eMedicine Specialties > Radiology > Pediatrics

Multilocular Cystic Nephroma: Imaging

Author: Henrique M Lederman, MD, PhD, Consulting Staff, Department of Radiology, LeBonheur Children's Medical Center and St Jude Children's Research Hospital; Professor of Radiology and Pediatric Radiology, Chief, Division of Diagnostic Imaging in Pediatrics, Federal University of Sao Paulo, Brazil
Coauthor(s): Peter J Hurh, Research Fellow, Department of Radiology, The Children's Hospital of Philadelphia
Contributor Information and Disclosures

Updated: Apr 7, 2009

Radiography

Findings

On plain radiographs, cystic nephroma and cystic partially differentiated nephroblastoma (CPDN) cannot be distinguished, just as they cannot be distinguished by their gross anatomic appearances. However, in either type of multilocular cystic renal tumor, radiography does reveal displacement of the bowel and adjacent structures if the lesion is of sufficient size. Calcification, although uncommon, can be present and is defined as curvilinear and peripheral localizations.8

On excretory urography, the kidneys function normally with multilocular cystic renal tumors. Because the mass is rarely suspected on the basis of the clinical findings, excretory urography reveals nonspecific stretching, displacement, extension of the tumor to the renal pelvis, and attenuation of the renal collecting system by the mass. Delayed or absent excretion is also demonstrated.

Degree of Confidence

A general nonspecific renal tumor can be identified by using radiography, with some degree of confidence. However, the use of radiography to diagnose multilocular cystic nephroma specifically results in a low degree of confidence. On plain radiographs, multilocular cystic renal tumor appears similar to other renal tumors because plain radiography cannot be used to differentiate between cystic and solid lesions.

False Positives/Negatives

The occurrence of false-positive and false-negative results is high because plain radiography cannot be used to differentiate between specific structures, and the study has low precision in the diagnosis of these tumors.

Computed Tomography


Multilocular cystic nephroma. CT shows a normal r...

Multilocular cystic nephroma. CT shows a normal right kidney and an enlarged left kidney with a large well-defined mass, with several hypoattenuating cysts. No enhancement or septation is noted.

Multilocular cystic nephroma. CT shows a normal r...

Multilocular cystic nephroma. CT shows a normal right kidney and an enlarged left kidney with a large well-defined mass, with several hypoattenuating cysts. No enhancement or septation is noted.


Multilocular cystic nephroma. CT shows a normal r...

Multilocular cystic nephroma. CT shows a normal right kidney and an enlarged left kidney with a large well-defined mass, with several cysts with hypoattenuation. No enhancement or septation is noted. Perfusion can be seen crossing the midline on part of the remaining left kidney.

Multilocular cystic nephroma. CT shows a normal r...

Multilocular cystic nephroma. CT shows a normal right kidney and an enlarged left kidney with a large well-defined mass, with several cysts with hypoattenuation. No enhancement or septation is noted. Perfusion can be seen crossing the midline on part of the remaining left kidney.


Multilocular cystic nephroma. CT shows a normal r...

Multilocular cystic nephroma. CT shows a normal right kidney and an enlarged left kidney with a large well-defined mass, with several cysts with hypoattenuation. No enhancement or septation is noted. Perfusion is seen crossing the midline on part of the remaining left kidney, with some signs of dilatation of the collecting system.

Multilocular cystic nephroma. CT shows a normal r...

Multilocular cystic nephroma. CT shows a normal right kidney and an enlarged left kidney with a large well-defined mass, with several cysts with hypoattenuation. No enhancement or septation is noted. Perfusion is seen crossing the midline on part of the remaining left kidney, with some signs of dilatation of the collecting system.


Multilocular cystic nephroma. CT shows a normal r...

Multilocular cystic nephroma. CT shows a normal right kidney and an enlarged left kidney with a large well-defined mass, with several cysts with hypoattenuation. No enhancement or septation is noted. Perfusion is seen crossing the midline on part of the remaining left kidney, with some signs of dilatation of the collecting system.

Multilocular cystic nephroma. CT shows a normal r...

Multilocular cystic nephroma. CT shows a normal right kidney and an enlarged left kidney with a large well-defined mass, with several cysts with hypoattenuation. No enhancement or septation is noted. Perfusion is seen crossing the midline on part of the remaining left kidney, with some signs of dilatation of the collecting system.


Multilocular cystic nephroma. CT shows a large mu...

Multilocular cystic nephroma. CT shows a large multilocular cystic nephroma involving the right kidney and crossing the midline.

Multilocular cystic nephroma. CT shows a large mu...

Multilocular cystic nephroma. CT shows a large multilocular cystic nephroma involving the right kidney and crossing the midline.


