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fm — 12/7/07
Prasad et al. Genitourinar y Imaging • Review
Benign Renal Neoplasms

Benign Renal Neoplasms in Adults:


Cross-Sectional Imaging Findings
Srinivasa R. Prasad1 OBJECTIVE. A broad spectrum of benign renal neoplasms in adults shows characteristic
Venkateswar R. Surabhi1 ontogeny, histology, and tumor biology. Benign renal tumors are classified into renal cell tu-
Christine O. Menias2 mors, metanephric tumors, mesenchymal tumors, and mixed epithelial and mesenchymal tu-
Abhijit A. Raut3 mors. Select benign tumors show characteristic anatomic distribution and imaging features.
Kedar N. Chintapalli1 However, because of overlapping of findings between benign and malignant renal tumors, his-
tologic evaluation may be required to establish a definitive diagnosis. Accurate preoperative
Prasad SR, Surabhi VR, Menias CO, Raut AA, characterization facilitates optimal patient management.
Chintapalli KN CONCLUSION. We attempt to provide a comprehensive, contemporary review of benign
renal neoplasms that occur in adults, focusing on cross-sectional imaging characteristics.

enign renal neoplasms that occur 79% sample adequacy [7]. However, patholo-

B in adults constitute a heteroge-


neous group of tumors with char-
acteristic histology and variable
gists advise caution when interpreting tumors,
specifically those with oncocytic features or hy-
brid or collision tumors [7, 9]. Laparoscopic
clinicobiologic profiles. The 2004 World partial nephrectomies and percutaneous abla-
Health Organization (WHO) classification tions are being increasingly performed to treat
schemata categorizes benign renal neoplasms small renal tumors and to establish a definitive
on the basis of histogenesis (cell of origin) diagnosis [10, 11].
and histopathology [1] (Appendix 1). Renal
neoplasms are thus classified into renal cell, Renal Cell Neoplasms
metanephric, mesenchymal, and mixed epi- Oncocytoma
thelial and mesenchymal tumors. Oncocytoma is a benign renal cell neoplasm
Keywords: benign tumors, CT, kidney, MRI, renal
neoplasms, sonography
Recent advances in imaging technology that accounts for approximately 5% of all adult
have resulted in the detection of incidental renal primary renal epithelial neoplasms in surgical
DOI:10.2214/AJR.07.2724 masses in seemingly asymptomatic patients. series [1]. Oncocytoma is hypothesized to
Although renal cell carcinoma (RCC) is by far originate from or differentiate toward type A
Received June 13, 2007; accepted after revision
the most lethal urologic malignancy, benign tu- intercalated cells of the cortical collecting duct
July 16, 2007.
mors constitute a significant proportion of [12, 13]. The peak age of incidence is in the
1Department of Radiology, University of Texas Health masses in patients who undergo surgery. In a re- seventh decade; men are more likely to be af-
Science Center at San Antonio, 7703 Floyd Curl Dr., cent study of 143 patients with presumed soli- fected than women. Most tumors occur spo-
San Antonio, TX 78229. Address correspondence to tary RCC, the authors found 16.1% of patients radically in asymptomatic patients.
S. R. Prasad (prasads@uthscsa.edu).
who underwent partial nephrectomy had be- Oncocytoma is histologically composed of
2Department of Radiology, Mallinckrodt Institute of nign masses [2]. Other studies have also found nests and acini of large polygonal cells with
Radiology, St. Louis, MO. that a significant proportion of solid renal mitochondria-rich eosinophilic cytoplasm
masses are histologically benign [3–5]. Also, [1]. Oncocytomas do not show diffuse cyto-
3Department of Radiology, King Edward Memorial Hospital,
percutaneous renal mass biopsy is being in- plasmic Hale colloidal iron staining, in con-
Mumbai, India.
creasingly performed to preoperatively charac- tradistinction to chromophobe RCCs.
CME terize renal masses and to establish definitive Oncocytomas typically appear as solitary,
This article is available for CME credit. See www.arrs.org diagnoses [3, 4, 6, 7]. Recent advances in histo- well-demarcated, unencapsulated, fairly ho-
for more information. pathology, immunocytochemistry, and cytoge- mogeneous renal cortical tumors. Bilateral,
AJR 2008; 190:158–164
netics assist in fairly accurate characterization multicentric oncocytomas are seen in heredi-
of most renal masses and help guide optimal tary syndromes of renal oncocytosis and Birt-
0361–803X/08/1901–158
patient management [1, 7, 8]. A recent study of Hogg-Dubé syndrome (in association with
© American Roentgen Ray Society 66 renal mass biopsies found 98% accuracy and the chromophobe subtype and other RCC

