Renal Neoplasm

Clinical Clinical Urography Urography Neoplastic Neoplastic Disease Disease

MARIA THERESA M. NAVARRO, MD. Fourth Year Radiology Resident Department of Medical Imaging Quirino Memorial Medical Center

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

BENIGN NEOPLASMS OF THE RENAL PARENCHYMA

Benign Neoplasms of the Renal Parenchyma

ADENOMA • 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

Benign Neoplasms of the Renal Parenchyma ADENOMA • 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.

Benign Neoplasms of the Renal Parenchyma

ADENOMA • 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)

Benign Neoplasms of the Renal Parenchyma

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

Benign Neoplasms of the Renal Parenchyma

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

Benign Neoplasms of the Renal Parenchyma

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

Benign Neoplasms of the Renal Parenchyma

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 T2weighted 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

Benign Neoplasms of the Renal Parenchyma

• • • • •

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

Benign Neoplasms of the Renal Parenchyma

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

Benign Neoplasms of the Renal Parenchyma

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 T1weighted 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).

Benign Neoplasms of the Renal Parenchyma

MULTILOCULAR CYSTIC RENAL TUMOR (Multilocular Cystic Nephroma)

Pathology unilateral and solitary gross inspection, thick well-defined fibrous capsule numerous “honeycombed” cystic areas locules do not communicate with each other or with the renal pelvis separated by fibrous septa

MULTILOCULAR CYSTIC RENAL TUMOR (Multilocular Cystic Nephroma)

Clinical Presentation equally prevalent among males and females 90% of the lesions in males occur in the 1st two years of life lesions in females – equally divided between patients younger than 5 years and between 40 and 60 years.

MULTILOCULAR CYSTIC RENAL TUMOR (Multilocular Cystic Nephroma)

Clinical Presentation childhood: nonpainful abdominal mass, although hematuria or urinary tract symptoms may lead to discovery adults: abdominal pain, hematuria and UTI

MULTILOCULAR CYSTIC RENAL TUMOR (Multilocular Cystic Nephroma)

Imaging Ultrasound or CT numerous distinct cystic areas located within well-defined encapsulated mass septations 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 rule DDX: 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.

MULTILOCULAR CYSTIC RENAL TUMOR (Multilocular Cystic Nephroma)

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

Angiography avascular, hypovascular, or hypervascular does not allow lesion to be distinguished from partially necrotic or hypovascular renal cell carcinoma

Benign Neoplasms of the Renal Parenchyma

JUXTAGLOMERULAR CELL TUMOR (Reninoma) resemble hemangiopericytomas arise in juxtaglomerular cells 50% of patients are younger than 20 years slight female preponderance plasma renin activity is elevated  hypertension patients exhibit secondary hyperaldosteronism with hypokalemia.

Reninoma

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)

JUXTAGLOMERULAR CELL TUMOR (Reninoma)

Renin can be produced by other causes: Wilm’s tumor Renal cell carcinoma Hypernephroma Imaging small size of these tumors makes them difficult to detect excretory urography and other imaging studies are normal Ultrasound shows a solid tumor, frequently with an echogenic pattern CT and MRI also demonstrate a solid tumor

Benign Neoplasms of the Renal Parenchyma

RENOMEDULLARY INTERSTITIAL CELL TUMOR (Fibroma) derived from medullary interstitial cells FIBROMAS – most common benign tumor of the renal medulla

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

Benign Neoplasms of the Renal Parenchyma

LEIOMYOMA of the KIDNEY can arise from smooth muscle in the renal capsule, cortical vessels, or pelvicalyceal system most common finding is a palpable abdominal or flank mass with or without pain majority of cases present in childhood 2/3 cases found in women ¾ develop in the lower pole of the kidney Ultrasound 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 Neoplasms of the Renal Parenchyma RENAL CELL CARCINOMA Clear cell type Papillary type Granular cell type Chromophobe cell type Sarcomatoid type Collecting duct type CARCINOID TUMOR SMALL CELL CARCINOMA LYMPHOID AND HEMATOPOEITIC TUMORS Lymphoma and leukemia Plasmacytoma