Multilocular cystic nephroma. CT shows a large mu...

Multilocular cystic nephroma. CT shows a large multilocular cystic nephroma involving the right kidney and crossing the midline.

Multilocular cystic nephroma. CT shows a large mu...

Multilocular cystic nephroma. CT shows a large multilocular cystic nephroma involving the right kidney and crossing the midline.


Findings

CT findings are dependent on the size of the cyst and the amount of stromal tissue. In most cases, the mass is identified with the following CT findings9 :

  • Well-defined margins
  • Multicystic architecture
  • Enhancing septa
  • Herniation into the renal collecting system

Cystic spaces are not enhancing and demonstrate CT numbers slightly higher than those of water. In some cases, the entire mass or portions of the mass may appear solid because of smaller closely spaced cysts.

Degree of Confidence

CT results in a high degree of confidence in the diagnosis of multilocular cystic nephroma. CT images define the structures of lesions on the kidneys well.

False Positives/Negatives

False-positive and false-negative rates are low because of the accuracy of the method.

Magnetic Resonance Imaging


Multilocular cystic nephroma. Axial MRI shows a l...

Multilocular cystic nephroma. Axial MRI shows a large multilocular cystic nephroma involving the right kidney and crossing the midline.

Multilocular cystic nephroma. Axial MRI shows a l...

Multilocular cystic nephroma. Axial MRI shows a large multilocular cystic nephroma involving the right kidney and crossing the midline.


Multilocular cystic nephroma. Axial MRI shows a l...

Multilocular cystic nephroma. Axial MRI shows a large multilocular cystic nephroma involving the right kidney and crossing the midline.

Multilocular cystic nephroma. Axial MRI shows a l...

Multilocular cystic nephroma. Axial MRI shows a large multilocular cystic nephroma involving the right kidney and crossing the midline.


Multilocular cystic nephroma. Sagittal MRI shows ...

Multilocular cystic nephroma. Sagittal MRI shows a large multilocular cystic nephroma involving the right kidney.

Multilocular cystic nephroma. Sagittal MRI shows ...

Multilocular cystic nephroma. Sagittal MRI shows a large multilocular cystic nephroma involving the right kidney.


Findings

  • MRI demonstrates the low signal intensity of the tumor capsule.
  • Observations of nonenhanced MRIs have shown encapsulated masses with dividing septa between cystic spaces.
  • Kettritz et al6 found the following:
    • On T1-weighted sequences, signal intensity varies from low to very high and from low to intermediate.
    • T2-weighted sequencing resulted in low signal intensity in the tumor capsule and the intermediate septations.
    • The signal intensity of the cysts was high in all cases.
    • Variable signal intensity from the cyst contents is attributed to differing concentrations of old hemorrhage and protein.

Degree of Confidence

MRI results in a high degree of confidence in the diagnosis of multilocular cystic nephroma. MRI images define the structures of lesions on the kidneys well.

False Positives/Negatives

False-positive and false-negative rates are low because of the accuracy of the method.

Ultrasonography


Multilocular cystic nephroma. Sonograms show a no...

Multilocular cystic nephroma. Sonograms show a normal right kidney and, within an enlarged left kidney, multiple anechoic cystic changes separated by hyperechoic septa.

Multilocular cystic nephroma. Sonograms show a no...

Multilocular cystic nephroma. Sonograms show a normal right kidney and, within an enlarged left kidney, multiple anechoic cystic changes separated by hyperechoic septa.


Multilocular cystic nephroma. Sonogram shows mult...

Multilocular cystic nephroma. Sonogram shows multiple anechoic cystic changes separated by hyperechoic septa within an enlarged left kidney.

Multilocular cystic nephroma. Sonogram shows mult...

Multilocular cystic nephroma. Sonogram shows multiple anechoic cystic changes separated by hyperechoic septa within an enlarged left kidney.


Multilocular cystic nephroma. Sonogram shows mult...

Multilocular cystic nephroma. Sonogram shows multiple anechoic cystic changes separated by hyperechoic septa within an enlarged left kidney.

Multilocular cystic nephroma. Sonogram shows mult...

Multilocular cystic nephroma. Sonogram shows multiple anechoic cystic changes separated by hyperechoic septa within an enlarged left kidney.


Multilocular cystic nephroma. Color Doppler sonog...

Multilocular cystic nephroma. Color Doppler sonogram shows multiple anechoic cystic changes separated by hyperechoic septa within an enlarged left kidney without Doppler flow.

Multilocular cystic nephroma. Color Doppler sonog...

Multilocular cystic nephroma. Color Doppler sonogram shows multiple anechoic cystic changes separated by hyperechoic septa within an enlarged left kidney without Doppler flow.


