renal neoplasm

Clinical UrographyClinical UrographyNeoplastic DiseaseNeoplastic Disease

Clinical UrographyClinical UrographyNeoplastic DiseaseNeoplastic Disease

MARIA THERESA M. NAVARRO, MD.MARIA THERESA M. NAVARRO, MD.Fourth Year Radiology ResidentFourth Year Radiology ResidentDepartment of Medical ImagingDepartment of Medical Imaging

Quirino Memorial Medical CenterQuirino Memorial Medical Center

Source: Pollack, Howard. Clinical Urography 2nd. ed, Volume 2.

BENIGN NEOPLASMS OF THE RENAL PARENCHYMA

BENIGN NEOPLASMS OF THE RENAL PARENCHYMA

Benign Neoplasms of the Renal Parenchyma

ADENOMAADENOMA• Pathology

– tubulopapillary adenomas, metanephric adenomas, or oncocytomas

• Clinical Presentation– symptoms related to size– generally asymptomatic – discovered incidentally at the time of surgery, autopsy

or radiologic examination


ADENOMAADENOMA• Imaging

– when adenoma is of sufficient size or calcified, maybe detected as a mass on plain abdominal radiograph

– when solid, tubopapillary adenomas tend to appear as well-defined, homogenous masses on CT or ultrasound

– angiography --> demonstrates a sharply marginated mass that maybe vascular or hypervascular

– difficult to differentiate from other solid renal tumors

Calcified Renal Adenoma

Renal Adenoma

A. The right renal mass is inhomogenous in density and enhances less than the normal right renal parenchyma.

B. Renal arteriogram shows that the mass is well-defined and entirely intrarenal within the upper pole of the right kidney. Although the mass is vascular, its vessels lack obvious malignant features. The mass is indistinguishable from renal cell carcinoma.


ADENOMAADENOMA• Treatment

– because renal adenoma cannot currently be distinguished from renal cell carcinoma short of pathological study, these lesions must be treated as renal cell carcinoma until proven otherwise

– close radiologic follow-up (<2.5 cm)– surgery (larger tumors)


ONCOCYTOMA ONCOCYTOMA (PROXIMAL TUBULAR ADENOMA WITH ONCOCYTIC FEATURES)

• Pathology– oncocytes are relatively large epithelial cells with

granular eosinophilic cytoplasm– vary in size from lesions smaller than 1 cm in diameter

to huge masses– benign or malignant– neoplasms with oncocytic features that exhibit

anaplasia are considered to be renal cell carcinomas


ONCOCYTOMA ONCOCYTOMA (PROXIMAL TUBULAR ADENOMA WITH ONCOCYTIC FEATURES)

• Clinical Presentation– 1.7:1 male to female ratio – peak incidence in the 6th and 7th decade– rarely encountered in pediatric patients– although flank pains and gross hematuria are

occasionally present, majority are asymtomatic– hypertension– palpable flank mass


ONCOCYTOMA (PROXIMAL TUBULAR ADENOMA WITH ONCOCYTIC FEATURES)

• Imaging– no features have been described in plain film

examination or excretory urography– ultrasound and CT well-defined, smooth, relatively

homogenous• central stellate scar particularly in large tumors• although suggestive, features can be exhibited by renal cell

carcinoma• pathologic confirmation is necessary


ONCOCYTOMA (PROXIMAL TUBULAR ADENOMA WITH ONCOCYTIC FEATURES)

• Imaging– MRI homogenous signal intensity that is low to

moderate on T1-weighted images and relatively high on T2-weighted images

– Angiography well-defined tumor, often having “spoke-wheel” pattern of vascularity

• vascular pattern is orderly, unlike renal cell carcinoma, is without venous shunting and vascular puddling

• not pathognomonic

Oncocytoma

ONCOCYTOMA

Coronal contrast-enhanced nephrographic phase (b) and delayed excretory phase (c) MR images show the mass with heterogeneous enhancement. The central cleft (arrow) is enhanced on the delayed image, thereby excluding necrosis. There are no features to reliably help distinguish oncocytoma from RCC. Oncocytoma was confirmed at histopathologic analysis performed after left nephrectomy.

Oncocytoma

SELECTIVE RIGHT RENAL ARTERIOGRAM

•hypervascular mass

•Three features suggestive but not pathognomonic

•peripheral rim vessel

•radiating pattern of branch vessels emanating from the peripheral rim

relatively hypovascular center

•Renal carcinoma uncommonly demonstrate this constellation of features


ANGIOMYOLIPOMA (HAMARTOMA)ANGIOMYOLIPOMA (HAMARTOMA)• contain vascular, smooth muscle, and fatty

elements• commonly referred to as hamartomas ( benign

tumor consisting of tissues that normally occur in the organ of origin )

• choristoma ( benign tumor composed of tissues not normally occuring within the organ of origin)

• fat and muscle elements do not normally occur within the parenchyma of the kidney

• 20% have tuberous sclerosis


ANGIOMYOLIPOMA (HAMARTOMA)ANGIOMYOLIPOMA (HAMARTOMA)

• Clinical Presentation– can occur in patients with or without concomitant

tuberous sclerosis– angiomyolipomas in patients without tuberous

sclerosis are oftened discovered because of clinical symptom

• most are found incidentally during CT or ultrasound examination for unrelated condition

– 90% of angiomyolipomas unassociated with tuberous sclerosis are unilateral

• majority occur in women beyond 40 years


ANGIOMYOLIPOMA (HAMARTOMA)ANGIOMYOLIPOMA (HAMARTOMA)

• Clinical Presentation– 80% of patients with tuberous sclerosis have renal

angiomyolipomas• tend to be multiple and bilateral• no sex predilection

– most are asymptomatic– flank pain is the most frequent symptom of bleeding– hemorrhage (tumors >3.5 to 4 cm size)– hypertension (initial clinical manifestation)– chronic renal failure (with tuberous sclerosis, bilateral

angiomyolipomas)

Tuberous Sclerosis

Multiple bilateral renal masses distort the collecting system

Angiomyolipoma

Intensely echogenic appearance of the mass in the anterior aspect of the right kidney, which is typical of an angiomyolipoma containing a large amount of fat.

