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340 PART II Abdominal and Pelvic Sonography

manifestations are similar; early cortical calcification may be men and women equally.122 VonHippel–Lindau(VHL) disease
suggested by increased cortical echogenicity. With progressive is the best known inherited RCC syndrome; 24% to 45% of
calcification, a continuous, shadowing calcified rim develops. patients who have VHL disease will develop RCC. Most of
these lesions are multicentric and bilateral and are clear cell
carcinomas.124-126 Other inherited renal cancer syndromes
GENITOURINARY TUMORS include hereditarypapillaryrenalcancer,Birt-Hogg-Dubé syn-
drome,hereditaryleiomyomaRCC,familialrenaloncocytoma,
Renal Cell Carcinoma hereditarynonpolyposiscoloncancer,and medullaryRCC. An
Renal cell carcinoma (RCC) accounts for approximately 3% of increased incidence of RCC in patients with tuberoussclerosis
all adult malignancies and 86% of all primary malignant renal has also been reported.123 Another important but nonsyndromic
parenchymal tumors.120 There is a 2 : 1 male predominance, and risk factor for RCC is the acquiredcystickidneydiseasethat
peak age is 50 to 70 years. The cause is unknown, although occurs in patients receiving long-term hemodialysis or peritoneal
weak associations with smoking,121 chemical exposure, asbestosis, dialysis. The RCCs in these patients are small and hypovascular
obesity, and hypertension have been shown. The vast major- and tend to be relatively less aggressive.127,128
ity of RCCs are sporadic, but an estimated 4% occur in the Histologic subtypes of RCC include clear cell (70%-75%),
context of inherited syndromes.122,123 These “inherited” RCCs papillary(15%), chromophobe(5%), oncocytic(2%-3%), and
occur at an earlier age, are multifocal and bilateral, and affect collecting duct or medullary (<1%) tumors. Patients with
papillary, chromophobe, and oncocytic tumors have a much
better prognosis than those with clear cell and collecting duct
tumors. Attempts have been made to differentiate histologic
subtypes at imaging, largely based on enhanced-CT kinetics, but
overlapping patterns have precluded attempts at preoperative
imaging classification. However, potentially relevant features may
be shown at ultrasound; for example, lack of necrosis and the
presence of calcification appear to be associated with a better
prognosis (papillary and chromophobe subtypes).129
Before the advent of cross-sectional imaging, most patients
with RCC presented with advanced metastatic disease. In a 1971
series the classicdiagnostictriadof flank pain, gross hematuria,
and palpable renal mass was seen in 4% to 9% of patients at
presentation.130 Systemic symptoms (e.g., anorexia, weight loss)
are common with advanced disease. Manifestations reported
secondary to hormone production include erythrocytosis
(erythropoietin), hypercalcemia (parathormone, vitamin
D metabolites, prostaglandins), hypokalemia (adrenocor-
ticotropic hormone [ACTH]), galactorrhea (prolactin),
hypertension (renin), and gynecomastia (gonadotropin).
FIG. 9.44 Medullary Sponge Kidney. Sagittal sonogram shows RCC metastases to virtually every organ in the body have been
markedly increased renal medullary echogenicity (“medullary rings”). described.

A B
FIG. 9.45 Medullary Nephrocalcinosis in Two Patients. (A) Anderson-Carr-Randall kidney. Sagittal sonogram demonstrates increased echogenicity
in a rimlike pattern around all medullary pyramids and several punctate, shadowing calculi. (B) Sagittal sonogram shows extensive medullary calcification
in a patient with renal tubular acidosis.
CHAPTER 9 The Kidney and Urinary Tract 341

