Renal Tumors


 True or False (T/F)

1. There is a strong association of nephroblastomatosis with a second
primary malignancy of the central nervous system.
2. Angiomyolipomas associated with tuberous sclerosis occur at a later
age and tend to be smaller in size than the sporadic lesions.
3. Lymphangiomyomatosis is an idiopathic disease that occurs in young
women and consists of smooth muscle hamartomas along the
lymphatic system.
4. Malignant fibrous histiocytoma was formerly known as
hemangiopericytoma, and is a rare spindle cell neoplasm found most
frequently in the pleura.
5. The most common appearance of renal lymphoma is that of a solitary
mass.
 True or False (T/F)

6. Tumor invasion of the renal vein and into the IVC is commonly
seen in renal adenocarcinoma, but is rare in renal metastases.
7. In patients with leukemia, kidneys are often symmetrically
enlarged.
8. Renal metastases demonstrate low signal intensity on T2-
weighted images
9. Category IIF masses are mildly atypical, but still likely benign
10. Category III cystic renal masses less than 3 cm are most likely
benign and do not need long-term follow-up.
Renal Tumors

Renal Parenchymal
Tumors

Rhabdoid tumor
Nephroblastomatosis
Mesonephric Blastoma
 Other Renal Parenchymal Tumors

Rhabdoid Tumor of the Kidney (RTK)


 Uncommon childhood tumor (median age: 11mos)
 Originally considered a sarcomatoid variant of
Wilms tumor
 Strong association with second CNS primary
(astrocytoma, ependymoma, or PNET) [ 1. False ]
 Metastasis from RTK to brain are common
 large, centrally located, heterogeneous soft-tissue masses,
involving the renal hilum with indistinct margins
Rhabdoid Tumor of
the Kidney (RTK)
 Other Renal Parenchymal Tumors

Nephroblastomatosis

 results from an arrest in normal nephrogenesis
 characterized by persistent nephrogenic blastema
 NOT malignant but associated with Wilms tumor
 may be diffuse or multinodular
 treatment: anti-neoplastic drugs
 Other Renal Parenchymal Tumors

Nephroblastomatosis

Radiographic features:
 dependent on the size and distribution of the embryologic remnants
 Multi-focal: usually microscopic nodules; difficult to image
 Diffuse: kidneys are enlarged; collecting system maybe deformed by
parenchymal nodules
 Lesions may be hypoechoic, but may also be iso- or hyperechoic
 Subcapsular location

Angiographic features
 normal-caliber main renal artery
 hypovascular peripheral nodules which do not blush with contrast
 kidney has a scalloped appearance
 Other Renal Parenchymal Tumors

Nephroblastomatosis

 

Other Renal Parenchymal

Tumors
Nephroblastomatosis
 Other Renal Parenchymal Tumors

Mesoblastic Nephroma

 benign tumor
 detected at birth or during first few months of life
 congenital Wilms tumor or fetal mesenchymal
hamartoma
 large, averaging over 6 cm in diameter
 histopath: interlacing sheets of fibromatous cells;
small bundles of fibromatous cells diffusely
interspersed in the adjacent renal parenchyma
 cut surface shows whorled appearance resembling
leiomyoma
 Other Renal Parenchymal Tumors

Mesoblastic Nephroma

 rarely extends into the renal vein or renal pelvis
 Ssx: nontender abdominal mass
 Tx: surgical excision, nephrectomy, chemo/radiotherapy

 Utz: homogenously echoic (most common); occasional

hyperechoic foci are seen; areas of necrosis or
hemorrhage are readily detected as hypoechoic regions
 CT: uniform, solid, intrarenal mass
 Angiography: moderately vascular, with tumor
vascularity
 Other Renal Parenchymal Tumors

Mesoblastic Nephroma

Mesenchymal Tumors

Angiomyolipoma
Fibrous Tumor
Osteosarcoma
Lipoma
Leiomyoma and Leiomyosarcoma
Malignant Fibrous Histiocytoma
Hemangioma
Lymphangioma
Juxtaglomerular tumor
 Mesenchymal tumors

Angiomyolipoma

 Hamartomatous tumors composed of mature
adipose tissue, thick-walled blood vessels, and sheets
of smooth muscle
 Mean age at presentation: 41 years
 Usually asymptomatic
 If with symptoms: flank pain, hematuria and
hypertension
 Treatment: usually surgical (tumorectomy or partial
nephrectomy; selective arterial embolization; elective
exploration with renal-sparing surgery)
 Mesenchymal tumors

Angiomyolipoma

 Presentation may vary
Sporadic Tuberous sclerosis
Solitary Multiple
Unilateral Bilateral
Female predilection No sex predilection
Older age Younger age
[2. False ]
Smaller in size Larger in size
Associated with multiple
renal cysts
 Mesenchymal tumors