ANGIOMYOLIPOMA

38-year-old woman with documented tuberous sclerosis complex and renal angiomyolipomas. Axial in-phase T1-weighted 2D gradient-refocused echo MR image shows bilateral multicentric renal masses that have increased signal intensity (arrows).

ANGIOMYOLIPOMA

38-year-old woman with documented tuberous sclerosis complex and renal angiomyolipomas. Axial fat-saturated T2-weighted 2D gradient-refocused echo MR image shows marked drop in signal intensity of masses (arrows).


MULTILOCULAR CYSTIC RENAL TUMORMULTILOCULAR CYSTIC RENAL TUMOR

(Multilocular Cystic Nephroma)(Multilocular Cystic Nephroma)

Pathologyunilateral and solitarygross inspection, thick well-defined fibrous capsulenumerous “honeycombed” cystic areaslocules do not communicate with each other or with the renal pelvis separated by fibrous septa



Clinical Presentationequally prevalent among males and females90% of the lesions in males occur in the 1st two years of lifelesions in females – equally divided between patients younger than 5 years and between 40 and 60 years.



Clinical Presentation

childhood:nonpainful abdominal mass, although hematuria or urinary tract symptoms may lead to discovery

adults:abdominal pain, hematuria and UTI



ImagingUltrasound or CT

numerous distinct cystic areas located within well-defined encapsulated massseptations maybe as much as several millimeters in thickness but should be regular than nodular.moderate contrast enhancement of the septa on CT scans is the ruleDDX: cystic renal carcinoma or Wilm’s tumor

Multilocular Cystic Renal Tumor

Multilocular Cystic Renal Tumor

Enhanced CT scan shows a loculated, partially solid and partially cystic mass arising from the anterior aspect of the right kidney. Fibrous septa often enhance with bolus

injection of contrast medium.



ImagingMagnetic Resonance Imaging

like CT and ultrasound, may depict multiloculated nature of the lesions

Angiographyavascular, hypovascular, or hypervasculardoes not allow lesion to be distinguished from partially necrotic or hypovascular renal cell carcinoma


JUXTAGLOMERULAR CELL TUMORJUXTAGLOMERULAR CELL TUMOR(Reninoma)

resemble hemangiopericytomasarise in juxtaglomerular cells50% of patients are younger than 20 yearsslight female preponderanceplasma renin activity is elevated hypertensionpatients exhibit secondary hyperaldosteronism with hypokalemia.

23-year-old woman with hypertension refractory to standard treatment. Axial unenhanced CT scan shows large, expansile right renal mass (arrow) that was histologically proven to be juxtaglomerular cell neoplasm (reninoma)

Reninoma

JUXTAGLOMERULAR CELL TUMORJUXTAGLOMERULAR CELL TUMOR(Reninoma)

Renin can be produced by other causes:Wilm’s tumorRenal cell carcinomaHypernephroma

Imagingsmall size of these tumors makes them difficult to detectexcretory urography and other imaging studies are normalUltrasound shows a solid tumor, frequently with an echogenic patternCT and MRI also demonstrate a solid tumor


derived from medullary interstitial cellsFIBROMAS – most common benign tumor of the renal medulla

RENOMEDULLARY INTERSTITIAL CELL RENOMEDULLARY INTERSTITIAL CELL TUMORTUMOR (Fibroma)

57-year-old woman with incidental medullary fibroma (arrowhead). Patient underwent radical nephrectomy for renal cell carcinoma (Ca, arrow) of right kidney.


LEIOMYOMA of the KIDNEYLEIOMYOMA of the KIDNEY

can arise from smooth muscle in the renal capsule, cortical vessels, or pelvicalyceal systemmost common finding is a palpable abdominal or flank mass with or without painmajority of cases present in childhood2/3 cases found in women¾ develop in the lower pole of the kidneyUltrasound and CT

sharply marginated solid mass Angiography

hypovascular or hypervascular

Leiomyoma of the Kidney

A. CT scan prior to contrast administration reveals and elliptical hyperdense mass involving the dorsal aspect of the left kidney

B. Following contrast administration, there is central enhancement in the lesion, which appears to originate peripherally, and its elongated, eccentric appearance is consistent with a lesion of capsular origin.

Renal cell carcinoma could also present in this fashion

ADULT MALIGNANT RENAL PARENCHYMAL

NEOPLASMS

ADULT MALIGNANT RENAL PARENCHYMAL

NEOPLASMS

Adult Malignant Neoplasms of the Renal Parenchyma

RENAL CELL CARCINOMAClear cell type

Papillary type

Granular cell typeChromophobe cell typeSarcomatoid typeCollecting duct type

CARCINOID TUMORSMALL CELL CARCINOMALYMPHOID AND HEMATOPOEITIC TUMORS

Lymphoma and leukemia

Plasmacytoma


MESENCHYMAL TUMORSLeiomyosarcoma

LiposarcomaHemangiopericytoma

Malignant fibrous histiocytoma and fibrosarcomaRhabdomyosarcoma

AngiosarcomaOsteosarcoma

NEPHROBLASTOMA (WILM’S TUMOR)

METASTATIC CARCINOMA, SARCOMA AND MELANOMARENAL INVASION BY ADJACENT NEOPLASMS

Renal medullary carcinomaInvasive renal pelvic carcinomaRetroperitoneal sarcomaVisceral carcinomas


RENAL CELL CARCINOMARENAL CELL CARCINOMA

most common adult renal cancermost common adult renal cancer

2% of adult malignanciesmale-to-female annual incidence ratio – 1.5:1usually present in the ages 57 to 70 years (median age at diagnosis of 57 years)


Pathology

arise from tubular epithelial cellsmay originate from any segment of the nephron or from the collecting tubules or ductsclear cell type of RCC

most common70% of cases


Patterns of Spread

predominantly expansile growth patternsuperficial tumors ultimately reach the renal capsuletumors deep in the kidney often extend toward the renal sinus and pelvis and may invade themperinephric fat and may involve ipsilateral adrenal gland


