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Renal epithelioid angiomyolipoma: magnetic resonance imaging characteristics

Abdominal Radiology volume 43, pages 2756–2763 (2018)Cite this article

Abstract

Objective

The aim of the study was to analyze MR imaging features of renal epithelioid angiomyolipoma (EAML).

Methods

This study included 17 patients with histopathologically confirmed renal EAML who underwent renal MRI scanning before radical or partial nephrectomy. MR images were retrospectively reviewed and correlated with pathological findings.

Result

Fifteen lesions (88.2%) appeared as round or oval. The tumor-kidney interface was round in 14 lesions (82.4%). Fifteen tumors (88.2%) presented mainly isointensity on T1WI, and eleven tumors (64.7%) presented mainly hypointensity on T2WI. Twelve lesions (70.6%) showed restricted diffusion on DWI, and the mean ADC value was 1.23 ± 0.28 × 10−3mm2/s. Minimal fat component was identified as loss of signal intensity on opposed-phase MR images in 6 cases (35.3%). Sixteen lesions (100%) demonstrated inhomogeneous enhancement, and 7 of 16 masses (43.8%) showed reticular enhancement. Rapid wash-in and wash-out enhancement was seen in 13 masses (81.3%). In the corticomedullary phase, the mass showed markedly enhancement in 14 cases (87.5%). The irregular vessels and hemorrhage were detected in 4 cases (23.5%) and 7 cases (41.2%), respectively. One patient (5.9%) had a lymph node involvement at initial diagnosis, and showed distant metastasis after operation. In the immunohistochemical analysis, 15 tumors (88.2%) were positive for melanocytic marker (HMB45 or Melan-A), and all cases (100%) were negative for epithelial-associated markers (CK or AE1/AE3).

Conclusion

The presence of hypointensity on T2WI, restricted diffusion on DWI, round tumor-kidney interface, reticular, and marked enhancement (rapid wash-in and wash-out) should further raise suspicion for renal EAML. The diagnosis may be confirmed by pathological analysis.

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References

  1. Martignoni G, Reuter VE, Fletcher CDM, World Health Organization (2016) Angiomyolipoma of World Health Organization Classification of Tumours of the Urinary System and Male Genital Organs. Lyon: IARC Press, pp 62–65

    Google Scholar 

  2. Martignoni G, Bonetti F (1995) Renal epithelioid oxyphilic neoplasm (REON), a pleomorphic variant of renal angiomyolipoma. Int J Surg Pathol 2:539

    Google Scholar 

  3. Mai KT, Collins JP (1996) Epithelioid cell variant of renal angiomyolipoma. Histopathology 28:277–280

    Article  PubMed  CAS  Google Scholar 

  4. Martignoni G, Reuter VE, Fletcher CDM, World Health Organization (2016) Epithelioid angiomyolipoma of World Health Organization Classification of Tumours of the Urinary System and Male Genital Organs. World Health Organization. Lyon: IARC Press, pp 65–66

    Google Scholar 

  5. Aydin H, Magi-Galluzzi C, Lane BR, et al. (2009) Renal angiomyolipoma clinicopathologic study of 194 cases with emphasis on the epithelioid histology and tuberous sclerosis association. Am J Surg Pathol 33(2):289–297

    Article  PubMed  Google Scholar 

  6. Jinzaki M, Silverman SG, Akita H, et al. (2014) Renal angiomyolipoma: a radiological classification and update on recent developments in diagnosis and management. Abdom Imaging 39(3):588–604

    Article  PubMed  PubMed Central  Google Scholar 

  7. Israel GM, Hindman N, Hecht E, Krinsky G (2005) The use of opposed-phase chemical shift MRI in the diagnosis of renal angiomyolipomas. AJR Am J Roentgenol 184(6):1868–1872

    Article  PubMed  Google Scholar 

  8. Cui L, Zhang JG, Hu XY, et al. (2012) CT imaging and histopathological features of renal epithelioid angiomyolipomas. Clin Radiol 67(12):e77–e82

    Article  PubMed  CAS  Google Scholar 

  9. Tsukada J, Jinzaki M, Yao M, et al. (2013) Epithelioid angiomyolipoma of the kidney: radiological imaging. Int J Urol 20(11):1105–1111