Imaging and Treatment Approaches shown to predict malignancy with 97% sensitivity, 45% specificity,
Before the advent of CT, renal tumors less than 3 cm represented and 90% accuracy. CEUS was superior to CT in the staging and
5% of lesions, whereas now these small lesions represent 9% to characterization of RCC, as well as in the subgroup of patients
38% of all renal tumors.131 Jamis-Dow et al.132 found that CT with cystic lesions.140
was more sensitive than ultrasound for the detection of small The increased detection of small, incidental lesions and better
renal masses (<1.5 cm) and that both ultrasound and CT could understanding of the natural history of these tumors have led
equally characterize a mass larger than 1 cm. They also demon- to less aggressive approaches to RCCs. The traditional surgical
strated that a combination of ultrasound and CT allowed accurate approach, radical nephrectomy, is now usually reserved for larger,
characterization of a lesion larger than 1.0 cm in 95% of cases. central lesions. The greater likelihood of small, benign, solid
With the advent of helical CT, respiratory misregistration and lesions in older patients141 and the limited metastatic potential
partial volume averaging are minimal; thus nephrographic-phase of small (<3 cm) lesions142 have prompted a “watchful waiting”
helical CT scans enable better lesion detection and characterization approach, particularly in older or ill patients.143 Nephron-sparing
and typically is sufficient for diagnosis.133-136 surgery (open/laparoscopic partial nephrectomy, laparoscopic
A particular role of MRI is in the characterization of high- cryoablation, percutaneous radiofrequency ablation/cryoablation)
attenuation renal masses.137 Most centers, however, still reserve may be offered to younger patients or older patients unwilling
renal MRI for patients with (1) an allergy to iodinated contrast, or unable to undergo imaging surveillance (Fig. 9.46). Primary/
(2) CT-indeterminate renal masses, or (3) extent of vascular secondary efficacy and long-term survival rates with these
involvement inadequately determined by ultrasound and CT. techniques are likely comparable to those resulting from tradi-
Previously, renal MRI was also performed to assess lesional tional nephrectomy.144 Gervais et al.145 found that radiofrequency
enhancement in patients with renal insufficiency. However, ablation of exophytic RCCs up to 5 cm in size can be performed
recognition of the central role of gadolinium in the development successfully. Tumors with a component in the renal sinus are
of nephrogenic systemic fibrosis highlighted the potential risks more difficult to treat. Retrospective studies have suggested that
in these patients.138 Thus ultrasound has again assumed a pre- cryoablation may be the preferred ablation modality in this
eminent role for mass characterization in patients with renal setting146 although a recent large meta-analysis has indicated
insufficiency at risk for nephropathy after iodinated contrast little difference in complication rates between techniques.147
exposure at CT or for nephrogenic systemic fibrosis after gado-
linium exposure at MRI. Sonographic Appearance
Contrast-enhanced ultrasound (CEUS) also holds promise Most RCCs are solid. Tumors may be hypoechoic, isoechoic, or
for assessment of renal lesions. Quaia et al. compared sonography hyperechoic (Fig. 9.47, Video 9.2). An early ultrasound series
without contrast material versus CEUS and CT for the charac- reported that the majority of RCCs are isoechoic (86%), whereas
terization of 40 complex cystic renal masses and found that the the minority is hypoechoic (10%) or echogenic (4%).148 Later
diagnostic accuracy of CEUS (80%-83%) was better than that series noted the ultrasound appearance of the smaller RCCs that
of nonconstrast sonography (30%) and CT (63%-75%) for all are now often depicted with cross-sectional imaging. These smaller
readers.139 In another European study of 143 lesions, CEUS was RCCs (<3 cm) are often echogenic compared with surrounding

A B
FIG. 9.46. Ultrasound-Guided Cryoablation of Renal Cell Carcinoma. (A) Transverse sonogram shows placement of a cryoablation probe
within the posterior aspect of a small (<3 cm) echogenic renal cell carcinoma. A prior ultrasound-guided biopsy had confirmed clear cell carcinoma.
(B) Corresponding noncontrast-enhanced CT shows the ablation zone as a low attenuation “ice-ball.” Ultrasound may facilitate renal biopsy and
probe placement, although the ablation is monitored under CT.
342 PART II Abdominal and Pelvic Sonography

A B C

D E F

G H I
FIG. 9.47 Sonographic Appearances of Renal Cell Carcinoma on Sagittal Sonograms. (A) Tiny incidental hypoechoic tumor. (B) Exophytic
echogenic upper-pole tumor with peripheral cystic spaces and larger uniform echogenic lower-pole tumor in same patient. (C) Small echogenic
nodule simulating an angiomyolipoma. (D) Exophytic echogenic midpole renal mass. (E) Exophytic hypoechoic upper-pole renal mass. (F) Large
central renal sinus mass with no associated caliectasis. (G) Large solid heterogeneous mass in the lower pole of the kidney compressing the renal
pelvis with upper-pole caliectasis. (H) Large infiltrative renal mass with maintenance of reniform shape. (I) Large upper-pole cystic mass showing
numerous thick internal septations. See also Video 9.2.