Patterns of Spread

direct invasion of the adjacent viscera, including the colon, duodenum, pancreas, liver, spleen, may also occur.metastases often develop in the regional lymph nodes (para-aortic and paracaval lymph nodes above and below the renal vessels)renal venous (23%) and IVC (7%) tumor extension vena caval involvement-vena caval involvement- 3x more common with right-sided tumors (owing to the shorter length of the right-renal vein)


Patterns of Spread

most thrombi involve only the infrahepatic vena cavaLUNGS – most frequent site for metastases in RCCvenous tumor emboli usually reach the right side of the heart via IVC

Small Renal Cell Carcinoma

Selective left renal arteriogram reveals an exophytic neoplasm in the upper pole of the kidney. Note the peripheral lucent zone (arrowheads) representing the

tumor pseudocapsule.

Renal Cell Carcinoma

Small renal cell carcinoma found incidentally in a 65 year old man.

A. Excretory urography with tomography shows a small exophytic cortical mass

B. Longitudinal sonogram exhibits a solid mass slightly more echogenic than the normal renal parenchyma (Stage I papillary renal carcinoma)

A. Excretory urography exhibits enlargement of the upper pole of the right kidney with displacement of the collecting system.

B. Selective renal arteriography shows a hypovascular tumor located medially



The tumor displays a homogenous blush in the nephrographic phase.

A lucent zone or pseudocapsule demarcates the mass from normal renal parenchyma.

Histologically, the pseudocapsule consisted of fibrous tissue and compressed renal parenchyma.


Contrast-enhanced CT of the neoplasm invades the collecting system.

Staging of Renal Cell Carcinoma (Robson Classification)

STAGE I – tumor is confined to renal capsule

STAGE II – tumor extends through the renal capsule into the perinephric fat but is confined within the renal fascia. Involvement of the ipsilateral adrenal gland is possible in this stage

STAGE III A – Tumor involves the renal vein and/or inferior vena cava

STAGE III B – Tumor involves regional lymph nodes

STAGE III C – Tumor involves both renal venous structures and regional lymph nodes

STAGE IV A – Tumor extends through renal fascia to involve adjacent organs (other than the ipsilateral adrenal)

STAGE IV B – Tumor has metastasized to distant sites

Stage III A Renal Cell Carcinoma

Radiological Evaluation of Renal Cell CarcinomaRadiological Evaluation of Renal Cell Carcinoma

RENAL MASS

SOLID FLUID-FILLED

PSEUDOTUMORS

WIDE VARIETY OF RENAL TUMORS


Oncocytoma

Angiomyolipoma

Metastasis

Lymphoma


EXCRETORY UROGRAPHY1. Calcification

always raises the suspicion of malignancycentral calcfication strongly suggests malignancy (87%)peripheral calcification 20% are malignant

2. Renal Contour Deformities and Alteration in the Renal Axisrenal cell carcinoma often causes a focal bulge in the renal contour or enlargement of the affected kidneytilting of the renal axis may occur if the mass grown in an exophytic manner, especially medially


A. Excretory urography with tomography displays a low-density mass causing a focal bulge in the lower pole of the right kidney. It causes displacement and stretching of the adjacent calyces.

B. Contrast-enchanced CT scan clearly indicates the solid but heterogenous nature of the tumor. The perinephric fat and renal fascia are normal, suggesting tumor confinement within the renal capsule. (Stage I renal cell carcinoma)


EXCRETORY UROGRAPHY3. Abnormalities of the Collecting System

distortion of the renal outline maybe absent with centrally located masscan be diagnoses urographically from infundibular, calyceal, or pelvic displacement or obliteration.unlike cysts, which cause displacement of the renal collecting system, renal cell carcinomas invade the calyces, or renal pelvis, causing smooth or irregular filling defects.obstruct the collecting system, leading to localized hydrocalyces or even generalized unilateral hydronephrosispelvic and ureteral notching may occur owing to periureteric and peripelvic venous collateral veins forming as a result of renal vein obstruction caused by tumor extension.

Renal cell carcinoma invading the collecting system.

A. Excretory urography shows a filling defect in the infundibula of the mid region and lower pole calyces. B. Tomography shows a lucent region in the nephrogram in the midregion and lower pole.



EXCRETORY UROGRAPHY4. Abnormal Blood Vessels

renal cell carcinomas are usually hypervascularabnormal vessels may be seen both in and around the tumor

5. Defects in the Nephrogramsome tumors are similar in density to renal parenchymaothers less dense than normal renal tissuetumors with extensive central necrosis, the perfused part of the lesion is sometimes seen as a radiodense thickwall with irregular inner margin surrounding the radiolucent necrotic center.


EXCRETORY UROGRAPHY6. Absence of Excretion by Affected Kidney

absence of contrast excretion by a kidney containing a carcinoma usually indicates renal vein occlusion owing to venous tumor extensionmay also be due extensive renal infiltration or ureteropelvic junction obstruction by tumorpyelotumor backflow on RGP, contrast may dissect between the lesion and surrounding renal parenchyma

7. Metastatic Diseaselung bases seen on abdominal radiographs may show metastases or malignant pleural effusionlumbar spine and pelvic bones should be examined (common sites)

CT Evaluation of Renal Cell CarcinomaCT Evaluation of Renal Cell Carcinoma

APPEARANCES ON UNENHANCED SCANS

unenhanced scans help determine whether a renal mass enhancesfacilitate distinction between hemorrhagic cysts and carcinomasmost tumors have similar attenuation values similar to renal parenchyma (30 to 50 HU)inhomogenous appearance denoting changes in internal compositionrecent tumor hemorrhage (high density areas)small renal tumors without hemorrhage, necrosis, or alterations of renal contours maybe missed


TUMOR CALCIFICATION

• nonperipheral punctate or curvilinear calcification strongly suggests renal cell carcinoma

• cystic renal cell carcinomas may show peripheral curvilinear calcification

• hemorrhagic simple renal cysts may also show both peripheral calcification and high attenuation values

• CT is of little help in making definitive distinction between calcified tumor and cyst

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Right renal cell carcinoma with curvilinear, nonperipheral calcification

A. A non-enhanced CT scan shows a curvilinear calcification in a large renal mass.

B. A contrast-enhanced CT scan exhibits a mass (arrows) arising posteriorly and extending beyond the limits of the tumor calcification.


