Uncommon malignant renal tumors and atypical presentation of common ones: a guide for radiologists

  • Benjamin LagunaEmail author
  • Antonio C. Westphalen
  • C. T. Guimarães
  • Zhen Whang
  • Jeff Simko
  • Ronald Zagoria
Pictorial essay



While the typical imaging features of the more common RCC subtypes have previously been described, they can at times have unusual, but distinguishing features. Rarer renal tumors span a broad range of imaging features, but they may also have characteristic presentations. We review the key imaging features of atypical presentations of malignant renal tumors and uncommon malignant renal tumors.


Renal tumors have many different presentation patterns, but knowledge of the distinguishing MR and CT features can help identify both atypical presentation of common malignancies and uncommon renal tumors.


CT MRI RCC Kidney Neoplasm 


Compliance with ethical standards


No funding was obtained for this paper.

Conflict of interest

Antonio C. Westphalen is a scientific advisory board member for 3D Biopsy, LLC. Ronald Zagoria is a consultant for ReCor Medical, Inc. Benjamin Laguna, CT Guimarães, Zhen Whang, and Jeff Simko declare no conflicts of interest.

Ethical approval

This article does not contain any studies with human participants or animals performed by any of the authors.


  1. 1.
    Pedrosa I, Sun MR, Spencer M, et al. (2008) MR imaging of renal masses: correlation with findings at surgery and pathologic analysis. Radiographics 28:985–1003. CrossRefPubMedGoogle Scholar
  2. 2.
    Delahunt B, Srigley JR, Delahunt B, et al. (2013) CHAPTER 1 tumours of the kidney WHO histological classification of tumours of the kidney. Int Classif 32:1469–1489. CrossRefGoogle Scholar
  3. 3.
    Young JR, Margolis D, Sauk S, et al. (2013) Clear cell renal cell carcinoma: discrimination from other renal cell carcinoma subtypes and oncocytoma at multiphasic multidetector CT. Radiology 267:444–453. CrossRefPubMedGoogle Scholar
  4. 4.
    Kim JK, Kim TK, Kim CS, Kim K, Cho K (2002) Differentiation of subtypes of renal cell carcinoma on helical CT scans. Am J Roentgenol 178:1499–1506CrossRefGoogle Scholar
  5. 5.
    Kang SK, Huang WC, Pandharipande PV, Chandarana H (2014) Solid renal masses: what the numbers tell us. Am J Roentgenol 202:1196–1206. CrossRefGoogle Scholar
  6. 6.
    Sheth S, Scatarige JC, Horton KM, Corl FM, Fishman EK (2001) Current concepts in the diagnosis and management of renal cell carcinoma: role of multidetector CT and three-dimensional CT. Radiographics 21:S237–S254. CrossRefPubMedGoogle Scholar
  7. 7.
    Oliva MR, Glickman JN, Zou KH, et al. (2009) Renal cell carcinoma: T1 and T2 signal intensity characteristics of papillary and clear cell types correlated with pathology. Am J Roentgenol 192:1524–1530. CrossRefGoogle Scholar
  8. 8.
    Young JR, Coy H, Kim HJ, et al. (2017) Performance of relative enhancement on multiphasic MRI for the Differentiation of Clear Cell Renal Cell Carcinoma (RCC) from Papillary and Chromophobe RCC subtypes and oncocytoma. Am J Roentgenol 208:812–819. CrossRefGoogle Scholar
  9. 9.
    Lee-Felker SA, Felker ER, Tan N, et al. (2014) Qualitative and quantitative MDCT features for differentiating clear cell renal cell carcinoma from other solid renal cortical masses. Am J Roentgenol 203:W516–W524. CrossRefGoogle Scholar
  10. 10.
    Kang SK, Zhang A, Pandharipande PV, et al. (2015) DWI for renal mass characterization: systematic review and meta-analysis of diagnostic test performance. Am J Roentgenol 205:317–324. CrossRefGoogle Scholar
  11. 11.
    Hotker AM, Mazaheri Y, Wibmer A, et al. (2016) Use of DWI in the differentiation of renal cortical tumors. Am J Roentgenol 206:100–105. CrossRefGoogle Scholar
  12. 12.