Multilocular cystic nephroma. Color Doppler sonog...

Multilocular cystic nephroma. Color Doppler sonogram shows multiple anechoic cystic changes separated by hyperechoic septa within an enlarged left kidney without Doppler flow.

Multilocular cystic nephroma. Color Doppler sonog...

Multilocular cystic nephroma. Color Doppler sonogram shows multiple anechoic cystic changes separated by hyperechoic septa within an enlarged left kidney without Doppler flow.


Findings

Results of US depend on the amount of stroma and the size of the loculi.10

  • The appearance of multilocular cystic renal tumor includes multiple anechoic spaces separated by hyperechoic septa. This pattern is similar to that of multilocular cystic nephroma; however, if the loculi are small, the tumor mimics an echogenic solid mass.
  • In most patients, the renal origin of the mass can be confirmed by identifying a beak or claw of normal renal parenchyma around the periphery of a well-defined mass, by the splaying or displacement of the renal collecting system, and by synchronous motion of the mass and kidney with respiratory excursion.
  • Color Doppler US can also be used to evaluate tumors and can provide a noninvasive assessment of lesion vascularity. This is possible because of the Doppler-shifted signals of abnormally high velocity emitted by low-resistance neovascularity in some neoplasms.

Degree of Confidence

US can be used with a high degree of confidence. A diagnosis can be made with high precision because sonograms clearly depict the structure of the lesions.

False Positives/Negatives

False-positive and false-negative rates are low because of the accuracy of the method.

Nuclear Imaging

Findings

Scintigraphy of the kidneys can be performed. Scintigrams demonstrate a defect corresponding to the renal mass.

Degree of Confidence

Nuclear medicine studies result in a low degree of confidence in the diagnosis of multilocular cystic nephroma specifically. Although a general nonspecific renal mass can be identified, details cannot be differentiated.

False Positives/Negatives

The lack of precision results in high false-positive and false-negative rates because of the inaccuracy of the method.

Angiography

Findings

On angiographic examination, multilocular cystic renal tumors usually appear hypovascular, although they may also be avascular or hypervascular. Angiography may reveal features that are not specific for differential diagnosis. Magnetic resonance angiography may help if a preoperative evaluation of the vascular anatomy is needed.

Degree of Confidence

Angiography results in a high degree of confidence. The structures of lesions on the kidneys are well differentiated with this method, which results in a precise diagnosis.

False Positives/Negatives

Angiography has low false-positive and false-negative rates because of the accuracy of the method.

More on Multilocular Cystic Nephroma

Overview: Multilocular Cystic Nephroma
Imaging: Multilocular Cystic Nephroma
Multimedia: Multilocular Cystic Nephroma
References
Further Reading

References

  1. Boggs LK, Kimmelstiel P. Benign multilocular cystic nephroma: report of two cases of so-called multilocular cyst of the kidney. J Urol. 1956;76:530-41.

  2. Stamatiou K, Polizois K, Kollaitis G, Dahanis S, Zafeiropoulos G, Leventis C, et al. Cystic nephroma: a case report and review of the literature. Cases J. Oct 23 2008;1(1):267. [Medline].

  3. Silver IM, Boag AH, Soboleski DA. Best cases from the AFIP: Multilocular cystic renal tumor: cystic nephroma. Radiographics. Jul-Aug 2008;28(4):1221-5; discussion 1225-6. [Medline].

  4. Joshi VV, Beckwith JB. Multilocular cyst of the kidney (cystic nephroma) and cystic, partially differentiated nephroblastoma. Terminology and criteria for diagnosis. Cancer. Jul 15 1989;64(2):466-79. [Medline].

  5. Madewell JE, Goldman SM, Davis CJ Jr, et al. Multilocular cystic nephroma: a radiographic-pathologic correlation of 58 patients. Radiology. Feb 1983;146(2):309-21. [Medline].

  6. Kettritz U, Semelka RC, Siegelman ES, et al. Multilocular cystic nephroma: MR imaging appearance with current techniques, including gadolinium enhancement. J Magn Reson Imaging. Jan-Feb 1996;6(1):145-8. [Medline].

  7. Castillo OA, Boyle ET Jr, Kramer SA. Multilocular cysts of kidney. A study of 29 patients and review of literature. Urology. Feb 1991;37(2):156-62. [Medline].

  8. La Parra Casado C, Muro Velilla D, Molina Fàbrega R, Sangüesa Nebot C. [Radiologic findings in non-Wilms' renal tumors in children]. Radiologia. May-Jun 2008;50(3):215-24. [Medline].

  9. Han XN, Peng LR, Liu GH, Wang J. [Multiphasic spiral CT scanning features in 100 patients with small renal cell carcinoma]. Zhonghua Zhong Liu Za Zhi. May 2007;29(5):382-5. [Medline].