Angiomyolipoma

 Lymphangioleiomyomatosis
 a forme fruste of tuberous sclerosis
 an idiopathic disease that occurs in young women
 consists of smooth muscle hamartomas along the
lymphatic system [3. True ]
 most commonly involves intrathoracic lymphatics
 pulmonary findings: diffuse, reticular or
reticulonodular pattern and multiple small cysts; the
radiographic pattern may be honeycomb-like
 associated with renal hamartomas
 Mesenchymal tumors

Angiomyolipoma

 Plain abdominal radiograph: may demonstrate a relatively
lucent mass (if tumor has a large fatty component).
Calcifications are uncommon.
 Utz: highly echogenic renal mass
 Sonolucent areas (if with hemorrhage)
 Echogenic appearance may be mimicked by renal adenoCA
 If with relatively little fat, may be indistinguishable from other
renal masses
 CT (most reliable): renal mass of fat density
 If lipid poor: homogenous tumors with attenuation higher
than normal renal parenchyma, and homogeneous
enhancement after administration of contrast material
 Mesenchymal tumors

Angiomyolipoma


Highly echogenic pattern

 Mesenchymal tumors

Angiomyolipoma


No detectable fat content Multiple bilateral fatty tumors

 Mesenchymal tumors

Angiomyolipoma

 MRI: high signal intensity is seen on both TI - and T2-
weighted images
 Arteriography: tortuous, almost aneurysmally dilated
vessels; no vascular encasement
 Angiomyolipomas do not calcify
 Absence of fat does not exclude the diagnosis of
angiomyolipoma
 Mesenchymal tumors

Fibrous Tumor

 Solitary fibroma tumor; formerly known as
hemangiopericytoma
 A rare spindle cell neoplasm found most frequently in the
pleura [4. False ]
 May arise in the renal pelvis, cortex, or capsule
 Usually benign
 Treatment: surgical removal
 CT/MRI: tumor is usually well circumscribed, often large. greater
than 5 cm in diameter. Enhancement is homogenous, although
areas of necrosis may be seen.
 Mesenchymal tumors

Osteosarcoma

 rarely a primary renal neoplasm but may arise from a
fibrosarcoma undergoing metaplasia to tumor osteocytes
 Dx: presence of formed bone
 DDx: metastasis to the kidney from a primary skeletal
osteosarcoma
 primary renal osteosarcoma – elderly
 primary skeletal osteosarcoma - adolescents and young
adults
 Mesenchymal tumors

Lipoma

 rare
 small and seldom symptomatic
 middle-aged women
 CT: well-defined fatty mass, but may not be distinguishable from
an angiomyolipoma
 Mesenchymal tumors

Leiomyoma & Leiomyosarcoma


 arise from vessel walls or scattered muscle fibers of the
renal capsule
 found in the lower poles

Leiomyoma
 most are small, asymptomatic and female preponderance
 increased incidence in patients with tuberous sclerosis
 CT: well-defined, peripheral lesions demonstrating homogeneous
enhancement
 MRI: low signal intensity on both Tl- and T2-weighted images
 Mesenchymal tumors

Leiomyoma & Leiomyosarcoma


Leiomyosarcoma
 large and locally invasive by the time they become clinically
apparent
 tend to metastasize widely
 poor prognosis
 Tx: surgical resection (local recurrence is common)
 Mesenchymal tumors

Malignant Fibrous Histiocytoma


 Aka undifferentiated pleomorphic sarcoma
 Rarely seen as a primary renal tumor.
 Ages 13 to 68
 Symptoms are nonspecific, and most
 Tumors are large by the time they are detected
 Common metastatic sites: liver, lung, and bone
 Local recurrence is common
 Poor prognosis
 Mesenchymal tumors

Hemangioma

 uncommon renal tumors of endothelial cells and capillary
size vessels.
 cavernous - if the vessels are dilated
 usually small, ranging from several millimeters to 5 cm in
diameter
 most frequently located at the apex of the renal pyramids
 Ssx: hematuria (most common), colicky pain
 third or fourth decade
 M=F
 Mesenchymal tumors

Hemangioma

 Plain radiograph, urogram: normal
 CT: intense enhancement persisting into the venous phase (if
large)
 Selective renal angiography (most useful in dx): dense tangle
of vessels that may allow arteriovenous shunting is seen;
ipsilateral renal artery not usually enlarged
 vasoconstrictive drugs may improve the diagnostic
capability by constricting normal renal arteries and shunting
contrast into the hemangioma
 Mesenchymal tumors