    Clark PE (2009) The role of VHL in clear-cell renal cell carcinoma and its relation to targeted therapy. Kidney Int. 76:939–945CrossRefGoogle Scholar
  13. 13.
    Karlo CA, Luigi P, Paolo D, Chaim J, Hakimi AA (2014) Radiogenomics of clear-cell renal cell carcinoma: associations between CT imaging features and mutations. Radiology 270:464–471. CrossRefPubMedGoogle Scholar
  14. 14.
    Choyke PL, Glenn GM, Walther MM, et al. (1992) The natural history of renal lesions in von Hippel–Lindau disease: a serial CT study in 28 patients. Am J Roentgenol . CrossRefGoogle Scholar
  15. 15.
    Hes FJ, Feldberg MA (1999) Von Hippel–Lindau disease: strategies in early detection (renal-, adrenal-, pancreatic masses). Eur Radiol . CrossRefPubMedGoogle Scholar
  16. 16.
    Ng CS, Wood CG, Silverman PM, et al. (2008) Renal cell carcinoma: diagnosis, staging, and surveillance. Am J Roentgenol 191:1220–1232. CrossRefGoogle Scholar
  17. 17.
    Zagoria RJ, Dyer RB, Assimos DG, Scharling ES, Quinn SF (1991) Spontaneous perinephric hemorrhage: imaging and management. J Urol 145:468–471CrossRefGoogle Scholar
  18. 18.
    Childs DD, Clingan MJ, Zagoria RJ, et al. (2014) In-phase signal intensity loss in solid renal masses on dual-echo gradient-echo MRI: association with malignancy and pathologic classification. Am J Roentgenol 203:W421–W428. CrossRefGoogle Scholar
  19. 19.
    Taouli B, Thakur RK, Mannelli L, et al. (2009) Renal lesions: characterization with diffusion-weighted imaging versus contrast-enhanced MR imaging. Radiology 251:398–407. CrossRefPubMedGoogle Scholar
  20. 20.
    Vikram R, Ng CS, Tamboli P, et al. (2009) Papillary renal cell carcinoma: radiologic-pathologic correlation and spectrum of disease. Radiographics 29:741–747. CrossRefPubMedGoogle Scholar
  21. 21.
    Fondriest SA, Gowdy JM, Goyal M, Sheridan KC, Wasdahl DA (2015) Concurrent renal-cell carcinoma and cutaneous leiomyomas: a case of HLRCC. Radiol Case Rep 10:962. CrossRefPubMedPubMedCentralGoogle Scholar
  22. 22.
    PDQ Cancer Genetics Editorial Board (2002) Genetics of Kidney Cancer (Renal Cell Cancer) (PDQ®): Health Professional VersionGoogle Scholar
  23. 23.
    Prasad SR, Dalrymple NC, Surabhi VR (2008) Cross-sectional imaging evaluation of renal masses. Radiol Clin N Am 46:95–111CrossRefGoogle Scholar
  24. 24.
    Prasad SR, Humphrey PA, Catena JR, et al. (2006) Common and uncommon histologic subtypes of renal cell carcinoma: imaging spectrum with pathologic correlation. RadioGraphics 26:1795–1806. CrossRefPubMedGoogle Scholar
  25. 25.
    Raman SP, Johnson PT, Allaf ME, Netto G, Fishman EK (2013) Chromophobe renal cell carcinoma: multiphase MDCT enhancement patterns and morphologic features. Am J Roentgenol 201:1268–1276. CrossRefGoogle Scholar
  26. 26.
    Rosenkrantz AB, Hindman N, Fitzgerald EF, et al. (2010) MRI features of renal oncocytoma and chromophobe renal cell carcinoma. Am J Roentgenol . CrossRefGoogle Scholar
  27. 27.
    Paner GP, Amin MB, Alvarado-Cabrero I, et al. (2010) A novel tumor grading scheme for chromophobe renal cell carcinoma. Am J Surg Pathol 34:1233–1240. CrossRefPubMedGoogle Scholar
  28. 28.
    Kim JI, Cho JY, Moon KC, Lee HJ, Kim SH (2009) Segmental enhancement inversion at biphasic multidetector CT: characteristic finding of small renal oncocytoma. Radiology 252:441–448. CrossRefPubMedGoogle Scholar
  29. 29.
    Welsch MJ, Krunic A, Medenica MM (2005) Birt–Hogg–Dube syndrome. Int J Dermatol 44:668–673. CrossRefPubMedGoogle Scholar
  30. 30.
    Armah HB, Parwani AV (2010) Xp11.2 translocation renal cell carcinoma. Arch Pathol Lab Med 134:124–129. CrossRefPubMedGoogle Scholar
  31. 31.
    Chen X, Zhu Q, Li B, et al. (2016) Renal cell carcinoma associated with Xp11.2 translocation/TFE gene fusion: imaging findings in 21 patients. Eur Radiol . CrossRefPubMedPubMedCentralGoogle Scholar