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Benign Renal Neoplasms

A B
Fig. 1—72-year-old man with hereditary oncocytosis Fig. 2—64-year-old man with histologically proven oncocytoma. K = kidney.
syndrome. Coronal contrast-enhanced CT scan during A, Axial fat-saturated, T2-weighted gradient-refocused echo image shows expansile, solid right renal mass
nephrographic phase shows bilateral solid renal (arrow) with hyperintense central scar (S).
masses (arrows) that were characterized as B, Axial fat-saturated, gadolinium-enhanced T1-weighted 3D gradient-refocused echo image shows right kidney
oncocytomas on histopathology. mass (arrow) with hypointense central scar (S).

subtypes) [14] (Fig. 1). A characteristic cen- renal adenomas are indistinguishable from CT; large tumors appear as heterogeneous,
tral stellate fibrotic scar (more often seen with papillary RCCs [1, 20]. hypovascular masses with frequent foci of
large tumors) is seen in up to 33% of tumors Papillary adenomas are extremely small hemorrhage and necrosis [23, 24]. Calcifica-
[1] (Fig. 2). Hemorrhage may be found in up (< 5 mm) and may not be distinguished from tion is seen in 20% of cases. Metanephric ad-
to 20% of cases. A spoke-wheel pattern of other renal tumors (particularly RCC) and enoma shows a hypointense signal on T1-
feeding arteries associated with a homoge- pseudotumors on imaging studies. weighted MRI and a slightly hyperintense
neous nephrogram is a characteristic finding signal on T2-weighted MRI [25]. Meta-
on catheter angiography [15]. However, on- Metanephric Neoplasms nephric adenoma appears as an expansile hy-
cocytomas are indistinguishable from renal Metanephric neoplasms are a heteroge- poechoic or hyperechoic mass on sonogra-
cell carcinomas on the basis of imaging find- neous group of benign renal neoplasms that phy. True cystic forms of metanephric
ings alone. In addition, oncocytomas may be include metanephric adenoma (epithelial tu- adenoma are rare [26].
associated with RCCs either as hybrid tumors mor), metanephric stromal tumor (stromal
(pathologic features of both oncocytomas and neoplasm), and metanephric adenofibroma Mesenchymal Neoplasms
chromophobe or other RCC subtypes) or as (mixed epithelial and stromal neoplasm) [21]. Angiomyolipoma
collision tumors [9]. Thus, despite advances These tumors are histogenetically related to Angiomyolipoma (AML) is the most com-
in histopathologic techniques (including im- Wilms’ tumor and are postulated to represent mon benign mesenchymal neoplasm; it is com-
munocytochemistry and cytogenetics), a par- the most hyperdifferentiated, benign end of posed of variable proportions of blood vessels,
tial nephrectomy may be required for accu- the nephroblastoma spectrum [21]. Meta- smooth muscle, and adipose tissue [1]. AMLs
rate characterization [7]. nephric adenofibromas and metanephric stro- are now included under the umbrella term
mal tumors are essentially pediatric tumors “neoplasms of the perivascular epithelioid
Papillary Adenoma and will not be discussed in this article. cells,” which are also referred to as PEComas
Papillary adenomas are the most common Metanephric adenoma is a benign renal neo- [27]. Renal AMLs consist of two distinct his-
renal epithelial neoplasms. According to au- plasm with peak age of occurrence in the fifth tologic subtypes, classic triphasic and mono-
topsy series, approximately 40% of patients or sixth decade and a 2:1 female preponder- typic epithelioid. Epithelioid AMLs typically
older than 70 years harbor renal adenomas ance [1]. Metanephric adenoma is asymptom- do not show macroscopic fat and appear as
[1]. Papillary adenomas are also commonly atic in approximately 50% of patients; abdom- soft-tissue masses and are thus indistinguish-
found in patients with acquired renal cystic inal pain and hematuria are common clinical able from other solid renal masses. This rare
disease and in patients undergoing long-term symptoms. Polycythemia, a characteristic subtype of AML is potentially malignant and
hemodialysis [16]. A papillary adenoma-to- finding seen in approximately 10% of patients may exhibit aggressive biology, including re-
carcinoma sequence has been described that with metanephric adenoma, promptly disap- currence, metastasis, and death. It will not be
is akin to similar transformation in colonic pears after surgical resection [22]. further discussed in this article [27, 28].
adenomas [17, 18]. Metanephric adenoma is histologically Classic AML may occur either sporadi-
By definition, papillary adenomas measure characterized by the arrangement of monoto- cally or in association with tuberous sclerosis
5 mm or less [1]. They are usually subcapsu- nous small blue embryonal epithelial cells in complex (TSC). Sporadic renal AMLs show a
lar and solitary. Adenomas are histologically an acinar, tubular, or sheetlike configuration 4:1 female preponderance and are more likely
characterized by papillary or tubular cytoar- [21]. Abundant psammoma bodies are com- to be solitary and symptomatic [29]. Patients
chitecture and frequent psammoma bodies monly found. with TSC harbor small, multicentric, asymp-
[1]. Cytogenetic changes of papillary ade- Metanephric adenoma typically appears as tomatic AMLs; 80% of patients with severe
nomas include loss of the Y chromosome and a well-defined, unencapsulated, solitary solid TSC have renal AMLs [30]. The morphology
combined trisomy of chromosomes 7 and 17 mass [21, 22] (Fig. 3). It commonly appears of AMLs depends on the relative proportions
[19]. Histologic and genetic abnormalities of as a hyperattenuating mass on unenhanced of various components. Profuse elastin-poor,