CONTRAST-ENHANCED SCANS• renal cell carcinomas usually show enhancement following

intravenous administration of contrast• but the increase in attenuation value is always less than that

of surrounding normal parenchyma• offers the best opportunity to visualize the wall of a cystic

renal cell carcinoma, which is invariably thick although not necessarily irregular

• important point in differentiation from uncomplicated benign renal cysts, which walls of barely perceptible thickness


CONTRAST-ENHANCED SCANS• renal cell carcinomas sometimes bleed spontaneously,

resulting in subcapsular and/or perinephric hemorrhage• sometimes the first clinical manifestation of tumor• CT achieves an accuracy greater than 95% in the diagnosis of

renal cell carcinoma

• CT is an accurate technique for abdominal staging of renal cell carcinoma


CYSTIC RENAL CELL CARCINOMACYSTIC RENAL CELL CARCINOMA

most renal cell carcinomas contain small cystic areas, however, sometimes the cystic component predominates and such lesions are called cystic renal cell carcinomas.cystic renal cell carcinomas.

1.1. UnilocularUnilocularmost common most common radiological pattern (50% of cases)radiological pattern (50% of cases)



2. Multilocular 2. Multilocular • 30% of cases30% of cases• dystrophic calcification in the tumor capsule or in the dystrophic calcification in the tumor capsule or in the

septa between the locules may be seen on plain filmssepta between the locules may be seen on plain films• Sonography and CT – best techniqueSonography and CT – best technique• Radiologic findings – indistinguishable from multilocular Radiologic findings – indistinguishable from multilocular

cystic nephromacystic nephroma



3.3. Discrete Mural Tumor Nodule in a Cystic MassDiscrete Mural Tumor Nodule in a Cystic Massleast common (fewer than 20% of cases)least common (fewer than 20% of cases)excretory urogram shows nonspecific renal massexcretory urogram shows nonspecific renal masstumor nodule is often located at the base of the cyst, and tumor nodule is often located at the base of the cyst, and the cyst fluid provides an excellent acoustic window for the cyst fluid provides an excellent acoustic window for showing the tumor nodule on sonographyshowing the tumor nodule on sonography

Cystic Renal Cell Carcinoma

(A) Precontrast image demonstrates a homogeneous low-attenuation mass in the left kidney. (B) Postcontrast image demonstrates a small peripheral nodule of enhancing tumour in the wall of the cyst (Bosniak class IV).


PAPILLARY RENAL CELL CARCINOMAPAPILLARY RENAL CELL CARCINOMA• 5% to 15% of renal cell carcinomas5% to 15% of renal cell carcinomas• at least 50% of the tumor is composed of cells arranged at least 50% of the tumor is composed of cells arranged

around fronds of fibrovascular stroma that project into around fronds of fibrovascular stroma that project into cystic tumor spacescystic tumor spaces

• radiographically, show peripheral calcification on radiographically, show peripheral calcification on excretory urography and CTexcretory urography and CT

• usually hypovascular angiographicallyusually hypovascular angiographically• CT shows a low tumor stageCT shows a low tumor stage• no consistent sonographic patternno consistent sonographic pattern


MALIGNANT LYMPHOMAMALIGNANT LYMPHOMA

• primary lymphoma arising in the kidney is very rareprimary lymphoma arising in the kidney is very rare• in Non-Hodgkin’s lymphoma (10x) > Hodgkin’s diseasein Non-Hodgkin’s lymphoma (10x) > Hodgkin’s disease• Large cell lymphoma – Large cell lymphoma – most common type most common type of Non-Hodgkin’s of Non-Hodgkin’s

lymphoma (60% of all cases) when there is lymphomatous lymphoma (60% of all cases) when there is lymphomatous involvement of then kidneyinvolvement of then kidney

• immunocompromised patientsimmunocompromised patients• bilateral renal masses – bilateral renal masses – most common present most common present (40% to 60% of all (40% to 60% of all

cases)cases)• does not commonly produce urinary tract symptomsdoes not commonly produce urinary tract symptoms• occurs late in the courseoccurs late in the course


MALIGNANT LYMPHOMAMALIGNANT LYMPHOMA

• Excretory UrographyExcretory Urography – insensitive technique – insensitive technique• Sonography – Sonography – markedly hypoechoic relative to renal parenchyma markedly hypoechoic relative to renal parenchyma

(sometimes cyst-like), however, shows little posterior acoustic (sometimes cyst-like), however, shows little posterior acoustic enhancementenhancement

• Computed tomographyComputed tomography accurately depicts the presence, location, accurately depicts the presence, location, and size of renal lesionsand size of renal lesions

• multiple nodules – most common CT manifestation multiple nodules – most common CT manifestation • CT also important in evaluating the course of the renal disease and CT also important in evaluating the course of the renal disease and

its response to therapyits response to therapy

Non-Hodgkin’s Lymphoma

(A) Unenhanced CT demonstrates homogeneous enlargement of both kidneys. (B) Postcontrast examination demonstrates multiple focal areas of reduced enhancement within the kidneys, consistent with lymphoma. Hepatic and pancreatic deposits of lymphoma can also be seen.