Adult Malignant Neoplasms of the Renal Parenchyma MESENCHYMAL TUMORS Leiomyosarcoma Liposarcoma Hemangiopericytoma Malignant fibrous histiocytoma and fibrosarcoma Rhabdomyosarcoma Angiosarcoma Osteosarcoma NEPHROBLASTOMA (WILM’S TUMOR) METASTATIC CARCINOMA, SARCOMA AND MELANOMA RENAL INVASION BY ADJACENT NEOPLASMS Renal medullary carcinoma Invasive renal pelvic carcinoma Retroperitoneal sarcoma Visceral carcinomas

Adult Malignant Neoplasms of the Renal Parenchyma

RENAL CELL CARCINOMA most common adult renal cancer 2% of adult malignancies male-to-female annual incidence ratio – 1.5:1 usually present in the ages 57 to 70 years (median age at diagnosis of 57 years)

RENAL CELL CARCINOMA

Pathology arise from tubular epithelial cells may originate from any segment of the nephron or from the collecting tubules or ducts clear cell type of RCC most common 70% of cases

RENAL CELL CARCINOMA

Patterns of Spread predominantly expansile growth pattern superficial tumors ultimately reach the renal capsule tumors deep in the kidney often extend toward the renal sinus and pelvis and may invade them perinephric fat and may involve ipsilateral adrenal gland

RENAL CELL CARCINOMA

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- 3x more common with right-sided tumors (owing to the shorter length of the right-renal vein)

RENAL CELL CARCINOMA

Patterns of Spread most thrombi involve only the infrahepatic vena cava LUNGS – most frequent site for metastases in RCC venous 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)

Renal Cell 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

Renal Cell Carcinoma

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.

Renal Cell Carcinoma

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 Carcinoma RENAL MASS SOLID

PSEUDOTUMORS WIDE VARIETY OF RENAL TUMORS Renal Cell Carcinoma Oncocytoma Angiomyolipoma Metastasis Lymphoma

FLUID-FILLED

Radiological Evaluation of Renal Cell Carcinoma

EXCRETORY UROGRAPHY 1. Calcification always raises the suspicion of malignancy central calcfication  strongly suggests malignancy (87%) peripheral calcification  20% are malignant 2. Renal Contour Deformities and Alteration in the Renal Axis renal cell carcinoma often causes a focal bulge in the renal contour or enlargement of the affected kidney tilting of the renal axis may occur if the mass grown in an exophytic manner, especially medially

Renal Cell Carcinoma

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)

Radiological Evaluation of Renal Cell Carcinoma

EXCRETORY UROGRAPHY 3. Abnormalities of the Collecting System distortion of the renal outline maybe absent with centrally located mass can 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 hydronephrosis pelvic 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

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.

Radiological Evaluation of Renal Cell Carcinoma EXCRETORY UROGRAPHY 4. Abnormal Blood Vessels renal cell carcinomas are usually hypervascular abnormal vessels may be seen both in and around the tumor 5. Defects in the Nephrogram some tumors are similar in density to renal parenchyma others less dense than normal renal tissue tumors 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.

Radiological Evaluation of Renal Cell Carcinoma

EXCRETORY UROGRAPHY 6. 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 extension may also be due extensive renal infiltration or ureteropelvic junction obstruction by tumor pyelotumor backflow  on RGP, contrast may dissect between the lesion and surrounding renal parenchyma 7. Metastatic Disease lung bases seen on abdominal radiographs may show metastases or malignant pleural effusion lumbar spine and pelvic bones should be examined (common sites)

CT Evaluation of Renal Cell Carcinoma APPEARANCES ON UNENHANCED SCANS unenhanced scans help determine whether a renal mass enhances facilitate distinction between hemorrhagic cysts and carcinomas most tumors have similar attenuation values similar to renal parenchyma (30 to 50 HU) inhomogenous appearance denoting changes in internal composition recent tumor hemorrhage (high density areas) small renal tumors without hemorrhage, necrosis, or alterations of renal contours maybe missed

CT Evaluation of Renal Cell Carcinoma

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

Renal Cell Carcinoma

1472 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.