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Fig. 3—Contrast-enhanced axial CT scan in 60-year- Fig. 4—43-year-old woman with hematuria. Transverse Fig. 5—58-year-old woman with angiomyolipoma of
old woman with hematuria shows hypoattenuating, sonogram shows uniformly echogenic mass (arrows) kidney. Sagittal contrast-enhanced CT scan shows
expansile solid mass (arrows) in left kidney. Radical in upper pole of left kidney (K) that was proven to be exophytic renal mass (arrows) with foci of
nephrectomy showed mass to be metanephric angiomyolipoma. macroscopic fat (arrowhead).
adenoma. K = kidney.

A B
Fig. 6—38-year-old woman with documented tuberous sclerosis complex and renal angiomyolipomas. Fig. 7—55-year-old woman who underwent partial
A, Axial in-phase T1-weighted 2D gradient-refocused echo MR image shows bilateral multicentric renal masses nephrectomy for serendipitously detected renal mass.
that have increased signal intensity (arrows). Axial contrast-enhanced CT scan shows exophytic
B, Axial fat-saturated T2-weighted 2D gradient-refocused echo MR image shows marked drop in signal intensity soft-tissue mass (arrow). Histopathology showed lipid-
of masses (arrows). poor angiomyolipoma.

dysmorphic blood vessels predispose to aneu- [33]. Approximately 4.5% of AMLs may not such as Sturge-Weber and Klippel-Trénaunay
rysm formation and hemorrhage [29]. Large show identifiable macroscopic fat and are in- and with systemic angiomatosis [1]. Cavern-
tumor size (> 4 cm) and diameter of the in- distinguishable from RCC on imaging studies ous hemangiomas are more common than the
tralesional aneurysms (> 5 mm) correlate di- alone (Fig. 7). Recent studies indicate that in capillary variants.
rectly with tumor-related hemorrhage in contradistinction to RCCs, AMLs with mini- Hemangioma of the kidney occurs as an
AMLs [31]. mal fat show uniform, prolonged contrast en- unencapsulated, unicentric, solitary tumor
On sonography, small AMLs appear uni- hancement and a higher signal intensity index that frequently arises from the renal pyra-
formly hyperechoic without a hypoechoic rim on double-echo, chemical shift FLASH MRI mids or the pelvis [1, 37]. Hemangiomas
or intralesional cysts [32] (Fig. 4). Large [34, 35]. show variable echogenicity on sonography
AMLs appear as variegated masses with mac- and hyperintensity on T2-weighted MRI
roscopic fat, hemorrhage, and hypervascular Hemangioma [38] (Fig. 8A). Contrast-enhanced CT and
soft-tissue components. Intralesional aneu- Renal hemangioma is a rare benign mesen- MRI of renal hemangiomas may show
rysms are seen in large tumors as well. The chymal neoplasm that consists of multiple early, intense enhancement (Fig. 8B). Per-
presence of macroscopic fat on CT or MRI is endo thelium-lined, blood-filled vascular sistent contrast enhancement on delayed
characteristic of AMLs (Fig. 5). Loss of sig- spaces [1]. It commonly affects young adults images is fairly characteristic of renal he-
nal intensity on frequency-selective fat-sup- with no specific sex predilection. Recurrent mangiomas [37].
pressed MRI definitively identifies macro- episodes of hematuria and renal colic are typ-
scopic fat [30] (Fig. 6). However, a multitude ical presenting symptoms; however, inciden- Lymphangioma
of renal neoplasms, including RCC, oncocy- tal diagnosis in asymptomatic patients is also Lymphangioma of the kidney is a rare be-
toma, lipoma, and liposarcoma, may show ei- common [36]. Hemangiomas of the kidney nign cystic tumor that most often arises from
ther intratumoral fat or engulfed perirenal fat may be associated with systemic syndromes the peripelvic region or renal sinus [1]. It

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A B
Fig. 8—60-year-old man with hematuria and histologically proven hemangioma. Fig. 9—47-year-old man with bilateral multiple renal
A, Axial fat-saturated T2-weighted 2D gradient-refocused echo MR image shows hyperintense left kidney mass sinuses and perinephric lymphangiomatosis.
in renal sinus (arrow). Unenhanced axial CT scan shows multicentric cystic
B, Axial fat-saturated gadolinium-enhanced T1-weighted 3D gradient-refocused echo MR image shows contrast masses in renal sinus and perinephric spaces
enhancement of left renal sinus mass (arrows). (arrows).

Fig. 10—43-year-old woman with renal leiomyoma of Fig. 11—23-year-old woman with hypertension Fig. 12—57-year-old woman with incidental medullary
capsular origin. Axial contrast-enhanced CT scan refractory to standard treatment. Axial unenhanced CT fibroma (arrowhead). Patient underwent radical
shows large, fairly homogeneous exophytic mass scan shows large, expansile right renal mass (arrow) nephrectomy for renal cell carcinoma (Ca, arrow) of
(arrows) arising from left kidney (K). that was histologically proven to be juxtaglomerular right kidney.
cell neoplasm (reninoma). K = kidney, M = mass.