SARCOMATOID RENAL CELL CARCINOMASARCOMATOID RENAL CELL CARCINOMAtumors that arise from renal tubular epithelium but tumors that arise from renal tubular epithelium but show pleiomorphic metaplastic transformation of show pleiomorphic metaplastic transformation of carcinoma cells so that they resemble sarcomas carcinoma cells so that they resemble sarcomas histologicallyhistologicallyhighly aggressive tumors highly aggressive tumors frequently invade the renal pelvis, abdominal wall, frequently invade the renal pelvis, abdominal wall, renal vein, regional lymph nodesrenal vein, regional lymph nodes


• RENAL SARCOMARENAL SARCOMA

1 % of malignant renal parenchymal tumors1 % of malignant renal parenchymal tumorsrare malignant tumors that arise from the nonepithelial rare malignant tumors that arise from the nonepithelial mesodermal tissues of the kidneymesodermal tissues of the kidneymost common types are: leiomyosarcoma, most common types are: leiomyosarcoma, hemangiopericytoma, liposarcoma, hemangiopericytoma, liposarcoma, rhabdomyosarcoma, fibrosarcoma, osteogenic rhabdomyosarcoma, fibrosarcoma, osteogenic sarcomasarcoma


RENAL SARCOMARENAL SARCOMA

28 to 78 years of age28 to 78 years of agesize of renal sarcomas – 5.5 cm to 23 cmsize of renal sarcomas – 5.5 cm to 23 cmprognosis very poorprognosis very poorindistinguishable from RCCsindistinguishable from RCCs

Renal Sarcoma

LEIOMYOSARCOMALEIOMYOSARCOMA

58% of all renal sarcomas 58% of all renal sarcomas most common most common sarcoma of the kidneysarcoma of the kidney44thth decade of life decade of lifetwice as common in women as in mentwice as common in women as in menindistinguishable from renal cell carcinoma radiographicallyindistinguishable from renal cell carcinoma radiographically

Renal Sarcoma

HEMANGIOPERICYTOMAHEMANGIOPERICYTOMA

rare renal tumors, 20% of renal sarcomasrare renal tumors, 20% of renal sarcomas18 to 49 years, women18 to 49 years, womenusually arise from the renal capsule and do not invade the usually arise from the renal capsule and do not invade the renal parenchymarenal parenchymahowever, extensive parenchymal invasion may occurhowever, extensive parenchymal invasion may occurpotentially malignantpotentially malignantradiographically, radiographically, present as renal masses with displacement present as renal masses with displacement and distortion of the renal collecting systemand distortion of the renal collecting system

Renal Sarcoma

LIPOSARCOMALIPOSARCOMA

rare renal tumors, 20% of renal sarcomasrare renal tumors, 20% of renal sarcomasusually arise in the renal capsule, probably from usually arise in the renal capsule, probably from undifferentiated mesenchymal cellsundifferentiated mesenchymal cellsor in the perinephric fat, causing compression but not or in the perinephric fat, causing compression but not invasion of the renal parenchymainvasion of the renal parenchyma

Renal Sarcoma

RHABDOMYOSARCOMARHABDOMYOSARCOMA

3.8 % of renal sarcomas in adults3.8 % of renal sarcomas in adultsprobably arises from undifferentiated mesenchymal cellsprobably arises from undifferentiated mesenchymal cellshighly aggressive tumors and rapidly extend to the renal highly aggressive tumors and rapidly extend to the renal veins, inferior vena cava, and right atrium.veins, inferior vena cava, and right atrium.


WILM’S TUMOR OCCURING IN ADULTSWILM’S TUMOR OCCURING IN ADULTS

WILM’S TUMOR or NEPHROBLASTOMA, is the WILM’S TUMOR or NEPHROBLASTOMA, is the most most common renal neoplasm of childhoodcommon renal neoplasm of childhoodpeak incidence during 2peak incidence during 2ndnd year of life, 50% of cases year of life, 50% of cases undiagnosed before the 3undiagnosed before the 3rdrd birthday birthday75% before the age of 5 years75% before the age of 5 yearsmay also occur in adolescents and adultsmay also occur in adolescents and adultsarise in the renal parenchyma and cause marked renal arise in the renal parenchyma and cause marked renal destructiondestruction



IVU IVU focal renal masses focal renal masses nonfunction of the affected kidney due to renal vein nonfunction of the affected kidney due to renal vein occlusionocclusion ureteral obstructionureteral obstructionextensive parenchymal tumor replacementextensive parenchymal tumor replacementtumor calcification maybe distinguishing featuretumor calcification maybe distinguishing feature



CT CT inhomogenous mass with large areas of low density inhomogenous mass with large areas of low density due to tumor hemorrhage or necrosisdue to tumor hemorrhage or necrosisdemonstrate perinephric extension or venous tumor demonstrate perinephric extension or venous tumor thrombusthrombus

Wilm’s Tumor in a 46-year old mas



MRIMRIheterogenous signal intensity in the large renal heterogenous signal intensity in the large renal mass with associated necrosis and hemorrhagemass with associated necrosis and hemorrhage

ANGIOGRAPHYANGIOGRAPHYsmall areas of neovascularity in a relatively small areas of neovascularity in a relatively hypovascular masshypovascular mass



PROGNOSISPROGNOSISpoorer response to the combination of poorer response to the combination of chemotherapy, surgery, and radiation therapy than chemotherapy, surgery, and radiation therapy than the childhood tumorthe childhood tumorpoorer prognosis in older patientspoorer prognosis in older patients


RENAL METASTASESRENAL METASTASES

THREE TUMORS WITH THE HIGHEST THREE TUMORS WITH THE HIGHEST FREQUENCIES OF RENAL METASTASESFREQUENCIES OF RENAL METASTASESlung carcinomalung carcinomabreast carcinomabreast carcinomacarcinoma of the opposite kidneycarcinoma of the opposite kidney

Neoplasms of the Neoplasms of the Renal Collecting Renal Collecting System,Pelvis, & System,Pelvis, &

UreterUreter

Neoplasms of the Neoplasms of the Renal Collecting Renal Collecting System,Pelvis, & System,Pelvis, &

UreterUreter

Neoplasms of the Renal Collecting System,Pelvis, & Ureter

• Fetal mesonephros – where collecting systems of the urinary tract develop

• Metanephric blastema – cell origin of the renal parenchyma

Primary Renal Neoplasms of the PelvisPrimary Renal Neoplasms of the Pelvis

10% of renal tumors75% - 80% are malignantmost are transitional cell carcinomafar less common than hypernephroma 2-3x more common than ureteric neoplasmsbladder carcinoma 50x more common than ureteric neoplasmsdivided mainly into :