    Article  PubMed  Google Scholar 

  10. Froemming AT, Boland J, Cheville J, Takahashi N, Kawashima A (2013) Renal epithelioid angiomyolipoma: imaging characteristics in nine cases with radiologic-pathologic correlation and review of the literature. AJR Am. J. Roentgenol 200(2):W178–W186

    Article  PubMed  Google Scholar 

  11. Zhong Y, Shen Y, Pan J, et al. (2017) Renal epithelioid angiomyolipoma: MRI findings. Radiol Med 122(11):814–821

    Article  PubMed  Google Scholar 

  12. Thoeny HC, De Keyzer F (2007) Extracranial applications of diffusion-weighted magnetic resonance imaging. Eur Radiol 17(6):1385–1393

    Article  PubMed  Google Scholar 

  13. Sufana Iancu A, Colin P, Puech P, et al. (2013) Significance of ADC value for detection and characterization of urothelial carcinoma of upper urinary tract using diffusion-weighted MRI. World J Urol 31(1):13–19

    Article  PubMed  CAS  Google Scholar 

  14. Brimo F, Robinson B, Guo C, et al. (2010) Renal epithelioid angiomyolipoma with atypia: a series of 40 cases with emphasis on clinicopathologic prognostic indicators of malignancy. Am J Surg Pathol 34(5):715–722

    PubMed  Google Scholar 

  15. Faraji H, Nguyen BN, Mai KT (2009) Renal epithelioid angiomyolipoma: a study of six cases and a meta-analytic study. Development of criteria for screening the entity with prognostic significance. Histopathology 55(5):525–534

    Article  PubMed  Google Scholar 

  16. Verma SK, Mitchell DG, Yang R, et al. (2010) Exophytic renal masses: angular interface with renal parenchyma for distinguishing benign from malignant lesions at MR imaging. Radiology 255(2):501–507

    Article  PubMed  Google Scholar 

  17. Jinzaki M, Silverman SG, Akita H, Mikami S, Oya M (2017) Diagnosis of renal angiomyolipomas: classic, fat-poor, and epithelioid types. Semin Ultrasound CT MR 38(1):37–46

    Article  PubMed  Google Scholar 

  18. Chen Y, Zhu Z, Dai JR, et al. (2010) Imaging findings of benign renal angiomyolipoma with minimal fat content. Chin Clin Med Imaging 21(10):705–708

    Google Scholar 

  19. Vikram R, Ng CS, Tamboli P, et al. (2009) Papillary renal cell carcinoma radiologic-pathologic correlation and spectrum of disease. Radiographics 29(3):741–754

    Article  PubMed  Google Scholar 

  20. Pedrosa I, Sun MR, Spencer M, et al. (2008) MR imaging of renal masses: correlation with findings at surgery and pathologic analysis. Radiographics 28(4):985–1003

    Article  PubMed  Google Scholar 

  21. Chung MS, Choi HJ, Kim MH, Cho KS (2014) Comparison of t2-weighted MRI with and without fat suppression for differentiating renal angiomyolipomas without visible fat from other renal tumors. AJR Am J Roentgenol 202:765–771

    Article  PubMed  Google Scholar 

  22. Low G, Sahi K, Dhliwayo H (2012) Low T2 signal intensity on magnetic resonance imaging: a feature of minimal fat angiomyolipomas. Int J Urol 19(1):90–91

    Article  PubMed  Google Scholar 

  23. Kim Y, Sung DJ, Sim KC, et al. (2017) Renal tumors with low signal intensities on T2-weighted MR image: radiologic-pathologic correlation. Abdom Radiol 42(8):2108–2118

    Article  Google Scholar 

  24. Yamakado K, Tanaka N, Nakagawa T, et al. (2002) Renal angiomyolipoma: relationships between tumor size, aneurysm formation, and rupture. Radiology 225(1):78–82

    Article  PubMed  Google Scholar 

  25. Schieda N, Kielar AZ, Al McInnes MD, Flood TA (2015) Ten uncommon and unusual variants of renal angiomyolipoma (AML): radiologic-pathologic correlation. Clin Radiol 70(2):206–220