may also uncommonly arise from the lym- most common target site of leiomyomas; Juxtaglomerular Cell Neoplasm (Reninoma)
phatics of the capsule or the cortex [39]. His- rarely, leiomyomas originate from the renal Juxtaglomerular cell (JGC) neoplasm is an
tologically, lymphangiomas consist of com- pelvis or cortex. Intersecting fascicles of spin- extremely rare, benign renal neoplasm of
municating endothelium-lined spaces that dle cells that show immunoreactivity to actin myoendocrine cell origin [46]. The peak age
contain clear fluid [1]. The septa may show or desmin (smooth-muscle markers) are char- of incidence is in the second and third decades
lymphoid cells. acteristic histologic features [1]. and a 2:1 female preponderance is seen. JGC
Renal lymphangioma may occur either as Leiomyomas of the kidney commonly ap- neoplasm is clinically characterized by a triad
an isolated finding or in association with peri- pear as well-circumscribed, homogeneous, ex- of findings: poorly controlled hypertension,
nephric or systemic lymphangiomatosis [39]. ophytic solid masses that show uniform en- hypokalemia, and high plasma renin activity
It may appear as a localized process or a dif- hancement on contrast-enhanced CT [42] [47]. Histologically, JGC neoplasm consists
fusely cystic lesion. Lymphangioma typically (Fig. 10). Larger tumors are heterogeneous be- of sheets of polygonal or spindle cells and a
appears as a well-demarcated, uni- or mul- cause of hemorrhage and cystic or myxoid de- characteristic, complex, hemangiopericytic
tilocular cystic neoplasm that most com- generation [43, 44]. Calcification is uncom- angioarchitecture [1]. The presence of rhom-
monly arises from the renal sinus region or in mon. However, the CT findings of leiomyomas boid renin protogranules is diagnostic of JGC
the perinephric space [40, 41] (Fig. 9). of the kidney may be variable and may include neoplasm [46].
cystic, complex cystic–solid, or purely solid JGC neoplasm typically appears as a uni-
Leiomyoma morphology [44]. Renal leiomyomas may lateral, well-circumscribed, cortical tumor
Renal leiomyomas are rare benign smooth- show hypervascularity on catheter angiogra- that usually measures less than 3 cm [13]
muscle neoplasms that mostly occur in adults phy because they are predominantly supplied (Fig. 11). Despite profuse vascularity, JGC
as incidental findings [1]. Renal capsule is the by capsular vessels [42, 45]. neoplasms appear hypovascular on contrast-

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A B
Fig. 13—40-year-old woman with histologically proven Fig. 14—50-year-old woman with cystic nephroma.
mixed epithelial and stromal tumor of kidney. Axial A, Coronal contrast-enhanced CT scan shows lobulated, expansile, cystic mass (M) in left kidney (arrow) that
contrast-enhanced CT scan shows large complex compresses calyces (C).
cystic left kidney (K) mass (arrows) with septations and B, Coronal T2-weighted MR image shows multilocular, septated cystic mass in left kidney (arrow) that herniates
solid components. into renal pelvis. C = calyces.