  32. 32.
    Weleber RG, Watzke RC, Shults WT, et al. (2005) Clinical and electrophysiologic characterization of paraneoplastic and autoimmune retinopathies associated with antienolase antibodies. Am J Ophthalmol 139:780–794. CrossRefPubMedGoogle Scholar
  33. 33.
    Klatte T, Streubel B, Wrba F, et al. (2012) Renal cell carcinoma associated with transcription factor E3 expression and Xp11.2 translocation: incidence, characteristics, and prognosis. Am J Clin Pathol 137:761–768. CrossRefPubMedGoogle Scholar
  34. 34.
    Woo S, Kim SY, Lee MS, et al. (2015) MDCT findings of renal cell carcinoma associated with Xp11.2 translocation and TFE3 gene fusion and papillary renal cell carcinoma. Am J Roentgenol 204:542–549. CrossRefGoogle Scholar
  35. 35.
    Suzigan S, López-Beltrán A, Montironi R, et al. (2006) Multilocular cystic renal cell carcinoma: a report of 45 cases of a kidney tumor of low malignant potential. Am J Clin Pathol 125:217–222. CrossRefPubMedPubMedCentralGoogle Scholar
  36. 36.
    Hindman NM, Bosniak MA, Rosenkrantz AB, Lee-Felker S, Melamed J (2012) Multilocular cystic renal cell carcinoma: comparison of imaging and pathologic findings. Am J Roentgenol 198:20–26. CrossRefGoogle Scholar
  37. 37.
    Li T, Chen J, Jiang Y, et al. (2016) Multilocular cystic renal cell neoplasm of low malignant potential: a series of 76 cases. Clin Genitourin Cancer 14:e553–e557. CrossRefPubMedGoogle Scholar
  38. 38.
    Freire M, Remer EM (2009) Clinical and radiologic features of cystic renal masses. Am J Roentgenol 192:1367–1372. CrossRefGoogle Scholar
  39. 39.
    Kwon KA, Oh SY, Kim HY, et al. (2014) Clinical features and treatment of collecting duct carcinoma of the kidney from the korean cancer study group genitourinary and gynecology cancer committee. Cancer Res Treat 46:141–147. CrossRefPubMedPubMedCentralGoogle Scholar
  40. 40.
    Dason S, Allard C, Sheridan-Jonah A, et al. (2013) Management of renal collecting duct carcinoma: a systematic review and the McMaster experience. Curr Oncol . CrossRefPubMedPubMedCentralGoogle Scholar
  41. 41.
    Ciszewski S, Jakimów A, Smolska-ciszewska B (2015) Collecting (Bellini) duct carcinoma : a clinical study of a rare tumour and review of the literature. Can Urol Assoc J . CrossRefPubMedPubMedCentralGoogle Scholar
  42. 42.
    Kuroda N, Toi M, Hiroi M, Enzan H (2002) Review of collecting duct carcinoma with focus on clinical and pathobiological aspects. Histol Histopathol 17:1329–1334Google Scholar
  43. 43.
    Hu Y, Lu G-M, Li K, Zhang L-J, Zhu H (2014) Collecting duct carcinoma of the kidney: imaging observations of a rare tumor. Oncol Lett 7:519–524. CrossRefGoogle Scholar
  44. 44.
    Pickhardt PJ, Siegel CL, McLarney JK (2001) Collecting duct carcinoma of the kidney: are imaging findings suggestive of the diagnosis? Am J Roentgenol 176:627–633. CrossRefGoogle Scholar
  45. 45.
    Yoon SK, Nam KJ, Rha S-H, et al. (2006) Collecting duct carcinoma of the kidney: CT and pathologic correlation. Eur J Radiol 57:453–460. CrossRefGoogle Scholar
  46. 46.
    Baig MA, Lin Y-S, Rasheed J, Mittman N (2006) Renal medullary carcinoma. J Natl Med Assoc 98:1171–1174PubMedPubMedCentralGoogle Scholar
  47. 47.
    Silvino MCM, Moniz CMV, Piotto GHM, et al. (2013) Renal medullary carcinoma response to chemotherapy: a referral center experience in Brazil. Rare Tumors . CrossRefGoogle Scholar
  48. 48.
    Srigley JR, Delahunt B (2009) Uncommon and recently described renal carcinomas. Mod Pathol . CrossRefPubMedGoogle Scholar
  49. 49.
    Davidson AJ, Choyke PL, Hartman DS, Davis CJ (1995) Renal medullary carcinoma associated with sickle cell trait: radiologic findings. Radiology 195:83–85. CrossRefPubMedGoogle Scholar
  50. 50.