80% papillary20% non papillary – considered malignant by most pathologists

Pelvic Papillomas Pelvic Papillomas and Transitional and Transitional Cell CarcinomasCell Carcinomas

Pelvic Papillomas Pelvic Papillomas and Transitional and Transitional Cell CarcinomasCell Carcinomas

Pelvic Papillomas and Transitional Cell CarcinomasPelvic Papillomas and Transitional Cell Carcinomas

Etiology exposure to variety of noxious stimuliinfection or stones identified in a significant number of patientsoccupational exposure to a host of chemicals including dyes, rubber, cable and plasticsmost cases- aromatic hydrocarbonsphenacetin abusemay increase incidence of TCCA in smokeras and coffee drinkers

Multiplicity frequent and important feature of transitional cell carcinoma25% of patients with renal pelvic papillomas ultimately develop carcinomaof those with multiple papillomas, 50% develop carcinomas


Pathologic Findings

Papillary Carcinoma – Grade I (Papilloma)grossly consists of long, cylindrical, villous processarises from a narrow base and is only a few millimeters in diameter but can be as large as several centimetersnonmalignant proliferation of transitional cells often associated with independent transitional cell carcinoma


Papillary Carcinoma – Grade II and abovetransitional cell mucosa has the capacity to undergo both benign and malignant squamous and/or glandular differentiationrelative frequency of epithelial carcinoma

90-92% transitional cell 8% squamous cell1 % adenocarcinoma or undifferentiated tumor of the renal pelvis


Epithelial Tumors of the Pelvis


Staging ClassificationStaging ClassificationI. Papillary or nonpapillary without invasionII. Papillary or nonpapillary, superficially invasive but limited to

the lamina propriaIII. Papillary or nonpapillary involving the muscularis only (it

may extend past the muscularis in the intrarenal portions of the renal pelvis if confined to the kidney)

IV. Tumor extending to the adventitial surface, involving adjacent structures and/or metastatic


Clinical Findingsmost frequently in adults60s or older male to female ratio is 2-3 : 1higher incidence in Balkan countries (Bulgaria, Romania, Greece, Yugoslavia)hematuria – most common symptom (70% to 80% )abdominal pain, mass, pyuriadysuria and urinary frequency reported more frequently with ureteral tumors

Transitional Cell CarcinomasTransitional Cell Carcinomas

Urine Cytology59% accuracy rate especially in Grade III and advanced stage tumor, in which there was 79% and better accuracy rate


• ImagingPlain films – of little helpIVU

most common finding is filling defect, either single, or multiplemaybe smooth, but usually irregular, stippled, serrated and frondlikemaybe flat with minimal or no intraluminal intrusion in the nonpapillary types, or may have a pedicle



IVU shows a large transitional cell carcinoma of the pelvis deforming the upper pole calyx and infundibulum. Note the papillary configuration to the inferior edge of the lesion

IVU shows a smooth sharp cut-off of an upper pole calyx


IVU “stipple sign” – trapping of contrast material within the interstices of the tumorif the tumor obstructs, global nonfunction maybe presentphantom calyx – calyceal infundibulum is obstructed, involved calyx is not opacified

Stipple Sign in Transitional Cell CarcinomaStipple Sign in Transitional Cell Carcinoma


Intravenous UrographyFive Distinct Urographic Rule

1) discrete filling defects (35%)2) filling defects with distended calyces (26%)3) calyceal obliteration (19%)4) hydronephrosis with renal enlargement (6%)5) reduced function without renal enlargement (13%)


Note:

If a lesion is identified in one renal focus, a careful search should be instituted for other neoplasms involving the ipsilateral or contralateral collecting system as well as the bladder


RGPif kidney does not function on IVUmost readily demonstrates the extent of the tumormap the entire urothelial surface of both kidneys and ureters when IVU failed to ideally visualized these structuresfindings similar in IVU



Antegrade Pyelographyin hydronephrotic nonfunctioning kidneyfor decompressive and diagnostic purposesupper margins of the tumor can be delineatedurine for cytology can be collectedbrushings from the lesion can be obtained



Computed Tomographysolid, round or flat mass in the renal pelvisa ballooned tumor-filled calyx or calyceal group (oncocalyx)compression or invasion of the renal sinus fat

TRANSITIONAL CELL CA OF THE RENAL PELVIS

Axial nonenhanced CT scan shows a mass (arrow) in the right renal pelvis. The mass is slightly hyperattenuating relative to the urine and renal parenchyma.



Computed TomographyImportant CT characteristics are:

preservation of the reniform outline of the kidneytendency of the transitional cell carcinoma to spread medially through the hilushilar nodal enlargement can also be identified



Computed TomographyIn contradistinction to Hypernephromas

arise in the cortextherefore, usually distort and deform the renal outlineinvade the perinephric space laterally and through the Gerota’s fascia



Computed Tomographyreveal the tumor and its relationship to surrounding structuresaccurately distinguish early-stage (stage I and stage II) from advanced-stage (stage III and IV) staging of advanced disease by demonstrating gross parencymal invasion, tumor extension, metastatic spread


Ultrasonographysignificant role in differentiating renal pelvic defectstransitional cell carcinoma causes a separation of the central renal echo complex by an area of low intensity echoes representing the tumorif diffuse, the renal parenchyma may appear widened sonographically and exhibit a low intensity echo pattern


Angiographyhypovascular to avascularfine tortuous neovascularity may be encountered (56% to 82%) with an occasional tumor blushsome encasement of arteries and veins (15% to 82%)involvement of inferior vena cava and renal vein can occurmacroscopic venous is a late finding denoting poor prognosis


Magnetic Resonance Imaging

has little to offer in the diagnosis and management of renal transitional cell carcinoma


