Multilocular Cystic Nephroma Imaging
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In the past, multilocular cystic renal tumors have been considered to be lesions of developmental origin, hamartomas, or hamartomas with malignant potential (see the images below). In 1956, Boggs and Kimmelstiel first proposed the true neoplastic nature of the lesions in a case report, suggesting the term benign multilocular cystic nephroma for this condition. [1, 2, 3]
Joshi and Beckwith proposed a modification to the existing terminology. [4] Their modification emphasized a neoplastic rather than a developmental or hamartomatous origin. First, they recommended that the term cystic nephroma be used to describe a multicystic tumor lacking blastemal or other embryonal elements. Second, they suggested that the term cystic partially differentiated nephroblastoma (CPDN) be used to denote a predominantly cystic lesion without nodular solid regions and in which the septa contain blastemal or other embryonal elements. Furthermore, they proposed that both terms be used as subsets of the category term multilocular cystic renal tumor.
Ultrasonography (US) is the first radiologic examination performed for the evaluation of any abdominal mass. US can provide the imaging results necessary for diagnosing multilocular cystic nephroma. The diagnosis may be confirmed by using either CT or MRI. Together, US and CT may be the studies of choice because they enable the evaluation of cystic lesions, stromal tissue, and the perfusion of this stroma. No flow is seen within the cystic lesions. [5, 6, 7, 8, 9, 10]
The precision and accuracy of US depends on, and therefore is limited by, the operator’s skill. CT may not be chosen if the patient has a severe allergy to the contrast medium. Compared with US and fast CT, MRI is limited by the need for sedation in some patients.
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. [11]
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.
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.
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.
CT findings are dependent on the size of the cyst and the amount of stromal tissue (see the images below). [8] In most cases, the mass is identified with the following CT findings [12] :
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.
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-positive and false-negative rates are low because of the accuracy of the method.
MRI demonstrates the low signal intensity of the tumor capsule (see the images below).
Observations of nonenhanced MRIs have shown encapsulated masses with dividing septa between cystic spaces.
Kettritz et al [13] 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.
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-positive and false-negative rates are low because of the accuracy of the method.
Results of ultrasonography depend on the amount of stroma and the size of the loculi (see the images below). [14, 9]
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.
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-positive and false-negative rates are low because of the accuracy of the method.
Scintigraphy of the kidneys can be performed. Scintigrams demonstrate a defect corresponding to the renal mass.
Nuclear medicine studies have 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. The lack of precision results in high false-positive and false-negative rates because of the inaccuracy of the method.
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.
Angiography has a high degree of confidence. The structures of lesions on the kidneys are well differentiated with this method, which results in a precise diagnosis. As a result, angiography has low false-positive and false-negative rates.
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.
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. 2008 Oct 23. 1(1):267. [Medline].
Silver IM, Boag AH, Soboleski DA. Best cases from the AFIP: Multilocular cystic renal tumor: cystic nephroma. Radiographics. 2008 Jul-Aug. 28(4):1221-5; discussion 1225-6. [Medline].
Joshi VV, Beckwith JB. Multilocular cyst of the kidney (cystic nephroma) and cystic, partially differentiated nephroblastoma. Terminology and criteria for diagnosis. Cancer. 1989 Jul 15. 64(2):466-79. [Medline].
Dalla-Palma L, Pozzi-Mucelli F, di Donna A, Pozzi-Mucelli RS. Cystic renal tumors: US and CT findings. Urol Radiol. 1990. 12(2):67-73. [Medline].
Wilkinson C, Palit V, Bardapure M, Thomas J, Browning AJ, Gill K, et al. Adult multilocular cystic nephroma: Report of six cases with clinical, radio-pathologic correlation and review of literature. Urol Ann. 2013 Jan. 5(1):13-7. [Medline]. [Full Text].
Katabathina VS, Garg D, Prasad SR, Vikram R. Cystic renal neoplasms and renal neoplasms associated with cystic renal diseases in adults: cross-sectional imaging findings. J Comput Assist Tomogr. 2012 Nov-Dec. 36(6):659-68. [Medline].
Nicolau C, Buñesch L, Paño B, Salvador R, Ribal MJ, Mallofré C, et al. Prospective evaluation of CT indeterminate renal masses using US and contrast-enhanced ultrasound. Abdom Imaging. 2015 Mar. 40 (3):542-51. [Medline].
Karmazyn B, Tawadros A, Delaney LR, Marine MB, Cain MP, Rink RC, et al. Ultrasound classification of solitary renal cysts in children. J Pediatr Urol. 2015 Jun. 11 (3):149.e1-6. [Medline].
Subira Rios J, Sanchez Zalabardo JM, Elizalde Benito A, Navarro Gíl J, Hijazo Conejos JI, García-Magariño Alonso J, et al. [Multilocular cystic nephroma. Report of three new cases]. Arch Esp Urol. 2009 Jan-Feb. 62 (1):62-6. [Medline].
La Parra Casado C, Muro Velilla D, Molina Fàbrega R, Sangüesa Nebot C. [Radiologic findings in non-Wilms’ renal tumors in children]. Radiologia. 2008 May-Jun. 50(3):215-24. [Medline].
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. 2007 May. 29(5):382-5. [Medline].
Kettritz U, Semelka RC, Siegelman ES, et al. Multilocular cystic nephroma: MR imaging appearance with current techniques, including gadolinium enhancement. J Magn Reson Imaging. 1996 Jan-Feb. 6(1):145-8. [Medline].
Deeg KH, Gerdemann C, Weingärtner K, Seitz G. Sonographic diagnosis of an unusual case of multilocular cystic nephroma mimicking polycystic kidney disease. Ultraschall Med. 2008 Dec. 29 Suppl 5:264-7. [Medline].
Henrique M Lederman, MD, PhD 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.
Peter J Hurh Research Fellow, Department of Radiology, The Children’s Hospital of Philadelphia
Disclosure: Nothing to disclose.
Bernard D Coombs, MB, ChB, PhD Consulting Staff, Department of Specialist Rehabilitation Services, Hutt Valley District Health Board, New Zealand
Disclosure: Nothing to disclose.
Brian H Kopell, MD Associate Professor, Department of Neurosurgery, Icahn School of Medicine at Mount Sinai
Brian H Kopell, MD is a member of the following medical societies: Alpha Omega Alpha, American Association of Neurological Surgeons, American Society for Stereotactic and Functional Neurosurgery, Congress of Neurological Surgeons, International Parkinson and Movement Disorder Society, North American Neuromodulation Society
Disclosure: Received consulting fee from Medtronic for consulting; Received consulting fee from Abbott Neuromodulation for consulting.
Lori Lee Barr, MD, FACR, FAIUM Clinical Assistant Professor of Radiology, University of Texas Medical Branch at Galveston School of Medicine; 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 Roentgen Ray Society, Association of University Radiologists, Southern Medical Association, Undersea and Hyperbaric Medical Society, American Society of Pediatric Neuroradiology, Society of Radiologists in Ultrasound, Texas Radiological Society, American Association for Women Radiologists, American College of Radiology, American Institute of Ultrasound in Medicine, Radiological Society of North America, Society for Pediatric Radiology
Disclosure: Nothing to disclose.
Multilocular Cystic Nephroma Imaging
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