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KMID : 0880420210220121996
Korean Journal of Radiology
2021 Volume.22 No. 12 p.1996 ~ p.2005
Multidetector CT Characteristics of Fumarate Hydratase-Deficient Renal Cell Carcinoma and Papillary Type II Renal Cell Carcinoma
Yang Ling

Li Xue-Ming
Hu Ya-Jun
Zhang Meng-Ni
Yao Jin
Song Bin
Abstract
Objective: To investigate the multidetector computed tomography (MDCT) features of fumarate hydratase-deficient renal cell carcinoma (FH-deficient RCC) with germline or somatic mutations, and compare them with those of papillary type II RCC (pRCC type II).

Materials and Methods: A total of 24 patients (mean ¡¾ standard deviation, 40.4 ¡¾ 14.7 years) with pathologically confirmed FH-deficient RCC (15 with germline and 9 with somatic mutations) and 54 patients (58.6 ¡¾ 12.6 years) with pRCC type II were enrolled. The MDCT features were retrospectively reviewed and compared between the two entities and mutation subgroups, and were correlated with the clinicopathological findings.

Results: All the lesions were unilateral and single. Compared with pRCC type II, FH-deficient RCC was more prevalent among younger patients (40.4 ¡¾ 14.7 vs. 58.6 ¡¾ 12.6, p < 0.001) and tended to be larger (8.1 ¡¾ 4.1 vs. 5.4 ¡¾ 3.2, p = 0.002). Cystic solid patterns were more common in FH-deficient RCC (20/24 vs. 16/54, p < 0.001), with 16 of the 20 (80.0%) cystic solid tumors having showed typical polycystic and thin smooth walls and/or septa, with an eccentric solid component. Lymph node (16/24 vs. 16/54, p = 0.003) and distant (11/24 vs. 3/54, p < 0.001) metastases were more frequent in FH-deficient RCC. FH-deficient RCC and pRCC type II showed similar attenuation in the unenhanced phase. The attenuation in the corticomedullary phase (CMP) (76.3% ¡¾ 25.0% vs. 60.2 ¡¾ 23.6, p = 0.008) and nephrographic phase (NP) (87.7 ¡¾ 20.5, vs. 71.2 ¡¾ 23.9, p = 0.004), absolute enhancement in CMP (39.0 ¡¾ 24.8 vs. 27.1 ¡¾ 22.7, p = 0.001) and NP (50.5 ¡¾ 20.5 vs. 38.2 ¡¾ 21.9, p = 0.001), and relative enhancement ratio to the renal cortex in CMP (0.35 ¡¾ 0.26 vs. 0.24 ¡¾ 0.19, p = 0.001) and NP (0.43 ¡¾ 0.24 vs. 0.29 ¡¾ 0.19, p < 0.001) were significantly higher in FH-deficient RCC. No significant difference was found between the FH germline and somatic mutation subgroups in any of the parameters.

Conclusion: The MDCT features of FH-deficient RCC were different from those of pRCC type II, whereas there was no statistical difference between the germline and somatic mutation subgroups. A kidney mass with a cystic solid pattern and metastatic tendency, especially in young patients, should be considered for FH-deficient RCC.
KEYWORD
Fumarate hydratase, Carcinoma, Renal cell, Papillary, Hereditary leiomyomatosis and renal cell carcinoma
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