    Article  PubMed  CAS  Google Scholar 

  26. Beddy P, Genega EM, Ngo L, et al. (2014) Tumor necrosis on magnetic resonance imaging correlates with aggressive histology and disease progressionin clear cell renal cell carcinoma. Clin Genitourin Cancer 12:55–62

    Article  PubMed  Google Scholar 

  27. Corr P, Yang WT, Tan I (1994) Spontaneous haemorrhage from renal angiomyolipomata. Australas Radiol 38:132–134

    Article  PubMed  CAS  Google Scholar 

  28. Wang H, Cheng L, Zhang X, et al. (2010) Renal cell carcinoma: diffusion-weighted MR imaging for subtype differentiation at 3.0 T. Radiology 257(1):135–143

    Article  PubMed  Google Scholar 

  29. Sasamori H, Saiki M, Suyama J, et al. (2014) Utility of apparent diffusion coefficients in the evaluation of solid renal tumors at 3T. Magn Reson Med Sci 13(2):89–95

    Article  PubMed  Google Scholar 

  30. Cornelis F, Tricaud E, Lasserre AS, et al. (2014) Routinely performed multiparametric magnetic resonance imaging helps to differentiate common subtypes of renal tumours. Eur Radiol. 24(5):1068–1080

    Article  PubMed  CAS  Google Scholar 

  31. Taouli B, Thakur RK, Mannelli L, et al. (2009) Renal lesions: characterization with diffusion-weighted imaging versus contrast-enhanced MR imaging. Radiology. 251(2):398–407

    Article  PubMed  Google Scholar 

  32. Squillaci E, Manenti G, Di Stefano F, et al. (2004) Diffusion-weighted MR imaging in the evaluation of renal tumours. J Exp Clin Cancer Res 23(39–45):24

    Google Scholar 

  33. Nese N, Martignoni G, Fletcher CD, et al. (2011) Pure epithelioid PE comas (SO-called epithelioid angiomyolipoma) of the KIDNEY: a clinicopathologic study of 41 cases: detailed assessment of morphology and risk stratification. Am J Surg Pathol 35(2):161–176

    Article  PubMed  Google Scholar 

  34. Lei JH, Liu LR, Wei Q, et al. (2015) A four-year follow-up study of renal epithelioid angiomyolipoma: a multi-center experience and literature review. Sci Rep 5:10030

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  35. Serrano Frago P, Camisón CDAA, Sanz MJG, et al. (2006) Controversies related to epithelioid variant of renal angiomyolipoma: a review of the literature. Urology 67(846):e843–e845

    Google Scholar 

  36. Shitara K, Yatabe Y, Mizota A, et al. (2011) Dramatic tumor response to everolimus for malignant epithelioid angiomyolipoma. Jpn J Clin Oncol 41(6):814–816

    Article  PubMed  Google Scholar 

  37. Wolff N, Kabbani W, Bradley T, et al. (2010) Sirolimus and temsirolimus for epithelioid angiomyolipoma. J Clin Oncol 28:e65–e68

    Article  PubMed  Google Scholar 

  38. Shitara K, Yatabe Y, Mizota A, et al. (2011) Dramatic tumor response to everolimus for malignant epithelioid angiomyolipoma. Jpn J Clin Oncol 41:814–816

    Article  PubMed  Google Scholar 

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Author notes

  1. Xinying Cong

    Present address: China Rehabilitation Research Center, Beijing, 100068, China

Authors and Affiliations

  1. National Cancer Center/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, 100021, China

    Xinying Cong, Jin Zhang, Xiaojuan Xu, Miaomiao Zhang & Yan Chen

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  1. Xinying Cong
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  2. Jin Zhang
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Correspondence to Yan Chen.

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Funding

No funding was received for this study.

Conflict of interest

The authors declare that they have no conflict of interest.

Ethical approval

Informed consent was not required as it was a retrospective data analysis of MRI exams that had been acquired as part of clinical routine. Informed consent for Magnetic Resonance Imaging exams had routinely been obtained from each individual included in this study. This study is in accordance with the ethical standards of the institutional and national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

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Cong, X., Zhang, J., Xu, X. et al. Renal epithelioid angiomyolipoma: magnetic resonance imaging characteristics. Abdom Radiol 43, 2756–2763 (2018). https://doi.org/10.1007/s00261-018-1548-6

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