Pathology 8

Question 1

what are three examples of benign neoplasms of the kidney?

Answer 1

renal papillary adenoma, angiomyolipoma, oncocytoma

Question 2

what is renal papillary adenoma?

Answer 2

small discrete adenomas arising from the renal tubular epithelium

Question 3

how might angiomyolipoma present?

Answer 3

with tuberous sclerosis and intellectual disability

Question 4

how does an oncocytoma appear?

Answer 4

large benign appearing cells with eosinophilic cytoplasms with scattered large nuclei; mahogany brown

Question 5

when do renal cell carcinomas present?

Answer 5

older individuals- 6th-7th decade of life; usually males

Question 6

what is the most significant risk factor for renal cell carcinoma?

Answer 6

tobacco

Question 7

most renal cancer is sporadic, but unusual forms of autosomal dominant familial cancers occur, usually in younger individuals. what are 4 examples of familial variants of renal cancers?

Answer 7

von hippel lindau (VHL) syndrome, hereditary leiomyomatosis and renal cell cancer syndrome, hereditary papillary carcinoma, birt-hogg-dube syndrome

Question 8

what is von-hippel-lindau (VHL) syndrome?

Answer 8

hemangioblastomas of the CNS, cysts that involve the kidneys, renal cellcarcinomas, and pheochromocytomas

Question 9

what are the risk factors for renal cell carcinomas?

Answer 9

obesity, hypertension, unopposed estrogen therapy, and exposure to asbestos, petroleum products, and heavy metals; also increased in patients with ESRD, CKD, and acquired cystic disease and tuberous sclerosis

Question 10

renal cell carcinomas may arise in any portion of the kidney, but more commonly affect what?

Answer 10

the poles

Question 11

the classification of renal cell carcinoma is based on what?

Answer 11

correlative cytogenic, genetic, and histologic studies of both familial and sporadic tumors

Question 12

what are the different types of renal cell carcinomas?

Answer 12

clear cell carcinomas, papillary carcinoma, chromophobe carcinoma, Xp11 translocation carcinoma

Question 13

where do clear cell carcinomas likely arise?

Answer 13

proximal tubular epithelium-usually as solitary unilateral lesions

Question 14

why are clear cell carcinomas yellow?

Answer 14

there are prominent lipid accumulations

Question 15

how do tumor cells appear in clear cell carcinomas?

Answer 15

they have a rounded or polygonal shape and abundant clear or granular cytoplasm

Question 16

what occurs in 98% of clear cell type renal carcinomas?

Answer 16

there is a loss of sequences on the short arm of chromosome 3

Question 17

what is the purpose of the VHL gene?

Answer 17

it acts as a tumor suppressor gene in both sporadic and familial cancers

Question 18

what happens when VHL is deleted?

Answer 18

there is increased expression of HIF1, leading to increased expression of VEGF and IGF-1

Question 19

where is the VHL gene?

Answer 19

3p25.3

Question 20

buzzwords for papillary carcinoma?

Answer 20

psammoma bodies and interstitial foam cells

Question 21

what could lead to sporadic clear cell carcinoma?

Answer 21

deletions on chromosome 3 (cytogenetics)

Question 22

what could lead to hereditary clear cell?

Answer 22

loss of VHL, inactivated mutated VHL, and hypermethylation of VHL (genetics)

Question 23

what could lead to sporadic papillary carcinoma?

Answer 23

trisomy 7, 17, loss of Y

Question 24

what could lead to hereditary papillary carcinoma?

Answer 24

trisomy 7, mutated activated MET

Question 25

what are the characteristic features of Xp11 translocation carcinoma?

Answer 25

young children, translocations of the TFE3 gene at Xp11.2

Question 26

what are some symptoms of renal cell carcinomas?

Answer 26

flank pain, palpable mass, hematuria, constitutional symptoms

Question 27

what are the earliest manifestations of renal tumor sometimes?

Answer 27

systemic symptoms not related to the kidney (paraneoplastic syndromes)

Question 28

in addition to fever and constitutional symptoms, renal cell carcinomas produce a number of syndromes including what?

Answer 28

polycythemia, anemia, hypercalcemia, hypertension, hepatic dysfunction, feminization or masculinization, cushing syndrome, eosinophilia

Question 29

what type of epithelium lines the renal pelvis?

Answer 29

urothelium

Question 30

when does urothelial carcinoma of the renal pelvis present?

Answer 30

usually early on because they produce noticeable hematuria

Question 31

what could urothelial carcinoma of the renal pelvis lead to?

Answer 31

palpable hydronephrosis and flank pain

Question 32

what is Wilms tumor?

Answer 32

the most common primary renal tumor of childhood (2-5 yo)

Question 33

germline deletions on what chromosome and what genes in WAGI?

Answer 33

11p13 (WT1 and PAX6)

Question 34

symptoms of Denys-Drash?

Answer 34

gonadal dysgenesis and early onset nephropathy leading to renal failure

Question 35

what is the characteristic glomerular lesion in patients with denys-drash?

Answer 35

diffuse mesangial sclerosis

Question 36

what are patients with denys-drash at increased risk of developing?

Answer 36

germ cell tumors called gonadoblastomas

Question 37

symptoms of beckwith-wiedemann syndrome?

Answer 37

abnormal large cells in the adrenal cortex, hemihypertrophy, organomegaly, macroglossia, omphalocele

Question 38

what is observed in the vast majority of wilms tumors?

Answer 38

the classic triphasic combination of blastemal, stromal, and epithelial cell types

Question 39

what characterizes the blastemal component of the wilm tumor?

Answer 39

sheets of small blue cells