Renal neoplasms

Question 1

Benign kidney tumors

Answer 1

Renal papillary adenoma
Angiomyolipoma
Oncocytoma

Question 2

Malignant kidney tumors

Answer 2

Renal cell carcinoma
Transitional cell carcinoma of renal pelvis
Wilms tumor/nephroblastoma
Adrenal neuroblastoma

Question 3

Benign kidney tumor that grossly appears as small cortical pale yellow-gray nodules (<0.5 cm in diameter) that arises from tubular epithelium

Answer 3

Renal papillary adenoma

Question 4

Microscopy of kidney lesion shows complex, branching papillary process

Answer 4

Renal papillary adenoma or renal cell adenocarcinoma –> hard to differentiate

Question 5

Benign tumor of kidney with blood vessels, smooth muscle, and fat. Present in 25-50% of tuberous sclerosis due to loss of function in tumor suppressor gene, TSC1 and TSC2

Answer 5

Angiomyolipoma

Question 6

Syndrome characterized by hamartomatous lesions in the cerebral cortex causing epilepsy and mental retardation, skin lesions (Shagreen patches and ash leaf patches), and other benign tumors (like rhabdomyoma of the heart).

Answer 6

Tuberous sclerosis

Question 7

Benign renal tumor that is clinically important due to tendency to bleed spontaneously

Answer 7

Angiomyolipoma

Question 8

Gross appearance of renal angiomyolipoma

Answer 8

Large, yellowish, and firm tumor

Question 9

Gross morphology of renal oncocytoma

Answer 9

Tan or mahogany brownish colored lesion with central scar

Question 10

Benign kidney tumor that arises from intercalated cells of collecting ducts. Large pink cells on microscopy. Electron microscopy shows cells rich in mitochondria.

Answer 10

Oncocytoma

Question 11

Description of oncocytes

Answer 11

Large cells with are very pink

Question 12

Exposure that has a strong association with renal cell carcinoma

Answer 12

Cigarette smoking

Question 13

Familial variants (4%) of renal cell carcinoma

Answer 13

Von Hippel-Lindau disease
Hereditary/familial clear cell carcinoma
Hereditary papillary carcinoma

Question 14

Normal function of VHL gene

Answer 14

Downregulates hypoxia inducing factor-1 –> decreased production of VEGF, PDGF, and IGF-1

Question 15

Result of abnormal VHL gene in Von Hippel-Lindau disease

Answer 15

Increased cell growth and angiogenesis

Question 16

Manifestations of Von Hippel-Lindau disease not seen in hereditary/familial clear cell carcinoma

Answer 16

Hemangioblastomas of cerebellum, cysts, and multiple RCC, other than renal

Question 17

Mutation in hereditary papillary carcinoma

Answer 17

MET proto-oncogene resulting in multiple bilateral kidney tumors

Question 18

Inheritance of hereditary papillary carcinoma

Answer 18

Autosomal dominant

Question 19

Gene involved in 98% of all clear cell carcinomas, both familial and sporadic

Answer 19

VHL gene on chromosome 3p

Question 20

What is also regulated by VHL gene, other than HIF-1?

Answer 20

Expression of EPO

Question 21

Classifications of renal cell carcinoma

Answer 21

Clear cell carcinoma
Papillary carcinoma
Chromophobe renal cell carcinoma (great prognosis)
Collecting/Bellini duct carcinoma

Question 22

Renal tumor with a propensity to invade the renal V to spread to IVC and right heart

Answer 22

Renal cell carcinoma

Question 23

Spherical, bright yellow or gray-white, kidney lesion with sharply defined margins on upper or lower pole. Accompanied by hemorrhage and necrosis.

Answer 23

Renal cell carcinoma

Question 24

Microscopic features of clear cell renal carcinoma

Answer 24

Clear cells in nests, cords, and sheets with PAS positive cytoplasm filled with lipid and glycogen and delicate branching vasculature.