CT Evaluation of Renal Cell Carcinoma 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

CT Evaluation of Renal Cell Carcinoma 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

Adult Malignant Neoplasms of the Renal Parenchyma

CYSTIC 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. 1. Unilocular most common radiological pattern (50% of cases)

Adult Malignant Neoplasms of the Renal Parenchyma

CYSTIC RENAL CELL CARCINOMA 2. • •

• •

Multilocular 30% of cases dystrophic calcification in the tumor capsule or in the septa between the locules may be seen on plain films Sonography and CT – best technique Radiologic findings – indistinguishable from multilocular cystic nephroma

Adult Malignant Neoplasms of the Renal Parenchyma

CYSTIC RENAL CELL CARCINOMA 3. Discrete Mural Tumor Nodule in a Cystic Mass least common (fewer than 20% of cases) excretory urogram shows nonspecific renal mass tumor nodule is often located at the base of the cyst, and the cyst fluid provides an excellent acoustic window for showing the tumor nodule on sonography

Cystic Renal Cell Carcinoma

(A) Precontrast image demonstrates a homogeneous lowattenuation 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).

Adult Malignant Neoplasms of the Renal Parenchyma

• •

•

• • •

PAPILLARY RENAL CELL CARCINOMA 5% to 15% of renal cell carcinomas at least 50% of the tumor is composed of cells arranged around fronds of fibrovascular stroma that project into cystic tumor spaces radiographically, show peripheral calcification on excretory urography and CT usually hypovascular angiographically CT shows a low tumor stage no consistent sonographic pattern

Adult Malignant Neoplasms of the Renal Parenchyma

MALIGNANT LYMPHOMA • • •

• • • •

primary lymphoma arising in the kidney is very rare in Non-Hodgkin’s lymphoma (10x) > Hodgkin’s disease Large cell lymphoma – most common type of NonHodgkin’s lymphoma (60% of all cases) when there is lymphomatous involvement of then kidney immunocompromised patients bilateral renal masses – most common present (40% to 60% of all cases) does not commonly produce urinary tract symptoms occurs late in the course

Adult Malignant Neoplasms of the Renal Parenchyma

MALIGNANT LYMPHOMA • •

• • •

Excretory Urography – insensitive technique Sonography – markedly hypoechoic relative to renal parenchyma (sometimes cyst-like), however, shows little posterior acoustic enhancement Computed tomography accurately depicts the presence, location, and size of renal lesions multiple nodules – most common CT manifestation CT also important in evaluating the course of the renal disease and its 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.

Adult Malignant Neoplasms of the Renal Parenchyma

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

Adult Malignant Neoplasms of the Renal Parenchyma

•

RENAL SARCOMA 1 % of malignant renal parenchymal tumors rare malignant tumors that arise from the nonepithelial mesodermal tissues of the kidney most common types are: leiomyosarcoma, hemangiopericytoma, liposarcoma, rhabdomyosarcoma, fibrosarcoma, osteogenic sarcoma

Adult Malignant Neoplasms of the Renal Parenchyma

RENAL SARCOMA 28 to 78 years of age size of renal sarcomas – 5.5 cm to 23 cm prognosis very poor indistinguishable from RCCs

Renal Sarcoma

LEIOMYOSARCOMA 58% of all renal sarcomas most common sarcoma of the kidney 4th decade of life twice as common in women as in men indistinguishable from renal cell carcinoma radiographically

Renal Sarcoma HEMANGIOPERICYTOMA rare renal tumors, 20% of renal sarcomas 18 to 49 years, women usually arise from the renal capsule and do not invade the renal parenchyma however, extensive parenchymal invasion may occur potentially malignant radiographically, present as renal masses with displacement and distortion of the renal collecting system

Renal Sarcoma

LIPOSARCOMA rare renal tumors, 20% of renal sarcomas usually arise in the renal capsule, probably from undifferentiated mesenchymal cells or in the perinephric fat, causing compression but not invasion of the renal parenchyma

Renal Sarcoma

RHABDOMYOSARCOMA 3.8 % of renal sarcomas in adults probably arises from undifferentiated mesenchymal cells highly aggressive tumors and rapidly extend to the renal veins, inferior vena cava, and right atrium.