Hemangioma

 Mesenchymal tumors

Lymphangioma

 aka renal lymphangiectasia or lymphangiomatosis
 rare
 thought to be a developmental anomaly of the lymphatic
system
 multiloculated cystic mass
 often bilateral
 most frequently in the renal sinus or perinephric space
 asymptomatic
 Mesenchymal tumors

Lymphangioma

 Mesenchymal tumors

Juxtaglomerular tumor

 aka reninoma
 rare and benign
 3 to 7 cm in diameter
 curable cause of significant hypertension (younger)
 marked female preponderance
 histo: composed of small uniform cells with little nuclear
pleomorphism or mitotic activity; presence of renin confirmed
with an immunofluorescence antibody test
 Ssx: symptoms of moderate to severe hypertension,
hypokalemia, acute flank pain, hypotension, or anemia
 Tx: surgical (local tumorectomy or partial nephrectomy)
 Mesenchymal tumors

Juxtaglomerular tumor

 Plain abdominal radiograph: normal
 Excretory urography: mass, often peripheral in location,
does not distort the collecting system unless large in size
 Utz: echogenic mass
 CT with contrast: isodense tumor with normal renal
parenchyma
 Arteriography: splaying of adjacent vessels by a hypovascular
mass; small amount of tumor vascularity
 Mesenchymal tumors

Juxtaglomerular tumor

Renal Pelvic Tumors

Malignant: Transitional cell CA(MC), squamous cell,
undifferentiated, or adenocarcinomas
Benign: Papilloma (50%), Angioma, Fibroma, Myoma,
Polyp
Secondary tumors of the
kidney

Lymphoma
Leukemia
Multiple myeloma
Solid tumor metastases
 Secondary tumors of the Kidney

Lymphoma

 from hematogenous dissemination or direct extension
 more common in non-Hodgkin lymphomas than in
Hodgkin disease, and among patients with immune
deficiencies (including AIDS)
 often bilateral involvement
 Ssx: Fever, weight loss, palpable adenopathy
 seen as multiple lymphomatous masses (50%), solitary
mass, diffuse infiltration, perirenal lymphoma or
involvement of the renal sinus [5. False ]
 Secondary tumors of the Kidney

Lymphoma

 Utz: hypoechoic mass that
does not show enhanced
sound through transmission
 CT: homogeneous and
rounded masses with
diminished enhancement on
contrast
 MRI: medium signal
intensity on TlW and iso- or
hypointensity on T2W
 Secondary tumors of the Kidney

Lymphoma


Corticomedullary phase
Excretory phase
 Secondary tumors of the Kidney

Leukemia & Multiple Myeloma


Leukemia
 due to diffuse infiltration by leukemic cells
(lymphocytic>granulocytic)
 both kidneys are symmetrically enlarged; collecting system is
attenuated; filling defects [7. True ]

Multiple myeloma
 proliferation of plasma cells and abnormal serum and
urine proteins
 kidneys are smoothly enlarged; collecting system is attenuated
 Secondary tumors of the Kidney

Solid tumor metastases


 usually from carcinomas of the lung, breast, colon, and
melanoma
 tumor invasion of the renal vein and into the IVC is rare in
renal metastases [6. True ]
 ssx: hematuria and proteinuria; perinephric hematoma
 Utz: solid renal mass; echo-free areas
 CT: usually small and multiple masses; solitary large renal
metastasis (colon CA)
 MRI: high signal intensity on T2-W [8. False ]
 Secondary tumors of the Kidney

Solid tumor metastases


 Other

Renal capsular tumors
Indeterminate masses
 Other

Renal Capsular Tumors


 rare
 renal capsule is composed of fibrous tissue, nerves,
smooth muscle, blood vessels, and perirenal fat
 Ssx: flank mass, abdominal pain, and weight loss;
hematuria
 Plain abd radiograph & urography: soft tissue mass;
extrinsic compression or displacement of the kidney
 Indeterminate tumors

 atypical features that do not allow a confident
diagnosis, or the lesion is too small to assess reliably
 Bosniak classification
Category I
 satisfy the criteria of a benign simple cyst on CT or utz
UTZ criteria CT criteria:
• well-defined, sonolucent • Homogeneity
mass. • imperceptibly thin wall
• homogeneous and have a • CT density approximating
thin wall. that of water
• Good throughtransmission • lack of enhancement with
of sound waves with strong IVs contrast administration
posterior enhancement
 Indeterminate tumors

Category II
 minimally complicated cysts
 cysts with high attenuation, thin septations, and thin
rimlike calcifications
 less than 3 cm: most likely benign and do not need
long-term follow-up
Category IIF
 mildly atypical, but still likely benign [9. True ]
 followed for stability at 6- or 12-month intervals
 Indeterminate tumors

Category III
 Radiologically indeterminate [10. False ]
 Surgical exploration

Category IV
 Malignant