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Tumors of the Kidney and

Urinary bladder
Dr. Raid Jastania
Objectives
By the end of this session the student
should be able to:
List the common benign and malignant tumors
of the kidney
List the types of renal cell carcinoma
Know the clinical presentation of Wilms tumor
Understand the basis of the classification of
urothelial carcinoma
Case Presentation
A 63-year-old man was found to have
microscopic hematuria on urinalysis done
as part of a pre-employment physical
examination. The remainder of the
urinalysis was normal, as was the physical
exam. The patient did not complain of any
fever, weight loss, pain, malaise, weakness,
or urinary tract symptoms.
Hct: 57%
Hb: 19 g/dL
BUN: 12 mg/dL
Creatinine: 0.7 mg/dL
WBC: 7,450/mm3 with a normal
differential

Urine cytology: negative


Work-up for the hematuria was begun with
noninvasive imaging studies, which
included a renal ultrasound examination and
an intravenous pyelogram. A mass was seen
in the left kidney with both imaging studies.
The patient underwent nephrectomy.
Renal Cell Carcinoma
Renal Tumors
Benign:
Oncocytoma
Renal cell adenoma

Malignant:
Renal cell carcinoma
Wilms tumor
Urothelial carcinoma
Tumors of the Kidney
Renal cell carcinoma
Arise from tubular epithelium
85% of primary malignant tumors of the kidney
2-3% of cancer in adults
6th-7th decade of life, Men 2x> women
Increased risk in smokers, occupational
exposure to cadmium, in dialysis-associated
cysts
Clinical:
Hematuria 50%
Pain
Mass
Paraneoplastic syndrome:
Fever, polycythemia 5-10% (erythropoietin)
Hypercalcemia, hypertension, cushing syndrome
Metastases to lung, bone
Renal cell carcinoma: types
1. Conventional RCC (clear cell)
2. Papillary RCC
3. Chromophobe RCC
Renal cell carcinoma: types
1. Conventional RCC (clear cell RCC)
70-80% of RCC
Familial and sporadic
Associated with von Hippel-Lindau syndrome
VHL is autosomal dominant
Multiple tumors: hemantioblastoma of cerebellum
and retina, renal cysts, renal cell carcinoma
Germline mutation in VHL gene (3p25)
Loss of second allele by somatic mutation
Seen in sporadic RCC as well
Renal cell carcinoma: types
2. Papillary RCC
10-15% arise from proximal tubular epithelium
Multifocal, bilateral
Familial and sporadic
MET proto oncogene (7q31)
Trisomy 7, Mutation of chromosome 7
In sporadic cases: trisomy 7, 16, 17
Renal cell carcinoma: types
3. Chromophobe RCC:
5% arise from collecting ducts
Loss of Ch 1,2,6,10,13,17,21
Hypodiploidy
Good prognosis
Morphology:
Clear cell
Solitary, large, cortical, well defined
Yellow-orange, gray-white, cysts, hemorrhage,
necrosis
May extend to pelvis, ureters
May invade renal vein and inferior vena cava
Papillary
Bilateral, multiple
Chromophobe
Brown-tan
Micro:
Clear cell RCC
Lipid, glycogen
Clear cells
Round nuclei
Vascular
Papillary
Chromophobe:
Perinuclear halo, macrovesicles
Well defined cell membrane
Coventional RCC Papillary Chromophobe
Clear cell papillary Thick cell
membrane,
perinuclear halo
VHL gene 3p MET oncogene Loss of
7q chromosomes
Cortical, yellow Papillary, might Cortical, Brown
orange with be multifocal,
hemorrhage, bilateral
necrosis
Oncocytoma
Renal Cell Adenoma
Case Presentation
Our patient is a 5 year-old, Caucasian
female who presented to the primary
pediatric clinic in early spring with chief
complaints of cough, fever by touch,
and decreased activity for six days.
Our patient's illness began with
rhinorrhea and progressed to appetite
loss and fever that her parents felt was
unresponsive to acetaminophen.
On physical exam our patient appeared
worrisomely sick. She was fatigued. The
outstanding physical findings consisted of a
slightly erythematous throat. On abdominal
exam a mass of 9 cm width by 4 cm length
with regularly shaped margins was palpated
with light depth and verified with percussion in
the left upper quadrant. The mass was
smooth, slightly firm, oval, nonmobile, and did
not cross the midline. The child denied pain
during the exam, but was uncomfortable
during palpation.
Our patient was then admitted to the
children's hospital after her fever and
upper respiratory symptoms subsided
for biopsy. Biopsy confirmed diagnosis
of Wilms' tumor. The tumor was
shrunk with chemotherapy for five
months and then removed from the left
kidney via complete nephrectomy and
partial right nephrectomy.
Wilms Tumor
Wilms Tumor (Nephroblastoma)
Most common primary kidney tumor in
children
Occur commonly between 2-5 years
WT1 gene, WT2 gene
Risk with congenital malformation:
WAGR syndrome
Denys-Drash syndrome
Beckwith-Weidmann syndorme
Wilms Tumor (Nephroblastoma)
Risk with congenital malformation:
WAGR syndrome
Loss of ch 11p13 (WT1)
Aniridia, genetal abnormalities, mental retardation
Denys-Drash syndrome
Loss of ch 11p13 (WT1)
Gonadal dysgenesis
Renal abnormalities
Beckwith-Weidmann syndrome
Enlarged body organs (tongue, kideny, liver), adrenal enlargement,
hemihypertrophy (body segment enlargement)
Ch 11p15.5 (WT2)
Wilms Tumor (Nephroblastoma)
Clinical:
Mass
Cross the midline
Hematuria
Intestinal obstruction
Prognosis: good
2 year-survival: 90%
Wilms Tumor (Nephroblastoma)
Morphology:
Large well-circumscribed
10% bilateral, multiple
Soft homogeneous, tan-gray
Hemorrhage, cysts, necrosis
Triphasic:
Epithelial: tubules
Stroma: fibrous, myxoid
Blastema: small blue cells
Foci of anaplasia
Nephtogenic rests: precursor lesions
Case Presentation
A 73-year-old man presented with painless
hematuria and urinary frequency
Radical prostatocystectomy specimen
revealed urothelial carcinoma with
invasion of the muscularis,
circumferentially involving the bladder
base. Carcinoma in situ was seen at the
right ureteral margin. There was also
invasion of the prostate, local lymph nodes
and vascular structures
Tumors of the Urinary Bladder
Tumors of Urinary Bladder and
collecting system
Classification, ISUP (international society of urologic
pathology).
Benign
Urothelial papilloma
Malignant
Papillary
Papillary urothelial neoplasm of low malignant potential
Papillary urothelial carcinoma low grade
Papillary urothelial carcinoma high grade
Flat
Urothelial carcinoma in-situ
Invasive urothelial carcinoma
Squamous cell carcinoma
Tumors of Urinary Bladder and
collecting system
Clinical:
Painless hematuria
50-70 year, men 3x>women
Risk factors
Smoking
Industrial solvent, hydrocarbons, dyes
Cystitis
Schistosomiasis
cyclophosphamide
Tumors of Urinary Bladder and
collecting system
Clinical:
High recurrence rate
Fatal by ureteric obstruction
Overall survival 5y: 57%
Ureteric carcinoma 5y survival: 10%
Squamous cell carcinoma
Objectives
By the end of this session the student
should be able to:
List the common benign and malignant tumors
of the kidney
List the types of renal cell carcinoma
Know the clinical presentation of Wilms tumor
Understand the basis of the classification of
urothelial carcinoma
Pictures