Adult Malignant Neoplasms of the Renal Parenchyma

WILM’S TUMOR OCCURING IN ADULTS WILM’S TUMOR or NEPHROBLASTOMA, is the most common renal neoplasm of childhood peak incidence during 2nd year of life, 50% of cases undiagnosed before the 3rd birthday 75% before the age of 5 years may also occur in adolescents and adults arise in the renal parenchyma and cause marked renal destruction

Adult Malignant Neoplasms of the Renal Parenchyma

WILM’S TUMOR OCCURING IN ADULTS

IVU focal renal masses nonfunction of the affected kidney due to renal vein occlusion ureteral obstruction extensive parenchymal tumor replacement tumor calcification maybe distinguishing feature

Adult Malignant Neoplasms of the Renal Parenchyma

WILM’S TUMOR OCCURING IN ADULTS

CT inhomogenous mass with large areas of low density due to tumor hemorrhage or necrosis demonstrate perinephric extension or venous tumor thrombus

Wilm’s Tumor in a 46-year old mas

Adult Malignant Neoplasms of the Renal Parenchyma

WILM’S TUMOR OCCURING IN ADULTS

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

ANGIOGRAPHY small areas of neovascularity in a relatively hypovascular mass

Adult Malignant Neoplasms of the Renal Parenchyma

WILM’S TUMOR OCCURING IN ADULTS

PROGNOSIS poorer response to the combination of chemotherapy, surgery, and radiation therapy than the childhood tumor poorer prognosis in older patients

Adult Malignant Neoplasms of the Renal Parenchyma

RENAL METASTASES THREE TUMORS WITH THE HIGHEST FREQUENCIES OF RENAL METASTASES lung carcinoma breast carcinoma carcinoma of the opposite kidney

Neoplasms of the Renal Collecting System,Pelvis, & Ureter

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 Pelvis 10% of renal tumors 75% - 80% are malignant most are transitional cell carcinoma far less common than hypernephroma 2-3x more common than ureteric neoplasms bladder carcinoma 50x more common than ureteric neoplasms divided mainly into : 80% papillary 20% non papillary – considered malignant by most pathologists

Pelvic Papillomas and Transitional Cell Carcinomas

Pelvic Papillomas and Transitional Cell Carcinomas

Etiology exposure to variety of noxious stimuli infection or stones identified in a significant number of patients occupational exposure to a host of chemicals including dyes, rubber, cable and plastics most cases- aromatic hydrocarbons phenacetin abuse may increase incidence of TCCA in smokeras and coffee drinkers

Pelvic Papillomas and Transitional Cell Carcinomas

Multiplicity frequent and important feature of transitional cell carcinoma 25% of patients with renal pelvic papillomas ultimately develop carcinoma of those with multiple papillomas, 50% develop carcinomas

Pelvic Papillomas and Transitional Cell Carcinomas

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

Pelvic Papillomas and Transitional Cell Carcinomas

Papillary Carcinoma – Grade II and above transitional cell mucosa has the capacity to undergo both benign and malignant squamous and/or glandular differentiation relative frequency of epithelial carcinoma 90-92% transitional cell 8% squamous cell 1 % adenocarcinoma or undifferentiated tumor of the renal pelvis

Epithelial Tumors of the Pelvis

Pelvic Papillomas and Transitional Cell Carcinomas

Staging Classification I. Papillary or nonpapillary without invasion II. Papillary or nonpapillary, superficially invasive but limited to the lamina propria III. 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

Pelvic Papillomas and Transitional Cell Carcinomas Clinical Findings most frequently in adults 60s or older male to female ratio is 2-3 : 1 higher incidence in Balkan countries (Bulgaria, Romania, Greece, Yugoslavia) hematuria – most common symptom (70% to 80% ) abdominal pain, mass, pyuria dysuria and urinary frequency reported more frequently with ureteral tumors

Transitional Cell Carcinomas

Urine Cytology 59% accuracy rate especially in Grade III and advanced stage tumor, in which there was 79% and better accuracy rate

Transitional Cell Carcinomas

• Imaging Plain films – of little help IVU most common finding is filling defect, either single, or multiple maybe smooth, but usually irregular, stippled, serrated and frondlike maybe flat with minimal or no intraluminal intrusion in the nonpapillary types, or may have a pedicle

Epithelial Tumors of the Pelvis

Transitional Cell Carcinomas

IVU shows a smooth sharp cutoff of an upper pole calyx

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

Transitional Cell Carcinomas

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

Stipple Sign in Transitional Cell Carcinoma

Transitional Cell Carcinomas

Intravenous Urography Five 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%)

