Tumors of the Kidney and Urinary Bladder

Question 1

What would we expect to see on urine microcospy to lead us to suspect a tumor?

Answer 1

(+) RBCs

Normal RBC- extraglomerular process

(-) protein

Question 2

1. what is this?
   
   1. is it benign or malignant?
2. what does the tumor consist of?
3. where does the tumor originate?
4. what is the clinical significance of this tumor?

Answer 2

1. Angiomyolipoma
   
   1. benign
2. vessels, smooth muscles, fat
3. the tumor originates from the perivascular epithelioid cells
4. the clinical significance for this is a susceptibility to spontaneous hemorrhage
   5.

Question 3

Angiomyolipoma

1. genetic mutations
2. manifestations of these mutations?

Answer 3

Angiomyolipoma

1. caused by mutations in TSC1 or TSC2
2. these manifest as
   
   1. epilepsy and mental retardation
   2. skin abnormalities
   3. and other tumors

**TSC1 is involved in vascular proliferation- thats why these tumors have so many vessels

Question 4

1. what is this?
2. is it benign or malignant?
3. what type of tumor is this?
4. where does it arise?
5. the cytoplasm is rich in ________.
   
   1. what does this look like on histology?

Answer 4

an oncocytoma is an epithelial tumor that is thought to arise from intercalated cells of the collecting ducts. It benign tumor.

the cytoplasm is rich in mitochondria which makes the histology very pink.

Question 5

1. what is this?
2. when does it normally present?
3. is it benign or malignant?

Answer 5

This is a Mesoblastic Nephroma.it is acongenitalhamartoma found in thefirst 3 months of life.

it is a benign tumor

Question 6

Renal Papillary Adenoma

what is the main concern if the patient is diagnosed with this?

Answer 6

If a patient is diagnosed with renal papillary adenoma he sould be folowed to make sure that he doesn’t develop renal carcinoma because the only difference between the two is the size.

the adenoma is benign but the carcinoma is not.

Question 7

Renal Cell Carcinoma

1. most significant risk factor:
2. age at presentation:
3. Three classic diagnostic features
4. Where is it most likely to metastasize?
5. What are some paraneoplastic manifestations?

Answer 7

Renal Cell Carcinoma

1. The most significant risk factor is cigarette smoking
2. most common in adults
3. the three classic diagnostic features are:
   
   1. costovertebral pain
   2. palpable mass (depending on the size of the tumor)
   3. hematuria
4. most likely to metastasize to the lungs
5. hypercalcemia and increased hematocrit

Question 8

1. What is this?
2. what gene is affected?
3. what do we see on histology?

Answer 8

1. Clear cell carcinoma
2. VHL gene (3p25.3)
3. on histology we see rounded or polygonal shaped cells with abundant, clear cytoplasm with delicate, branching vasculature

**the lack of a tumor supressor gene stimulates cell growth and angiogenesis

Question 9

1. What is this?
2. Where does it arise?
3. What gene is associated with this?
4. This is the most common renal cell carinoma in patients with _______

Answer 9

1. This is papilary renal cell carcinoma
2. It arises from the distal convoluted tubule
3. its associated with MET gene 7q31 (a proto-onco gene)
4. this is the most common cancer in patients with cystic kidney disease

Question 10

This looks like a combo of the papillary and clear cell carcinomas and has a hign nuclear grade.

it generally shows up in young patients.

What gene is translocated?

Answer 10

Xp11 translocation carcinoma

this should be the primary differential in younger patients with a kidney tumor

the TFE3 gene is translocated resulting in its overexpression

Question 11

what is this?

where does it arise?

what do the nuclei look like on histology?

Answer 11

Chromophobe renal cell carcinoma arises from the intercalated cells of the collecting ducts.

the nuclei have a “raisinoid” appearance

Question 12

1. What is this?
2. it is the most common abdominal solid tumor in ____
3. when does the incidence peak?
4. it is a malignant tumor of _______ renal elements
   
   1. What are the three components of the tumor on histology?

Answer 12

1. This is a Wilms tumor
2. it is the most commom abdominal solid tumor in children
3. its peak incidence is between 2-5 years old
4. It is a malignant tumor of embryonal renal elements
   
   1. the three histological components are:
      
      1. blastemal
      2. epithelial
      3. stroma

Question 13

Von Hippel-Lindau Syndrome

1. associated with what type of carcinoma?
2. what are some associated features?

Answer 13

Von Hippel-Lindau Syndrome is an autosomal dominant cancer syndrome that involves a 3p-

1. it is associated with a clear cell carcinoma
2. associated with
   
   1. cerebellar hemangioblastoma
   2. retinal angiomas
   3. pheochromocytoma
   4. and cystic lesions in various organs

Question 14

What is this?

what would you see on an imaging study?

Answer 14

Urothelial carcinoma that arises from the urothelial lining anywhere from the calyx to the bladder. you will see the hydronephrosis (bear claw) on imaging

Question 15

This is a syndrome associated with Wilms tumor.

some associated findings include: aniridia, genito-urinary anomalies, and retardation

is it hereditary?

what gene is affected?

Answer 15

WAGR syndrome

this is a not hereditary defect. Its due to 11p deletion that occurs during development of egg or sperm

Question 16

This syndrome is associated with Wilms tumor.

it has an early onset nephropathy and diffuse mesangial sclerosis.

what is the inheritance pattern?

What is the Gene?

what are associated findings?

Answer 16

Denys-Drash syndrome

the early onset is due to the dominant inheritance pattern (affects the structure)

inactivation of WT1.

this syndrome produces gonadal dysgenesis and male pseudohermaphroditism

Question 17

This syndrome has the following characteristic features:

enlargement of organs, macroglossia, heihypertrophy, omphalocele, and abnormal large cells in the adrenal cortex.

what is the syndrome?

what causes it?

Answer 17

Beckwith-Wiedemann Syndrome

its due to genomic imprinting of 11p15.5

Question 18

WT1 mutations cause

Answer 18

Wilms tumor genesis

WT1 is a tumor supressor gene

Question 19

Tumors of the Urinary Bladder:

1. what is the dominant clinical symptom?
2. Risk Factors?
3. What is the most common type?
4. What type is most prominent in Egypt and Sudan?

Answer 19

Tumors of the Urinary Bladder:

1. The dominant clinical symptom is painless hematuria.
2. CIGARETTE SMOKING
3. The most common type is urothelial (transitional tumors)
4. The most prominent type of urinary bladder tumor in Egypt and Sudan is a squamous cell carcinoma
   
   1. associated with urinary schistosomiasis

Question 20

What do we need to be concerned about in a patient that has been diagnosed with a urothelial tumor?

Answer 20

Frequent recurrences; patients are closely followed up with periodic cytoscopies and urine cytologies for life

Question 21

An invasive urinary bladder tumor must reach what?

Answer 21

the detrusor muscle of the bladder