Renal Pathology 8: Renal Neoplasms (Dobson)

Question 1

Papillary adenomas

Answer 1

histologically do NOT differ from low-grade papillary renal cell carcinoma and share some immunohistochemical and cytogenetic features (trisomies 7 and 17)

Question 2

“mahogany brown” cut surface and central scar

Answer 2

Oncocytoma; composed of eosinophilic cells with small, round, benign-looking nuclei, comes from the intercalated cells of the collecting duct; can cause compression symptoms

Question 3

Renal cell carcinomas

Answer 3

more common in male older adults (60-70yrs); cigarette smoking is a risk factor; more commonly affect the poles of the kidney; presents with triad of symptoms; appears as yellow mass; paraneoplasms may be present

Question 4

Von Hippel-Lindau (VHL) syndrome

Answer 4

AD disorder; develop renal cysts and are bilateral and multiple; increased risk for hemangioblastoma of the cerebellum

Question 5

What is the most common type of renal cell carcinoma?

Answer 5

Clear cell type; most cases sporadic; loss of sequence in short arm of chromosome 3 (del 3p25.3)

Question 6

Papillary carcinoma

Answer 6

characterized by a papillary growth pattern; not associated with 3p deletions (unlike clear cell carcinoma); most common abn. is trisomies 7 and 17; Psammoma bodies may be present

Question 7

Chromophobe carcinoma

Answer 7

composed of cells with prominent cell membranes and pale eosinophilic cytoplasm, usually with a halo around the nucleus; will show multiple chromosomal losses and extreme hypodiploidy on cytogenic examination

Question 8

Xp11 translocation carcinoma

Answer 8

young patients; translocation of TFE3 gene located on Xp11.2

Question 9

Clinical presentation of renal cell carcinoma

Answer 9

Triad: flank pain, palpable mass, and hematuria; can also present with fever, weight loss and malaise

Question 10

Wilms tumor

Answer 10

Nephroblastoma; most common renal tumor of childhood; avg age is 3 yrs (kidney mass in child); comprised of blastema; WT1 or WT2 mutation on chromosome 11

Question 11

Triad of symptoms for renal cell carcinomas

Answer 11

Hematuria*, palpable mass and flank pain

Question 12

What paraneoplasms may be present with renal cell carcinoma?

Answer 12

EPO - lead to polycythemia
renin - lead to HTN
PTHrP - lead to hypercalcemia
ACTH - lead to Cushing’s

Question 13

Staging of renal cell carcinoma

Answer 13

T - based on tumor size and involvement of renal vein ( loves to go to renal vein)
N - spread to retroperitoneal* lymph nodes

Question 14

Wilms tumor is mostly comprised of what?

Answer 14

blastema (metanephric blastema - embryonic structure that gives rise to kidney)

Question 15

WAGR syndrome

Answer 15

Wilms
Anirida
Genital abnormality
Retardation (motor and mental)

Question 16

Beckwith-Wiedemann Syndrome

Answer 16

Wilms, neonatal hypoglycemia, muscular hemihypertrophy, organomegaly (tongue) think Beckwith-WIDEmann

Question 17

Risk factors for renal cell carcinoma

Answer 17

HTN, Obesity and Cigarette smoking

Question 18

Clear Cell Carcinoma

Answer 18

most common renal cell carcinoma; aggressive!! dysfunction VHL tumor suppressor gene; “2 hit” model; increased risk for hemangioblastomas of the cerebellum

Question 19

Which cancer increases the risk of hemangioblastomas of the cerebellum?

Answer 19

Clear Cell Carcinoma; VHL syndrome

Question 20

Cancer where Psammoma bodies may be present?

Answer 20

Papillary carcinoma

Question 21

Angiomyolipoma

Answer 21

Hamartoma; benign neoplasm of vessels, smooth muscle and fat; associated with Tuberous sclerosis; characterized by lesions of the cerebral cortex that produce epilepsy/retardation, skin problems and benign tumors

Question 22

Oncocytoma

Answer 22

comes from the intercalated cells of the collecting duct; can cause compression symptoms; “mahogany brown” cut surface and central scar

Question 23

Which cancer is associated with dialysis-associated cystic disease?

Answer 23

Papillary carcinoma; foam cells are common in the core