Transitional Cell Carcinomas

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

Transitional Cell Carcinomas

RGP if kidney does not function on IVU most readily demonstrates the extent of the tumor map the entire urothelial surface of both kidneys and ureters when IVU failed to ideally visualized these structures findings similar in IVU

Epithelial Tumors of the Pelvis Transitional Cell Carcinomas

Antegrade Pyelography in hydronephrotic nonfunctioning kidney for decompressive and diagnostic purposes upper margins of the tumor can be delineated urine for cytology can be collected brushings from the lesion can be obtained

Epithelial Tumors of the Pelvis Transitional Cell Carcinomas

Computed Tomography solid, round or flat mass in the renal pelvis a 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.

Epithelial Tumors of the Pelvis Transitional Cell Carcinomas

Computed Tomography Important CT characteristics are: preservation of the reniform outline of the kidney tendency of the transitional cell carcinoma to spread medially through the hilus hilar nodal enlargement can also be identified

Epithelial Tumors of the Pelvis Transitional Cell Carcinomas

Computed Tomography In contradistinction to Hypernephromas arise in the cortex therefore, usually distort and deform the renal outline invade the perinephric space laterally and through the Gerota’s fascia

Epithelial Tumors of the Pelvis Transitional Cell Carcinomas

Computed Tomography reveal the tumor and its relationship to surrounding structures accurately 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

Transitional Cell Carcinomas

Ultrasonography significant role in differentiating renal pelvic defects transitional cell carcinoma causes a separation of the central renal echo complex by an area of low intensity echoes representing the tumor if diffuse, the renal parenchyma may appear widened sonographically and exhibit a low intensity echo pattern

Transitional Cell Carcinomas

Angiography hypovascular to avascular fine tortuous neovascularity may be encountered (56% to 82%) with an occasional tumor blush some encasement of arteries and veins (15% to 82%) involvement of inferior vena cava and renal vein can occur macroscopic venous is a late finding denoting poor prognosis

Transitional Cell Carcinomas

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

Transitional Cell Carcinomas

Metastases hematogenous 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 liver direct extension to the retroperitoneum CT is the study of choice to detect extrarenal extension and nodal involvement osteolytic or osteoblastic bone metastases

squamous Cell Carcinomas

Squamous Cell Carcinomas Pathology 0.5% of all renal neoplasms 6.2% of renal pelvic tumors solid and flat, often ulcerating all are classified as nonpapillary and all are malignant high incidence of associated pelvic calculus (40%-80%) and pyelonephritis aggressive tumors with a strong tendency to infiltrate poor prognosis

Squamous Cell Carcinomas

Clinical Findings strong association between squamous cell carcinomas and chronic irritation (cigarettes, coffee), infection or calculus male to female ratio is equal often associated with schistosomiasis when ureteral reflux is present hematuria - present in most cases (late finding) weight loss and abdominal mass

Squamous Cell Carcinomas

Clinical Findings radiographically stone is usually present on the plain film kidney is usually enlarged but maintains its reniform outline mimic 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.

Adenocarcinoma of the renal pelvis

Adenocarcinoma of the Renal Pelvis pathological appearance similar to colonic carcinoma described as heaped up, glistening, mucoid, globular masses, but they can present as flat mass calculus, usually present in 2/3 of cases almost all patients have urinary tract infections hematuria most common presenting complaint

mesodermal tumors of the renal pelvis

MESODERMAL TUMORS OF THE RENAL PELVIS

Fibroepithelial Polyps Hemangiomas Leiomyomas Renal Medullary Interstitial Cell Tumors Malignant Mesodermal Tumors Secondary Tumors Involving the Renal Pelvis

tumors of the URETER

MALIGNANT EPITHELIAL TUMORS Transitional Cell Carcinomas Squamous Cell Carcinoma and Adenocarcinoma of the Ureter

BENIGN URETERAL TUMORS Benign Epithelial Tumors Inverted Papillomas Fibroepithelial Polyp and Other Benign Nonepithelial Tumors

Transitional Cell Carcinomas Etiology hyperplastic metaplastic changes secondary to chronic irritation causes similar to pelvic tumors and include calculi, infection, hormonal factors, and various carcinogens

