Renal Neoplasia

Question 1

What is the most common form of renal neoplasia?

Answer 1

Renal papillary adenoma

Question 2

What are the gross findings in renal papillary adenoma?

Answer 2

Cortical, discrete, yellow-gray, small, may be multiple.

Question 3

What genetic abnormalities are assocaited with renal papillary adenoma’s?

Answer 3

Trisomy 7 and 17

Question 4

What is a renal oncocytoma?

Answer 4

Rarely metastatic.
Arise from intercalated cells of renal cortical collecting ducts.
Represent 5-15% of primary renal epithelial neoplasms.
Rarely familial.

Question 5

What is the gross appearance of a renal oncocytoma?

Answer 5

Typically mahogany-brown and well-circumscribed often with a central stellate scar.

Question 6

What are the morphological features of a renal oncocytoma?

Answer 6

Abundant acidophilic, granular cytoplasm.

May show alveolar, nesting, tubular or solid pattern.

Question 7

What is a renal angiomyolipoma?

Answer 7

Tumors composed of blood vessels, smooth muscle, and adipose tissue.

Question 8

What do angiomyolipomas have a strong association with?

Answer 8

Tuberous sclerosis.

More common in women,

Question 9

What tumor suppressor genes, when lost, are associated with renal angiomyolipomas?

Answer 9

TSC1 and TSC2.

Question 10

What is the most common malignant tumor of the kidneys?

Answer 10

Renal Cell Carcinoma

Question 11

When in life and what sex do renal cell carcinomas present and affect?

Answer 11

Adults, 6-8th decade of life.

M:F 2:1

Question 12

What gives rise to renal cell carcinomas?

Answer 12

Renal tubular epithelium

Question 13

What are the risk factors for renal cell carcinomas?

Answer 13

Smoking, HTN, obiesty, estrogens, asbestos, chronic renal disease, tuberous sclerosis, acquired cystic diseases.

Question 14

What hereditary disease can lead to renal cell carcinoma and what are the characteristics of it?

Answer 14

VHL disease, loss of VHL tumor suppressor gene on chr 3.

Arise in younger adults, are bilateral, and autosomal dominant.

Question 15

What type of renal cell carcinoma makes of the majority of cases?

Answer 15

Clear Cell Carcinoma-> non-papillary, clear cytoplasm, sporadic.

Question 16

What is the second leading cause of renal cell carcinomas?

Answer 16

Papillary carcinoma-> papillary growth pattern, better prognosis.

Assocaited with trisomy 7, 16, 17.

Arise from (type B) intercalated cells of renal cortex collecting ducts.

Question 17

What is the lesion patter of sporadic vs hereditary papillary carcinoma?

Answer 17

Sporadic-> single lesions

Hereditary-> multiple lesions

• same goes for clear cell types

Question 18

What is the triad of clinical symptoms that renal cell carcinoma presents with?

Answer 18

Hematuria (many are microscopic)

Flank pain

Palpable flank mass

• also assocaited with non-specific symptoms such as weight loss, malaise, weakness, fever.

Question 19

What are the prognosises of renal cell carcinoma for papillary/ chromophobe, clear cell, and collecting duct/sarcomatioid/ medullary?

Answer 19

Papillary -> better

Clear cell-> average

collecting duct etc.-> worse

• mode of spread is typically hematogenous.

Question 20

What is the gross appearance of renal cell carcinoma?

Answer 20

Affects the upper poles of the kidneys.

Yellowish mass.

Question 21

What is the characteristic finding on histological exam of RCC clear cell type?

Answer 21

Hypernephroma. Cytoplasm is clear due to glycogen and iPod accumulation.

Question 22

What is the characteristic finding on histological exam of RCC papillary type?

Answer 22

Papillae and foamy macrophages in stalk.

Question 23

What is the characteristic finding on histological exam of RCC chromophobe type?

Answer 23

Pale, eosinophilic cells arranged in solid sheets, well-defined cell membranes, faintly granular cytoplasm with perinuclear clear halos.

Tend to concentrate around blood vessels.

Question 24

What is the name of transitional cell carcinoma of the kidney and where does it originate from?

Answer 24

Urothelial cell carcinoma, arises from urothelium that is present and lining the renal pelvis, ureter, bladder, and urethra.

Question 25

What is a common presentation of urothelial cell carcinoma?

Answer 25

Painless Hematuria that can lead to hydronephrosis and flank pain.

Question 26

What is the demographics for Wilms tumors?

Answer 26

4th most common childhood tumor.

Asians>whites>blacks

Question 27

What the 3 familial syndromes that are associated with Wilms tumors?

Answer 27

1) WAGR (33-40% have wilms) Wilms, aniridia, genital, retardation.
2) Denys-Drash Syndrome (90% have Wilms) gonadal and renal tumors
3) Beckwith-Wiedemann syndrome (non-WT1)

• 1&2 are WT1 assocaited with chr. 11

Question 28

What is the clinical presentation of a patient with a Wilms tumor?

Answer 28

Large abdominal mass, may exhibit pain, microscopic Hematuria, HTN

Question 29

What hisopathological feature would indicate a high clinical suspicion of a Wilms tumor?

Answer 29

Prescense of nephrogenic rests (precursor lesions).

Question 30

What is the typical triphasic histomorphology of Wilms tumors?

Answer 30

3 cell types are present: blastemal, epithelial, and stromal.

Anaplasia present- unfavorable, assocaited with p53 mutations.

Anaplasia absent- favorable

Question 31

What are the gross histological features of Wilms tumors?

Answer 31

Well-circumscribed margins

Tan-to-gray color

Large, expansive tumor in lower pole of kidney