  10. Deeg KH, Gerdemann C, Weingärtner K, Seitz G. Sonographic diagnosis of an unusual case of multilocular cystic nephroma mimicking polycystic kidney disease. Ultraschall Med. Dec 2008;29 Suppl 5:264-7. [Medline].

  11. Abt AB, Demers LM, Shochat SJ. Cystic nephroma: an ultrastructural and biochemical study. J Urol. Oct 1979;122(4):539-41. [Medline].

  12. Agrons GA, Wagner BJ, Davidson AJ, Suarez ES. Multilocular cystic renal tumor in children: radiologic-pathologic correlation. Radiographics. May 1995;15(3):653-69. [Medline].

  13. Banner MP, Pollack HM, Chatten J, Witzleben C. Multilocular renal cysts: radiologic-pathologic correlation. AJR Am J Roentgenol. Feb 1981;136(2):239-47. [Medline].

  14. Brown JM. Cystic partially differentiated nephroblastoma. J Pathol. Mar 1975;115(3):175-8. [Medline].

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  20. Edmonds W. Cystic adenoma of the kidney. Trans Pathol Soc London. 1892;43:89-90.

  21. Fowler M. Differentiated nephroblastoma: solid, cystic or mixed. J Pathol. Nov 1971;105(3):215-8. [Medline].

  22. Gervais DA, Whitman GJ, Chew FS. Multilocular cyst of the kidney. AJR Am J Roentgenol. Sep 1993;161(3):600. [Medline].

  23. Landing BH. Well-differentiated and polycystic (benign?) Wilms' tumor. Cancer Semin. 1968;2:110-3.

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Keywords

multilocular cystic nephroma, multilocular cystic renal tumor, cystic nephroma, cystic partially differentiated nephroblastoma, CPDN, multilocular cystic renal tumor, benign multilocular cystic nephroma, polycystic nephroblastoma, differentiated nephroblastoma, well-differentiated polycystic Wilms tumor, benign cystic differentiated nephroblastoma, cystic partially differentiated nephroblastoma

Contributor Information and Disclosures

Author

Henrique M Lederman, MD, PhD, Consulting Staff, Department of Radiology, LeBonheur Children's Medical Center and St Jude Children's Research Hospital; Professor of Radiology and Pediatric Radiology, Chief, Division of Diagnostic Imaging in Pediatrics, Federal University of Sao Paulo, Brazil
Henrique M Lederman, MD, PhD is a member of the following medical societies: Society for Pediatric Radiology
Disclosure: Nothing to disclose.

Coauthor(s)

Peter J Hurh, Research Fellow, Department of Radiology, The Children's Hospital of Philadelphia
Disclosure: Nothing to disclose.

Medical Editor

Lori Lee Barr, MD, FACR, FAIUM, Clinical Associate Professor of Radiology, University of Texas Health Science Center in San Antonio; Clinical Assistant Professor of Radiology, University of Texas Medical Branch at Galveston; Member, Board of Directors, Austin Radiological Association; Consulting Staff, Seton Health Network, Columbia/St David's Healthcare System, Healthsouth Rehabilitation Hospital of Austin, Georgetown Hospital, St Mark's Medical Center, Cedar Park Regional Medical Center
Lori Lee Barr, MD, FACR, FAIUM is a member of the following medical societies: American Association for Women Radiologists, American College of Radiology, American Institute of Ultrasound in Medicine, American Roentgen Ray Society, American Society of Pediatric Neuroradiology, Association of University Radiologists, Radiological Society of North America, Society for Pediatric Radiology, Society of Radiologists in Ultrasound, Southern Medical Association, Texas Radiological Society, and Undersea and Hyperbaric Medical Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Bernard D Coombs, MB, ChB, PhD, Consulting Staff, Department of Specialist Rehabilitation Services, Hutt Valley District Health Board, New Zealand
Disclosure: Nothing to disclose.

CME Editor

Robert M Krasny, MD, Consulting Staff, Department of Radiology, The Angeles Clinic and Research Institute
Robert M Krasny, MD is a member of the following medical societies: American Roentgen Ray Society and Radiological Society of North America
Disclosure: Nothing to disclose.

Chief Editor

Lawrence M Davis, MD, Assistant Professor of Diagnostic Imaging (Clinical), Department of Diagnostic Imaging, Warren Alpert Medical School at Brown University
Lawrence M Davis, MD is a member of the following medical societies: American College of Radiology, American Roentgen Ray Society, American Society of Neuroradiology, Radiological Society of North America, and Rhode Island Medical Society
Disclosure: Nothing to disclose.

 
 
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