    Berkenblit RG, Rozenblit AM, Levin TL, et al. (2005) Renal medullary carcinoma: CT and MRI features. Am J Roentgenol 185:268–272CrossRefGoogle Scholar
  51. 51.
    Omiyale AO, Venyo AKG (2013) Primary carcinoid tumour of the kidney: a review of the literature. Adv Urol . CrossRefPubMedPubMedCentralGoogle Scholar
  52. 52.
    Degnan AJ, Tadros SS, Tocchio S (2017) Pediatric neuroendocrine carcinoid tumors: review of diagnostic imaging findings and recent advances. Am J Roentgenol 208:868–877CrossRefGoogle Scholar
  53. 53.
    Lane B, Jour G, Zhou M (2009) Renal neuroendocrine tumors. Indian J Urol 25:155. CrossRefPubMedPubMedCentralGoogle Scholar
  54. 54.
    Lonergan GJ, Martínez-León MI, Agrons GA, Montemarano H, Suarez ES (1995) Nephrogenic rests, nephroblastomatosis, and associated lesions of the kidney. Radiographics 18:947–968. CrossRefGoogle Scholar
  55. 55.
    Lowe LH, Isuani BH, Heller RM, et al. (2000) Pediatric renal masses: Wilms tumor and beyond. Radiographics 20:1585–1603. CrossRefGoogle Scholar
  56. 56.
    Sethi AT, Das Narla L, Fitch SJ, Frable WJ (2010) Best cases from the AFIP: Wilms tumor in the setting of bilateral nephroblastomatosis. Radiographics . CrossRefPubMedGoogle Scholar
  57. 57.
    Samaratunga H, Delahunt B (2015) Mesenchymal tumors of adult kidney. Semin Diagn Pathol 32:160–171. CrossRefPubMedGoogle Scholar
  58. 58.
    Katabathina VS, Vikram R, Nagar AM, et al. (2010) Mesenchymal neoplasms of the kidney in adults: imaging spectrum with radiologic–pathologic correlation. RadioGraphics 30:1525–1540. CrossRefPubMedGoogle Scholar
  59. 59.
    Brisse HJ, Smets AM, Kaste SC, Owens CM (2008) Imaging in unilateral Wilms tumour. Pediatr Radiol 38:18–29CrossRefGoogle Scholar
  60. 60.
    Sheth S, Ali S, Fishman E (2006) Imaging of renal lymphoma: patterns of disease with pathologic correlation. Radiographics 26:1151–1168. CrossRefPubMedGoogle Scholar
  61. 61.
    Shetty S (2016) Primary renal lymphoma: a case report and review of literature. J Clin Diagn Res 10:9–11. CrossRefGoogle Scholar
  62. 62.
    Chen X, Hu D, Fang L, et al. (2016) Primary renal lymphoma: a case report and literature review. Oncol Lett 12:4001–4008. CrossRefPubMedPubMedCentralGoogle Scholar
  63. 63.
    Ganeshan D, Iyer R, Devine C, Bhosale P, Paulson E (2013) Imaging of primary and secondary renal lymphoma. Am J Roentgenol 201:W712–W719CrossRefGoogle Scholar
  64. 64.
    Purysko AS, Westphalen AC, Remer EM, et al. (2016) Imaging manifestations of hematologic diseases with renal and perinephric involvement. RadioGraphics 36:1038–1054. CrossRefPubMedGoogle Scholar
  65. 65.
    Bach AG, Behrmann C, Holzhausen HJ, et al. (2012) Prevalence and patterns of renal involvement in imaging of malignant lymphoproliferative diseases. Acta Radiol 53:343–348CrossRefGoogle Scholar
  66. 66.
    Choyke PL, White EM, Zeman RK, Jaffe MH, Clark LR (1987) Renal metastases: clinicopathologic and radiologic correlation. Radiology 162:359–363. CrossRefPubMedGoogle Scholar
  67. 67.
    Zhou C, Urbauer DL, Fellman BM, et al. (2016) Metastases to the kidney: a comprehensive analysis of 151 patients from a tertiary referral centre. BJU Int . CrossRefPubMedGoogle Scholar
  68. 68.
    Jain Y, Liew S, Taylor MB, Bonington SC (2011) Is dual-phase abdominal CT necessary for the optimal detection of metastases from renal cell carcinoma? Clin Radiol 66:1055–1059. CrossRefPubMedGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of Diagnostic RadiologyUniversity of California, San FranciscoSan FranciscoUSA
  2. 2.Department of Abdominal ImagingUniversity of California, San FranciscoSan FranciscoUSA
  3. 3.Department of Anatomic Pathology, Radiation Oncology, UrologyUniversity of California, San FranciscoSan FranciscoUSA

Personalised recommendations