Metastaseshematogenous spread is less common with renal pelvic tumors than with hypernephromas

because of the extensive lymphatic supply to the pelvis, lymphogenous involvement can occur early in the disease.lungs, lymph nodes and liverdirect extension to the retroperitoneumCT is the study of choice to detect extrarenal extension and nodal involvementosteolytic or osteoblastic bone metastases

squamous Cell squamous Cell CarcinomasCarcinomas

squamous Cell squamous Cell CarcinomasCarcinomas

Squamous Cell CarcinomasSquamous Cell Carcinomas

Pathology0.5% of all renal neoplasms6.2% of renal pelvic tumorssolid and flat, often ulceratingall are classified as nonpapillary and all are malignanthigh incidence of associated pelvic calculus (40%-80%) and pyelonephritisaggressive tumors with a strong tendency to infiltratepoor prognosis


Clinical FindingsClinical Findingsstrong association between squamous cell carcinomas and chronic irritation (cigarettes, coffee), infection or calculusmale to female ratio is equaloften associated with schistosomiasis when ureteral reflux is presenthematuria - present in most cases (late finding)weight loss and abdominal mass


Clinical FindingsClinical Findingsradiographicallystone is usually present on the plain filmkidney is usually enlarged but maintains its reniform outlinemimic xanthogranulomatous pyelonephritis (XGP) RGP may have cobblestone appearance

Squamous Cell Carcinoma

Squamous cell carcinoma in a 50-year-old man with chronic calculus disease and left flank pain. (a) Axial unenhanced CT scan shows a high-attenuation stone (arrow) in the left renal pelvis. A tiny amount of air (arrowhead) due to previously performed percutaneous nephrostomy is seen in the renal sinus. (b) Axial contrast-enhanced CT scan obtained during the excretory phase shows an infiltrative mass (arrows) in the renal pelvis that extends to the renal parenchyma. Note the metastatic lymph nodes (arrowhead) in the paraaortic space.

AdenocarcinomaAdenocarcinomaof theof the

renal pelvisrenal pelvis

AdenocarcinomaAdenocarcinomaof theof the

renal pelvisrenal pelvis

Adenocarcinoma of the Renal PelvisAdenocarcinoma of the Renal Pelvis

pathological appearance similar to colonic carcinomadescribed as heaped up, glistening, mucoid, globular masses, but they can present as flat masscalculus, usually present in 2/3 of casesalmost all patients have urinary tract infectionshematuria most common presenting complaint

mesodermal tumors mesodermal tumors of the renal pelvisof the renal pelvis

mesodermal tumors mesodermal tumors of the renal pelvisof the renal pelvis

Fibroepithelial PolypsHemangiomasLeiomyomasRenal Medullary Interstitial Cell TumorsMalignant Mesodermal TumorsSecondary Tumors Involving the Renal Pelvis

MESODERMAL TUMORS OF THE RENAL PELVIS

tumors of the tumors of the URETERURETER

tumors of the tumors of the URETERURETER

MALIGNANT EPITHELIAL TUMORSTransitional Cell CarcinomasSquamous Cell Carcinoma and Adenocarcinoma of the Ureter

BENIGN URETERAL TUMORSBenign Epithelial TumorsInverted PapillomasFibroepithelial Polyp and Other Benign Nonepithelial Tumors

Transitional Cell Carcinomas

Etiology hyperplastic metaplastic changes secondary to chronic irritationcauses similar to pelvic tumors and include calculi, infection, hormonal factors, and various carcinogens

Incidencebetween 1 in 1000 and 1 in 3600

PathologyPapillary – tumor is attached to the ureteral wall by a broad pedicleNonpapillary (40%)


Site of Metastases From Ureteral Tumorsmetastases from ureteral transitional cell ca is far more common than from bladder cancerureteral wall is thin and presents a poor barrier for tumor extensionextensive ureteral lymphatic drainage system – provide excellent pathway for tumor spreadrelative frequency as to the site of metastases:

retroperitoneal lymph nodes (34%) , distant lymph nodes (17%), liver (17%), lumbar vertebrae (13%), lungs (9%), kidneys (8%), adrenals (4%), spleen (2%), sacral vertebrae (2%), brain (2%), pancreas (2%), skind (2%)


Clinical Findings5th – 7th decades (but are seen in almost any age)2.4 : 1 male to female ratio70% lower 3rd of the ureterhematuria is the most common symptom (55% - 74%)pain is present in 50% of caseselevated LDH level (but nonspecific)

Plain Film Findingsdemonstrating metastatic lesions to lung or bony structures.associated renal or ureteral calculi may also be recognized.

Transitional Cell Carcinoma

IV urogram reveals a round, smooth filling defect in the distal end of the right ureter.

A bulb retrograde pyelogram defines the tunmor and shows some contrast in its interstices


Retrograde Studiesmainstaymainstay of the diagnostic armamentarium goblet sign – unique feature

dilatation of the ureter below the tumor in the shape of a champagne glass

Antegrade Pyelographysuperior extent of the tumor is readily identified and characterizedinferior extent can only be determined by RGP or CTrisk of seeding must be considered


Computed Tomographynot the primary study for diagnosis of ureteral tumorsplay a significant role in diagnosis and treatment

with nonfunctioning kidney secondary to obstruction by a ureteric tumor, CT will identify the dilated urine-filled collecting system and demonstrate the level of obstruction

Angiographysimilar to those seen in intrapelvic tumor (hypovascular mass)periureteral and peripelvic renal vessels usually supply the tumor


Treatmentnephroureterectomy with resection of a cuff of bladderwide excision of the tumor

BENIGN URETERAL TUMORSFibroepithelial Polyp

typical finding is “wormlike” intraureteral projections

secondary tumors of secondary tumors of the URETERthe URETER

secondary tumors of secondary tumors of the URETERthe URETER

SECONDARY TUMORS OF THE URETER

1. Secondary Tumors That Involve The Ureter via The Urinary Route

• graft implantation in the direction of the urinary flow from the primary site

• ureterovesical reflux from a bladder tumor• exposure of the entire ureteral epithelium to noxious

agents• lateral encroachment of renal pelvic or bladder

tumors through the lumen, submucosa, or mucosa of the ureter


2. Metastatic Tumors• melanoma, bladder, colon, breast, stomach, lung,

seminoma, lymphoma, esophagus, prostate, etc.• Criteria for True Metastases

a) involvement of the ureter by growth within the wallb) evidence of periureteral lymphatic involvementc) no evidence of direct extension or contiguity of tumor


3. Direct Extension From Neighboring Organs or Structures

• any pelvic or retroperitoneal neoplasm can invade and/or obstruct the ureter

• carcinoma of the cervix is the most important culprit

bladder cancerbladder cancer

Diagram shows the urothelium (a), lamina propria (b), muscularis propria (detrusor muscle) (c), and adventitia (d).