renal parenchyma; Forman et al.149 found that 77% of smaller been reported in RCCs with papillary, tubular, or microcystic
RCCs were echogenic, and Yamashita et al.150,151 reported 61% architecture and in tumors with minute calcification, necrosis,
as echogenic. cystic degeneration, or fibrosis.131 Macroscopic calcification
The small, echogenic RCC may be difficult to differentiate are identified in 8% to 18% of RCCs. This calcification may be
from a benign angiomyolipoma (AML) at ultrasound. Yamashita punctate, curvilinear, diffuse (rare), central, or peripheral.154-158
et al.151 emphasized the overlap in RCC/AML imaging appearance. Daniel et al.157 showed that central calcification was associated with
A thin, hypoechoic rim, thought to be a pseudocapsule at histol- a malignant tumor in 87% of cases. When posterior rim shadow-
ogy, was reported in 84% of RCCs and no AMLs. Weak shadowing ing or diffuse calcification make it impossible to characterize a
posterior to AMLs and hypoechoic halos or cystic spaces in renal lesion by ultrasound, CT is needed to identify additional
RCCs were also thought to be characteristic features.152 Nonethe- features of malignancy (e.g., enhancement of associated soft
less, although increased echogenicity of a solid renal lesion has tissue mass).159
high sensitivity for AML (99%), it is not specific. If a lesion is Papillary tumors account for 15% of all RCCs.129,160 The
of a size that would lead to a change in management, echogenic papillary type is characterized by slower growth, a lower stage at
renal lesions should be further assessed with either MRI or CT.153 presentation, and a better prognosis.161 Papillary tumors also tend
The exact pathologic basis for the hyperechoic appearance to be hypoechoic or isoechoic, although no consistent sonographic
of RCC is not understood, but increased echogenicity has pattern exists, because some may also be hyperechoic.160
CHAPTER 9 The Kidney and Urinary Tract 343

From 5% to 7% of all RCCs are cystictumors.162 Four histologic and unilocular subtypes are less aggressive.162 At sonography,
growth patterns within cystic RCCs have been described: multilocularcystic RCC appears as a cystic mass with internal
multilocular, unilocular, necrotic (cystic necrosis), and tumors septations. These septations may be thick (>2 mm), nodular,
originating in a simple cyst163 (Figs. 9.48 and 9.49). Recognition and may contain calcification (see Fig. 9.47). The characteristic
of subtypes have clinical significance because the multilocular ultrasound appearance of a unilocularcystic RCC is a debris-filled
mass with thick, irregular walls that may be calcified. The appear-
ance of necroticRCCs depends on the degree of tumor necrosis.
Tumors originating in a simplecyst are rare (excluding VHL
patients). At ultrasound, a mural tumor nodule will be found at
the base of a simple cyst. A combined approach with helical CT
and ultrasound allows accurate characterization of the internal
nature of most cystic renal lesions.164
The use of Doppler ultrasound165 for detection of tumor
vascularity has been reported. Most malignant renal tumors
(70%-83%) have Doppler shift frequency of 2.5 kHz.165-169 Similar
changes may be noted with inflammatory masses; however,
patients with renal infection should be clinically apparent.
Unfortunately, the absence of high-frequency Doppler shift does
not exclude malignancy.168 Confirmation of blood flow within
malignant solid and cystic renal tumors has also been performed
with microbubble contrast agents and low–mechanical index
pulse inversion sonography. Criteria for neovascularity used with
CT or MRI for mass characterization—septal and nodular
enhancement—can also be used with stable, second-generation
ultrasound contrast agents170,171 (Fig. 9.50). However, lack of US
Food and Drug Administration approval, reimbursement issues,
and logistics associated with technologist/radiologist-intensive,
contrast-enhanced sonography protocols have hindered wide-
spread adoption of this technique.

