Renal Tumors B&B

Question 1

what is the most common kidney tumor and who does it affect most?

Answer 1

renal cell carcinoma: epithelial tumor commonly from PCT

risk factors: males, ages 50-70, cigarette smoking, obesity

Question 2

what is the classic triad of renal cell carcinoma?

Answer 2

renal cell carcinoma: epithelial tumor commonly from PCT

many patients asymptomatic until advanced disease, 25% have metastasized at presentation

—> hematuria, flank pain, palpable abdominal mass

Question 3

which vein is often invaded in renal cell carcinoma and what sign is indicative of this?

Answer 3

renal cell carcinoma: epithelial tumor commonly from PCT

invades renal vein - can block left spermatic vein (which drains into renal vein) —> left varicocele

[right spermatic vein drains directly into IVC so cannot be affected]

Question 4

what are the common metastasis sites of renal cell carcinoma?

Answer 4

renal cell carcinoma: epithelial tumor commonly from PCT

spreads via venous system —> lung, bone, retroperitoneal lymph nodes

Question 5

what are 4 common paraneoplastic syndromes of renal cell carcinoma?

Answer 5

renal cell carcinoma: epithelial tumor commonly from PCT, many paraneoplastic syndromes associated

—> polycythemia (EPO secreting)
—> hypercalcemia (PTH related protein secreting)
—> HTN (renin secreting)
—> Cushing’s syndrome (ACTH secreting) - weight gain, HTN, hyperglycemia

Question 6

how would a biopsy of renal cell carcinoma appear?

Answer 6

renal cell carcinoma: epithelial tumor commonly from PCT

most common type is clear cell carcinoma - cells appear clear because they are filled with glycogen and lipids

Question 7

what mutation is associated with renal cell carcinoma?

Answer 7

renal cell carcinoma: epithelial tumor commonly from PCT

associated with sporadic (older smoker) or inherited (younger, bilateral) gene deletion on chromosome 3 - Von-Hippel-Lindau (VHL)

AD VHL mutation causes many tumors - renal cell carcinoma, cerebellar hemangioblastoma, retinal hemangioblastoma

Question 8

who do renal tumor patients with a sporadic vs inherited deletion of Von-Hippel-Lindau (VHL) gene on chromosome 3 present?

Answer 8

VHL deletion —> renal cell carcinoma

sporadic: older patient, smoker, single tumor

inherited (AD): younger patient, multiple/ bilateral tumors… VHL deletion causes many tumors - renal cell carcinoma, cerebellar hemangioblastoma, retinal hemangioblastoma

Question 9

how is renal cell carcinoma treated?

Answer 9

renal cell carcinoma: epithelial tumor commonly from PCT, most common kidney tumor, many patients present in advanced stage

—> surgical resection if early, poor response to chemo/radiation
—> recombinant cytokines (Aldesleukin - Interleukin 2), cause symptoms of flu

Question 10

Pt is a 64yo M with PMH of cigarette smoking and BMI of 40, presenting with a left varicocele, hematuria, recent weight loss and 99 temp, and polycythemia. What are you most concerned about?

Answer 10

renal cell carcinoma: epithelial tumor commonly from PCT, most common kidney tumor

clues:
- obese male smoker aged 50-70 (spontaneous VHL mutation)
- left varicocele from blockage of spermatic vein (because of renal vein invasion)
- hematuria (classic triad also includes flank pain and palpable abdominal mass)
- weight loss + fever
- polycythemia - EPO secretion by tumor (paraneoplastic syndrome)

Question 11

Pt is a 33yo M presenting with fever and recent weight loss, as well as hematuria and flank pain. Pt complains of progressive SOB for past 3 months. PE remarkable for left varicocele and hypercalcemia. FHx significant for retinal hemangioblastoma. What are you most concerned about?

Answer 11

renal cell carcinoma: epithelial tumor commonly from PCT, most common kidney tumor

clues:
- triad: hematuria + flank pain + palpable abdominal mass
- fever + weight loss
- SOB indicates lung metastasis (25% have metastases at presentation, lung/bone are common sites)
- left varicocele via blockage of L spermatic vein (bc of renal vein invasion —> spreads via venous system)
- hypercalcemia via PTHrP secretion by tumor (paraneoplastic syndrome)
- FHx of retinal hemangioblastoma - indicates inherited deletion in VHL gene (causes many tumors including renal cell carcinoma and cerebellar/retinal hemangioblastoma)

Question 12

Pt is a 3yo F presenting with a large palpable flank mass, hematuria, and hypertension. What are you most concerned about?