Question 25

Microscopic features of renal papillary carcinoma

Answer 25

Papillae with foam cells in the fibrovascular cores Psammoma bodies may also be present

Question 26

Classic triad of clinical features of renal cell carcinoma seen in 10%

Answer 26

Costovertebral pain Palpable mass Hematuria

Question 27

Paraneoplastic syndromes associated with renal cell carcinoma

Answer 27

Polycythemia Hypercalcemia HTN Hepatic dysfunction Cushing syndrome Eosinophilia Leukemoid reaction Amyloidosis Feminization or masculinization

Question 28

Associated with increased incidence of transitional cell carcinoma of renal pelvis

Answer 28

Balkan nephropathy Analgesic nephropathy

Question 29

Papillary, nodular, or flat kidney lesion. Microscopy shows delicate papillae lined by malignant urothelial cells (>7 thick). May be low or high grade depending on mitosis, polarity, or pleomorphism.

Answer 29

Transitional cell carcinoma of renal pelvis

Question 30

Why does TCC of renal pelvis become symptomatic early?

Answer 30

Due to its location

Question 31

Symptoms of TCC of renal pelvis

Answer 31

Hematuria Obstruction causing palpable hydronephrosis and flank pain

Question 32

Concomitant tumor seen with TCC of renal pelvis

Answer 32

Bladder tumor

Question 33

Four groups of congenital malformations associated with Wilms tumor

Answer 33

WAGR syndrome Denys-Drash syndrome Beckwith-Weidemann syndrome Mutations in gene encoding beta-catenin

Question 34

Syndrome associated with germline deletion of 11p13 (WT1)

Answer 34

WAGR syndrome

Question 35

Features of WAGR syndrome

Answer 35

Wilms tumor Aniridia Genital abnormalities Mental retardation

Question 36

Mutation associated with Denys-Drash syndrome

Answer 36

Germline abnormalities in WT1 affecting DNA-binding properties

Question 37

Features of Denys-Drash syndrome

Answer 37

Gonadal dysgenesis Early onset nephropathy --> diffuse mesangial sclerosis

Question 38

Syndrome associated with Wilms tumor and increased risk for gonadoblastoma

Answer 38

Denys-Drash syndrome

Question 39

Protein critical for normal renal and gonadal development that is associated with Wilms tumor when mutated

Answer 39

WT1

Question 40

Syndrome associated with non-classical example of tumorigenesis involving WT2 on chromosome 11p15.5

Answer 40

Beckwith-Weidemann syndrome

Question 41

Characteristics of Beckwith-Weidemann syndrome

Answer 41

Organomegaly Macroglossia Risk for other tumors

Question 42

Pathway affected by beta-catenin mutation in Wilms tumor

Answer 42

WNT pathway

Question 43

Precursor lesion of Wilms tumor

Answer 43

Nephroblastoma

Question 44

Nephrogenic rests that share genetic alterations with Wilms tumor and are considered pre-neoplastic. When present, increase risk of development of Wilms tumor in contralateral kidney

Answer 44

Nephroblastoma

Question 45

Large, solitary, well-circumscribed renal mass that is soft and tan-gray, with a foci of hemorrhage and necrosis. May also have cysts.

Answer 45

Wilms tumor

Question 46

Classic triphasic microscopic features of Wilms tumor

Answer 46

Epithelial - abortive glomeruli and tubules Blastemal - small blue cells Stromal - spindle cells and myxoid areas

Question 47

Describe anaplasia, sometimes seen in Wilms tumor

Answer 47

Large hyperchromatic pleomorphic nuclei with abnormal mitosis due to p53 mutation

Question 48

Significance of presence of anaplasia in Wilms tumor

Answer 48

Resistant to chemotherapy

Question 49

Features associated with poor prognosis in Wilms tumor

Answer 49

Anaplasia Loss of genetic material on chromosomes 11q and 16q Gain in chromosome 1q

Question 50

Malignancies with increased risk after radiation for Wilms tumor

Answer 50

Bone and soft tissue sarcomas Leukemias and lymphomas Breast cancer

Question 51

Tumor composed of small, primitive appearing cells that have dark nuclei and scant cytoplasm, with poorly defined cell borders growing in sheets with pleomorphism. Background is often faintly eosinophilic fibrillar material. Arranged in Homer-Wright pseudorosettes

Answer 51

Adrenal neuroblastoma

Question 52

Metabolites in the synthesis of epinephrine that are often elevated in urine with neuroblastoma, ganglioneuroblastoma, ganglioneuroma, and pheochromocytoma.

Answer 52

VMA and HVA

Question 53

What is a sign of poor prognosis in adrenal neuroblastoma?

Answer 53

Absence of VMA production