Biopsy and Prognosis


Recent pathologic and biopsy series of small (<3 cm), solid
enhancing renal masses have shown that a surprising number
of these lesions are benign (i.e., AML,oncocytoma,metanephric
adenoma). Moreover, image-directed needle biopsy (aspiration
FIG. 9.48 Cystic Growth Patterns of Renal Cell Carcinoma. Upper or core) has been shown to be a relatively safe and usually
pole, Multilocular; upper lateral, unilocular; lower lateral, cystic necrosis;
lower pole, origin in the wall of a simple cyst. (With permission from
conclusive procedure.172-176 Further advances in immunohistology
Yamashita Y, Watanabe O, Miyazaki H, et al. Cystic renal cell carcinoma. and greater acceptance of percutaneous ablation techniques
Acta Radiol. 1994;35:19-24.162)

A B
FIG. 9.49 Renal Cell Carcinoma Within Cyst. (A) Complex cyst with mural nodule. (B) Color Doppler shows flow within septation. (Courtesy
of Vikram Dogra, MD.)
344 PART II Abdominal and Pelvic Sonography

A B

C D
FIG. 9.50 Value of Color Doppler and Microbubble-Enhanced Sonography for Determining Vascularity of a Renal Mass. (A) Sagittal
sonogram shows an isoechoic lower-pole mass and an adjacent cyst; the simultaneous power Doppler image shows flow within the mass (biopsy
confirmed renal cell carcinoma). (B) Sonogram of a patient with renal failure shows a large complex cyst containing low-level echoes. The simultaneous
microbubble enhanced image shows no enhancement of the center of the mass, but it confirms enhancement of the nodular, thick wall of a cystic
renal cell carcinoma. (C) Baseline transverse sonogram of exophytic hypoechoic midpole renal mass. (D) Nephrographic phase image shows
enhancement of both normal kidney and relatively hypovascular renal cell carcinoma (arrow). (C and D courtesy of Ed Grant, MD.)

continue to increase the role of imaging-directed, particularly has led to clinical trials of novel, small-molecule-targeted inhibi-
ultrasound-guided, renal mass biopsy177 (Fig. 9.51). tors and monoclonal antibodies.180
For patients with imaging findings (or biopsy results) definitive
for RCC, the stageatdiagnosisdirectly impacts prognosis. The Pitfalls in Interpretation
Robson staging classification for RCC is the following: Ultrasound is inferior to CT and MRI for staging RCC. Unfor-
I: Tumor confined within renal capsule tunately, obesity and overlying bowel gas often make it difficult
II: Tumor invasion of perinephric fat to assess for lymphadenopathy or vascular involvement. In
III: Tumor involvement of regional lymph nodes or venous thin patients and in those with minimal bowel gas, however,
structures the renal veins and retroperitoneum can be well assessed with
IV: Invasion of adjacent organs or distant metastases ultrasound. Sonography is excellent for assessment of the
Five-year survival rates for patients with Robson stages I, II, intrahepatic IVC and for determination of the cephalad extent
III, and IV are 67%, 51%, 33.5%, and 13.5%, respectively.178 of venous tumor thrombus with RCC (Fig. 9.52). Habboub
Patients with stage I and stage II disease are treated surgically et al.181 found the accuracy of detecting renal vein and IVC
(partial or radical nephrectomy). Patients with stage III disease, involvement at sonography was 64% and 93%, respectively.
with extensive metastatic lymphadenopathy, are often treated The addition of color Doppler sonography improved accuracy
palliatively. Patients with stage III disease and tumor thrombus for diagnosing both renal vein and IVC thrombus to 87%
are treated with radical nephrectomy and thrombectomy. Patients and 100%, respectively. It is crucial to determine the location
with stage IV disease usually receive palliative treatment only,179 and extent of vascular tumor thrombus to plan the surgical
although greater understanding of the molecular biology of RCC approach.
CHAPTER 9 The Kidney and Urinary Tract 345

A B
FIG. 9.51 Renal Oncocytoma. (A) Sagittal sonogram shows a large, isoechoic, partially exophytic renal mass that cannot be differentiated
from renal cell carcinoma. (B) Ultrasound-guided biopsy of an isoechoic renal lesion (arrowheads) in another patient, performed before possible
cryoablation, confirms an oncocytoma.

A B

C D E
FIG. 9.52 Venous Thrombosis With Renal Cell Carcinoma. (A) Sagittal sonogram shows a huge infiltrating right upper-pole renal cell carcinoma.
(B) Transverse ultrasound and simultaneous color Doppler image shows nonocclusive but expansile malignant thrombus within right renal vein and
inferior vena cava (IVC). (C) Sagittal ultrasound of the same patient shows echogenic, nonocclusive thrombus within suprarenal IVC. Clot extends
to 1 cm below the hepatic venous confluence. (D) Sagittal sonogram in another patient shows expansile, malignant thrombus within retrohepatic
IVC. (E) Transverse sonogram in the same patient in D showing partially adherent malignant thrombus in the IVC.

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