Answer 12

Wilms’ Tumor: proliferation of metanephric blastema, most common renal malignancy in young children (avg. age 3)

associated with LOF of WT1 tumor suppressor (sporadic or part of syndrome)

HTN via renin secretion (paraneoplastic syndrome)

Question 13

WAGR Syndrome

Answer 13

WAGR syndrome: all related to deletion of WT1 gene chromosome 11 (tumor suppressor gene)

Wilms’ Tumor: most common renal malignancy in young children, proliferation of metanephric blastema

Aniridia: absence of iris, visual problems

Genital abnormalities: cryptorchidism, ambiguous genitalia

Retardation (mental)

Question 14

Beckwith Wiedemann Syndrome

Answer 14

pediatric overgrowth disorder
—> macrosomia (height/weight)
—> hemihyperplasia (muscles in one limb bigger than other)
—> macroglossia (tongue)
—> embyronal tumors - Wilms’ Tumor, neuroblastoma, rhabdomyosarcoma

Question 15

Pt is a 3yo M. Height and weight are noted to be in the 98th percentile. PE notes hemihyperplasia and macroglossia. Imaging is positive for a malignancy of the metanephric blastema. What is going on?

Answer 15

Beckwith Wiedemann Syndrome: pediatric overgrowth disorder
—> macrosomia (height/weight)
—> hemihyperplasia (muscles in one limb bigger than other)
—> macroglossia (tongue)
—> embyronal tumors - Wilms’ Tumor, neuroblastoma, rhabdomyosarcoma

Question 16

Renal angiomyolipoma

Answer 16

benign renal tumor of young children - blood vessels, smooth, muscle, fat

Associated with tuberous sclerosis: AD inheritance —> cortical tubers (brain), subependymal hamartomas, seizures, cardiac rhabdomyomas, “ash-leaf patches” (skin with no pigment)

Question 17

Pt is a 4yo F presenting with seizures and intellectual disability. PE notes leaf-like patches of skin with no pigment. Imaging shows an adipose renal tumor, as well as cortical tubers in the brain. What is likely going on?

Answer 17

Tuberous sclerosis: AD condition associated with

—> renal angiomyolipoma: benign renal tumor in young children of blood vessels/ smooth muscle/ fat
—> cortical tubers
—> subependymal hamartomas
—> seizures, mental retardation
—> cardiac rhabdomyomas
—> ash-leaf skin patches (no pigment)

Question 18

what is the most common tumor of the urinary tract system?

Answer 18

transitional cell carcinoma: usually in bladder, can also be in renal calyces/pelvis/ureters

often multifocal and recurrent - “field defect” of entire urothelium (separate tumors can be found in various locations)

Question 19

what risk factors are associated with transitional cell carcinoma?

Answer 19

transitional cell carcinoma: usually in bladder, can also be in renal calyces/pelvis/ureters, often multifocal and recurrent

risk factors:
- smoking
- cyclophosphamide
- phenacetin (old Tylenol formula)
- aniline dyes (hair dye)
- workplace exposures - rubber, textiles, leather, naphthalene (painters, machinists, printers)

Question 20

This cancer is most often found in the bladder, and is multifocal and recurrent due to “field defect.” Risk factors include smoking, naphthalene exposure, and cyclophosphamide (among others). What is?

Answer 20

transitional cell carcinoma: usually in bladder, can also be in renal calyces/pelvis/ureters, often multifocal and recurrent

most common urinary tract tumor

Question 21

Pt is a 71yo M smoker presenting with painless hematuria. No casts are found in the urine. What are you most concerned about? What test will you use to confirm the diagnosis, and how will you treat the presumptive diagnosis?

Answer 21

transitional cell carcinoma: usually in bladder, can also be in renal calyces/pelvis/ureters, often multifocal and recurrent, most common tumor of urinary tract

test of choice: cystosocpy and biopsy

treatment: surgical resection + radiation + chemotherapy with platinum-based drugs (cisplatin, carboplatin)

Question 22

what predisposing factor is needed for the development of squamous cell carcinoma of the bladder?

Answer 22

rare cancer, need chronic inflammation of the bladder

key risk factors: recurrent kidney stones or cystitis, or UTI with schistosoma haematobium

Question 23

with what urinary tract cancer is UTI with schistosoma haematobium associated?

Answer 23

schistosoma haematobium: trematode found in Africa/Middle East (Egypt), acquired from freshwater containing larvae —> usually cause hematuria

risk factor for squamous cell carcinoma (rare bladder cancer)

Question 24

in what circumstances does adenocarcinoma of the bladder occur? (3)

Answer 24

adenocarcinoma: very rare bladder cancer due to glandular proliferation

occurs in special circumstances:
- urachal remnant
- long history of cystitis
- exstrophy (external bladder)

Question 25

what type of very rare bladder cancer is associated with urachal remnants and exstrophy?

Answer 25

adenocarcinoma: glandular proliferation in bladder (very rare)