enhanced CT and MRI, possibly because of in this article according to WHO taxonomic Large mixed epithelial and stromal tumors
renin-induced vasoconstriction [48, 49]. JGC schemata. may herniate into the renal pelvis. The tumors
neoplasms may show delayed contrast en- typically show benign biologic behavior
hancement. Imaging findings of JGC neo- Mixed Epithelial and Stromal Tumor without recurrence or metastasis; however,
plasms are nonspecific and indistinguishable The entity mixed epithelial and stromal tu- aggressive mixed epithelial and stromal tu-
from other solid renal neoplasms. mor was previously called by several descrip- mors with sarcomatous transformation of the
tive names reflecting variegated tumor histol- stromal component have been described [60].
Renomedullary Interstitial Cell Tumor ogy. It is now thought that mixed epithelial
Also referred to as medullary fibromas, and stromal tumor was previously referred to Cystic Nephroma
renomedullary interstitial cell tumors are be- as leiomyomatous renal hamartoma, mul- Cystic nephroma is a benign cystic neo-
nign neoplasms that arise from renomedul- tilocular cyst with ovarian stroma, cystic plasm that affects predominantly middle-aged,
lary interstitial cells, small stellate cells that hamartoma of the renal pelvis, and adult me- perimenopausal women [1]. Adult-onset cystic
are thought to play a role in blood pressure soblastic nephroma [53–55]. The unifying nephroma is histogenetically and morphologi-
homeostasis [50]. Renomedullary interstitial term, mixed epithelial and stromal tumor, was cally different from pediatric cystic nephroma
cell tumors are common incidental findings first coined by Michal and Syrucek in 1998 [39, 61]. Morphologically, cystic nephromas
that are present in 50% of adults in autopsy [56]; two recent large series have largely con- are composed of encapsulated, noncommuni-
series [1]. tributed to our understanding of mixed epithe- cating cysts with thin septations. By definition,
Most renomedullary interstitial cell tumors lial and stromal tumors [57, 58]. cystic nephromas are characterized by the ab-
are small and typically measure less than 5 Mixed epithelial and stromal tumors occur sence of a solid component or necrosis [1]. On
mm. The renal pyramid is the characteristic almost exclusively in perimenopausal women histology, the cysts are lined by a monolayer of
location of renomedullary interstitial cell tu- (6:1 female preponderance); most patients are hobnail epithelium; the fibrous septa may be
mors [1]. Rarely, large renomedullary inter- receiving estrogen therapy [57, 58]. Twenty- paucicellular or cellular [1].
stitial cell tumors extend into the renal pelvis. five percent of the tumors present as inciden- Cystic nephroma appears as a well-demar-
They appear as nonenhancing, hypoattenuat- tal findings; most patients manifest nonspe- cated, solitary, multilocular cystic lesion with
