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延胡索酸水合酶缺陷型肾细胞癌影像学表现
Radiologic features of fumarate hydratase-deficient renal cell carcinoma
- 陆军军医大学学报 2023年45卷第19期 页码：2081-2084
- 作者机构：
1.400038 重庆，陆军军医大学(第三军医大学)第一附属医院：放射科
2.400038 重庆，陆军军医大学(第三军医大学)第一附属医院：病理科
- 作者简介：
[ 李晓明, E-mail: 359261069@qq.com LI Xiaoming, E-mail: 359261069@qq.com
- 基金信息：
DOI：10.16016/j.2097-0927.202305024
中图分类号： R730.2;R730.44;R737.11收稿：2023-05-07，
修回：2023-05-30，
纸质出版：2023-10-15
- 稿件说明：
移动端阅览
谢巧, 左国娇, 蔡萍, 等. 延胡索酸水合酶缺陷型肾细胞癌影像学表现[J]. 陆军军医大学学报, 2023,45(19):2081-2084.
Qiao XIE, Guojiao ZUO, Ping CAI, et al. Radiologic features of fumarate hydratase-deficient renal cell carcinoma[J]. Journal of Army Medical University, 2023, 45(19): 2081-2084.
谢巧, 左国娇, 蔡萍, 等. 延胡索酸水合酶缺陷型肾细胞癌影像学表现[J]. 陆军军医大学学报, 2023,45(19):2081-2084. DOI： 10.16016/j.2097-0927.202305024.
Qiao XIE, Guojiao ZUO, Ping CAI, et al. Radiologic features of fumarate hydratase-deficient renal cell carcinoma[J]. Journal of Army Medical University, 2023, 45(19): 2081-2084. DOI： 10.16016/j.2097-0927.202305024.
摘要
目的
2
探讨延胡索酸水合酶缺陷型肾细胞癌(fumarate hydratase-deficient renal cell carcinoma，FH-RCC)的临床和影像学特征，旨在提高临床对本病的认识。
方法
2
回顾性分析本院2020-2022年经手术病理确诊为FH-RCC患者的临床信息、病理数据和影像学特征，并进行描述性报告。
结果
2
纳入经病理证实的FH-RCC患者6例(8个肿瘤)，其中男性4例，女性2例，年龄27~48岁。影像学检查结果显示：62.5%(5/8)病灶为囊实性，37.5%(3/8)病灶为纯实性；1例病灶MRI呈混杂信号，DWI上病灶实性成分弥散受限；增强扫描71.4%(5/7)病灶实性部分呈渐进性强化；75.0%(6/8)侵犯肾窦，其中1例同时侵犯肾上腺，66.7%(4/6)首次检查发现腹膜后淋巴结转移。6例患者均行手术切除治疗，随访2~21(6.8±7.8)个月，66.7%(4/6)出现转移。
结论
2
FH-RCC影像学多表现为囊实性占位，囊壁菲薄，增强后实性部分呈渐进性强化。
Abstract
Objective
2
To investigate the clinical and imaging features of fumarate hydratase-deficient renal cell carcinoma (FH-RCC) in order to improve clinical understandings of this disease.
Methods
2
A retrospective study was conducted on all pathologically confirmed FH-RCC patients in our hospital from 2020 to 2022. Their clinical information
pathological data and imaging features were collected and analyzed with descriptive study.
Results
2
There were 6 pathologically confirmed FH-RCC patients (8 tumors) enrolled
and they were 4 males and 2 females
at an age of 27~48 year. Imaging Results showed that 62.5% (5/8) tumors were cystic solid lesions and 37.5% (3/8) were pure solid lesions. One lesion showed mixed signal on MRI and limited diffusion of the solid component of the lesion on diffusion-weighted imaging. Enhanced scanning displayed 71.4% (5/7) had progressive enhancement of the solid portion of the lesion
and 75.0% (6/8) had invasion of the renal sinus
including 1 case with simultaneous invasion of the adrenal gland. Retroperitoneal lymph node metastasis was 66.7% (4/6) cases on first examination. All 6 patients were treated with surgical resection
and 66.7% (4/6) had metastases at 2 to 21 (6.8±7.8) months of follow-up.
Conclusion
2
Radiological features of FH-RCC were mainly cystic solid occupancy with a thin cystic wall and progressive enhancement of the solid part after contrast enhancements.
关键词
Keywords
references
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