had an average age at diagnosis of 43.8 ± 13.1 years. Lesion characteristics including laterality, homogeneity, diameter (cm), nodularity, septations, T1 and T2 signal intensity, enhancement, and restricted diffusion were recorded. Imaging characteristics of the lesions were further compared to characteristics of benign simple cysts surgically removed at the same time point. The examined lesions had a mean diameter of 5.06 ± 3.80 cm, an average growth rate of 2.91 × 10−3 cm/day and an estimated annual growth rate of 1.06 cm/year. 50% of lesions demonstrated nodularity, 65% were mostly T2-hyperintense, 83% demonstrated restricted diffusion in solid portions of the lesions, and 65% had well-defined margins. 76% of patients demonstrated extra-renal manifestations, 53% lymphadenopathy, and 43% distant metastasis. Our analysis confirmed that while HLRCC-associated renal lesions demonstrate diversity in imaging presentations, the majority are unilateral and solitary, T2-hyperintense, heterogeneous with well-defined margins, and frequently demonstrate restricted diffusion and nodularity.
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影响因子：
6.6
作者：
Harmon, WJ;King, BF;Lieber, MM
通讯作者：
Lieber, MM
DOI：
10.1007/s00432-011-1083-0
发表时间：
2012-02-01
影响因子：
3.6
作者：
Li, Xue-Song;Yao, Lin;He, Zhi-Song
通讯作者：
He, Zhi-Song
DOI：
10.1158/1078-0432.ccr-13-0304
发表时间：
2013-07-01
期刊：
Clinical cancer research : an official journal of the American Association for Cancer Research
影响因子：
--
作者：
Linehan WM;Rouault TA
通讯作者：
Rouault TA
DOI：
10.2147/ijnrd.s42097
发表时间：
2014
影响因子：
2
作者：
Schmidt LS;Linehan WM
通讯作者：
Linehan WM
影响因子：
5.6
作者：
Merino, Maria J.;Torres-Cabala, Carlos;Linehan, William Marston
通讯作者：
Linehan, William Marston