ing renal medullary solid lesions without cal- cific symptoms of flank pain and hematuria. thin septations (Fig. 14A). The cystic mass
cification (Fig. 12). Pathologically, mixed epithelial and stromal may protrude into the renal pelvis and cause
tumor is a benign, bimorphic solid–cystic hemorrhage or urinary obstruction [62]
Mixed Epithelial and Mesenchymal neoplasm that consists of epithelium-lined (Fig. 14B).
Neoplasms cysts or microcysts and variably cellular spin-
Mixed epithelial and mesenchymal neo- dle-cell, ovarianlike (estrogen- or progester- Conclusion
plasms comprise two histologically distinct one-receptor positive) stroma [57, 58]. Benign renal tumors that occur in adults
entities: mixed epithelial and stromal tu- On imaging, mixed epithelial and stromal cover a wide spectrum and show character-
mors and cystic nephromas. However, re- tumors typically appear as expansile, com- istic histology, histogenesis, and anatomic
cent studies have found remarkable demo- plex, cystic–solid masses with heterogeneous distribution. Some benign tumors of the kid-
graphic, clinical, and pathologic similarities and delayed enhancement [59] (Fig. 13). The ney (such as angiomyolipomas, mixed epi-
among these entities and a new name, renal proportion of cystic and solid constituents thelial and mesenchymal tumors, leiomyo-
epithelial and stromal tumor, has been pro- varies in any given case. The stromal compo- mas, and hemangiomas) show characteristic
posed [51, 52]. This nomenclature is still nent of the tumor is thought to be responsible imaging findings and regional distribution
new and has yet to be universally accepted; for the hypointense signal on T2-weighted that permit their diagnosis (Appendix 2). Al-
we will discuss these two entities separately MRI with delayed contrast enhancement [59]. though leiomyomas originate from the renal

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APPENDIX 1: World Health Organization (WHO) Histological Classification of Benign Renal Neoplasms

Renal Cell Tumors Metanephric Tumors Mesenchymal Tumors Mixed Epithelial and Mesenchymal Tumors
Oncocytoma Metanephric adenoma Angiomyolipoma Cystic nephroma
Papillary adenoma Metanephric adenofibroma Leiomyoma Mixed epithelial and stromal tumor
Metanephric stromal tumor Hemangioma
Lymphangioma
Reninoma
Fibroma
Schwannoma

APPENDIX 2: Making Sense of Adult, Benign Renal Neoplasms: A Pattern-Based Imaging Approach

Soft-Tissue Mass Fatty Mass Cystic Mass Cortical Mass Medullary Mass
Oncocytoma Angiomyolipoma (AML) Cystic nephroma Leiomyoma Hemangioma
Lipid-poor AML Mixed epithelial and stromal tumor Oncocytoma Fibroma
Leiomyoma Metanephric adenoma (rare) AML Mixed epithelial and stromal tumor
Hemangioma Lymphangioma AML
Reninoma Leiomyoma
Fibroma
Schwannoma

F O R YO U R I N F O R M AT I O N

This article is available for CME credit. See www.arrs.org for more information.

164 AJR:190, January 2008

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