Incidence between 1 in 1000 and 1 in 3600

Pathology Papillary – tumor is attached to the ureteral wall by a broad pedicle Nonpapillary (40%)

Transitional Cell Carcinomas

Site of Metastases From Ureteral Tumors metastases from ureteral transitional cell ca is far more common than from bladder cancer ureteral wall is thin and presents a poor barrier for tumor extension extensive ureteral lymphatic drainage system – provide excellent pathway for tumor spread relative 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%)

Transitional Cell Carcinomas Clinical Findings 5th – 7th decades (but are seen in almost any age) 2.4 : 1 male to female ratio 70% lower 3rd of the ureter hematuria is the most common symptom (55% - 74%) pain is present in 50% of cases elevated LDH level (but nonspecific) Plain Film Findings demonstrating 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

Transitional Cell Carcinomas

Retrograde Studies mainstay of the diagnostic armamentarium goblet sign – unique feature dilatation of the ureter below the tumor in the shape of a champagne glass

Antegrade Pyelography superior extent of the tumor is readily identified and characterized inferior extent can only be determined by RGP or CT risk of seeding must be considered

Transitional Cell Carcinomas

Computed Tomography not the primary study for diagnosis of ureteral tumors play 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

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

Transitional Cell Carcinomas

Treatment nephroureterectomy with resection of a cuff of bladder wide excision of the tumor

BENIGN URETERAL TUMORS Fibroepithelial Polyp typical finding is “wormlike” intraureteral projections

secondary tumors of the 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

SECONDARY TUMORS 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 wall b) evidence of periureteral lymphatic involvement c) no evidence of direct extension or contiguity of tumor

SECONDARY TUMORS OF THE URETER

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 cancer

Normal bladder Wall

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

Neoplasms 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)

Neoplasms of the Urinary Bladder 95% of all bladder neoplasms are malignant 90% - 95% of urinary bladder malignancies are transitional cell carcinomas (urothelial tumors) 5% to 10% squamous cell carcinoma and adenocarcinoma midline abdominal mass extending from the anterior superior surface of the bladder, often associated with calcifications CT and MRI reveal a solitary, lobulated tumor arising from the dome of the bladder on the ventral surface.

Neoplasms of the Urinary Bladder 2/3 of malignant tumors are superficial papillary 1/3 of malignant tumors show infiltration in or beyond muscular layer of the bladder wall. 4th leading cause of cancer death in men 10th leading cause in women 6th and 7th decades 4 : 1 male to female ratio

Neoplasms of the Urinary Bladder

RADIOLOGIC IMAGING MODALITIES Ultrasound

transabdominal (suprapubic), transrectal, transvaginal, and intravesical ultrasound accuracy 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

Neoplasms of the Urinary Bladder

RADIOLOGIC IMAGING MODALITIES Ultrasound

transrectal and transvaginal is rarely used show 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.

Neoplasms of the Urinary Bladder

RADIOLOGIC IMAGING MODALITIES

intravesical ultrasound fares best for staging accuracy : 62% - 92% highest accuracy seen in superficial tumors disadvantages: inability to differentiate between malignant tumors and inflammation, etc. unreliable for large tumors invasive 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.

Neoplasms of the Urinary Bladder

RADIOLOGIC IMAGING MODALITIES Scintigraphy limited to the detection of bone metastases MRI is the most sensitive and specific technique to detect bone marrow metastases Magnetic Resonance Imaging superior in staging malignancy

Noninvasive papillary urothelial tumor. (a) Coronal T2weighted MR image shows an intermediate-signal-intensity mass (arrow) within the bladder lumen. The hypointense bladder wall is intact. (b) Coronal early phase gadoliniumenhanced dynamic T1-weighted MR image shows that the tumor enhances more than the bladder wall (arrow).

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

Neoplasms of the Urinary Bladder

RADIOLOGICAL EVALUATION MRI is the modality of choice in imaging urinary bladder anatomy and bladder cancer should be used only to obtain information that directly influences therapeutic management and outcome detection of urinary bladder cancer – by cystoscopy and cytological/ histological examination staging after diagnosis

Neoplasms of the Urinary Bladder OVERVIEW OF DIFFERENT STAGING TECHNIQUES

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/+

++

DIFFERENTIATION

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.

Neoplasms of the Urinary Bladder

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