Normal bladder Wall

Neoplasms of the Urinary BladderNeoplasms of the Urinary Bladder

Benign conditions of the urinary bladder can be subdivided into:

1. Benign variants ( benign proliferative changes of the urothelium and Brunn’s nests)2. Cystitis-like changes (cystitis cystica and cystitis glandularis)3. Benign nonepithelial tumors (leiomyoma, neurofibroma, nephrogenic adenoma)4. Proliferative inflammatory lesions simulating neoplasm (bladder papilloma in childhood, inflammatory pseudopolyp, fibrous polyp, villous papilloma of the colonic type, condyloma acuminatum, eosinophilic cystitis, endometriosis, malacoplakia, and amyloidosis)


95% of all bladder neoplasms are malignant90% - 95% of urinary bladder malignancies are transitional cell carcinomas (urothelial tumors)

5% to 10% squamous cell carcinoma and adenocarcinomamidline abdominal mass extending from the anterior superior surface of the bladder, often associated with calcificationsCT and MRI reveal a solitary, lobulated tumor arising from the dome of the bladder on the ventral surface.


2/3 of malignant tumors are superficialpapillary1/3 of malignant tumors show infiltration in or beyond muscular layer of the bladder wall.4th leading cause of cancer death in men10th leading cause in women6th and 7th decades4 : 1 male to female ratio


RADIOLOGIC IMAGING MODALITIESRADIOLOGIC IMAGING MODALITIES

Ultrasound

transabdominal (suprapubic), transrectal, transvaginal, and intravesical ultrasoundaccuracy of transabdominal ultrasound for local staging is 61% to 84%disadvantages:

inability to distinguish malignant tumors from chronic cystitis, local bladder wall hypertrophy, or blood clots.differentiation between superficial and deep invasion of bladder wall is not accurate accuracy in detecting LN metastases is very low


RADIOLOGIC IMAGINGRADIOLOGIC IMAGING MODALITIES MODALITIES

Ultrasound

transrectal and transvaginal is rarely usedshow bladder neck and trigone but not the dome and anterior wall of the urinary bladder

Urothelial carcinoma. Longitudinal US image of the bladder shows a large, hypoechoic urothelial carcinoma (arrow) within the bladder.



intravesical ultrasound fares best for staging accuracy : 62% - 92%highest accuracy seen in superficial tumorsdisadvantages:

inability to differentiate between malignant tumors and inflammation, etc.unreliable for large tumorsinvasive technique

Axial CT image shows a large, lobular mass within the bladder.

Axial CT image of the bladder shows an enhancing area of focal wall thickening (arrow), which represents a urothelial carcinoma. Flat lesions are more difficult to detect with radiologic studies, especially if the bladder lumen is not well distended.



Scintigraphylimited to the detection of bone metastasesMRI is the most sensitive and specific technique to detect bone marrow metastasesMagnetic Resonance Imagingsuperior in staging malignancy

Noninvasive papillary urothelial tumor. (a) Coronal T2-weighted MR image shows an intermediate-signal-intensity mass (arrow) within the bladder lumen. The hypointense bladder wall is intact. (b) Coronal early phase gadolinium-enhanced dynamic T1-weighted MR image shows that the tumor enhances more than the bladder wall (arrow).

Invasive urothelial carcinoma. Axial gadolinium-enhanced fat-suppressed T1-weighted MR image of the bladder shows tumor invasion into the perivesical fat (arrows).


RADIOLOGICAL EVALUATIONRADIOLOGICAL EVALUATION

MRI is the modality of choice in imaging urinary bladder anatomy and bladder cancershould be used only to obtain information that directly influences therapeutic management and outcomedetection of urinary bladder cancer – by cystoscopy and cytological/ histological examinationstaging after diagnosis

DIFFERENTIATION CLINICAL STAGING INCLUDING TUR

INTRAVESICAL SONOGRAPHY

CT MRI

T0-T + + + - - +

Tis – Ta + + + - -

Ta – T1 + + + + - -

T1- T2 + + + + - 0

T2a – T2b 0 0 / + - +

T3a – T3b - 0 + + + +

T3b – T4a - 0 + + +

T4a – T4b - - + + +

N0 – N + - - + +

M0 – M + - - 0/+ + +


OVERVIEW OF DIFFERENT STAGING TECHNIQUESOVERVIEW OF DIFFERENT STAGING TECHNIQUES

Diagram shows the stages of tumor invasion in bladder cancer. Tumors are considered superficial if they do not extend beyond the lamina propria (T1 or less). Once the muscle layer (muscularis propria) has been invaded (T2a or greater), the tumor is considered invasive.


RADIOLOGICAL EVALUATIONRADIOLOGICAL EVALUATION

superficial-appearing tumors – TUR and histological examinationIVU to rule out multifocal carcinoma in the upper collecting system or ureterfollow-up – cystoscopy every 3 to 6 months (no further imaging is needed)because MRI is the best best staging modality for invasive or staging modality for invasive or metastasized tumors, patients with stages appearing to be metastasized tumors, patients with stages appearing to be higher than T2 or with stage T1, grade 3 disease should be higher than T2 or with stage T1, grade 3 disease should be considered for further staging with MRI.considered for further staging with MRI.

renal neoplasm

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