Neoplasms

Question 1

Answer 1

Renal Papillary Adenoma

Question 2

Answer 2

Angiomyolipoma

Question 3

Deletions on Chromosome 3

Loss of VHL

Inactivated Mutated VHL

Hypermethylation of VHL

Answer 3

Sporadic Clear Cell

Question 4

Deletions on Chromosome 3

Loss of VHL

Inactivated Mutated VHL

Hypermethylation of VHL

Answer 4

Hereditary Clear cell

Question 5

Trisomy 7

Mutated Activated MET

Answer 5

Hereditary Papillary

Question 6

Trisomy 7, 17

Loss of Y

Mutated activated MET

Answer 6

Sporadic Papillary

Question 7

Answer 7

Clear Cell

• Cells with lipid-rich ample cytoplasm.

Question 8

Answer 8

Type 1 Papillary

• Small basophilic cells with scarce cytoplasm, organized in a spindle- shaped pattern, in a single layer of cells surrounding the basal membrane.

Question 9

Answer 9

Renal cell carcinoma, gross.

• Location: lower pole.
• Circumscribed
• Hemorrhagic red and cystic areas.

Question 10

Answer 10

Papillary Renal cell carcinoma, gross.

• Cystic with extensive hemorrhage

Question 11

Answer 11

Renal cell carcinoma, microscopic

Question 12

Answer 12

Large Papillary Tumor

• Papillary pattern
• Circumscribed.

Question 13

Answer 13

High-grade invasive transitional cell carcinoma.
• Ulceration and necrosis.

Question 14

Autosomal dominant hereditary neoplastic disorder characterized by the development of tumors in the cerebellum, spine, retina, inner ear, pancreas, adrenal glands, and kidneys).

Answer 14

Von Hippel Lindau

Question 15

Renal Papilary Adenoma, Angiomyolipoma, Oncocytoma

Answer 15

Benign Neoplasms

Question 16

Clear Cell Carcinoma, Papilary Renal Carcinoma, Chromophobe

Answer 16

Malignant Neoplasms

Question 17

Small,discrete adenomas arising from the renal tubular epithelium (more frequent papillary)

Answer 17

Renal Papillary Adenoma

Question 18

These are small tumors (usuallyl ess than 0.5cm in diameter)

They are present invariably within the cortex and appear grossly as pale yellow-gray discrete, well-circumscribed nodules.

Answer 18

Renal Papillary Adenoma

Question 19

• This is a benign neoplasm consisting of vessels, smooth

muscle, and fat originating from perivascular epithelioid cells.

• Present in 25% to 50% of patients with tuberous sclerosis (disease caused by loss-of-function mutations in the TSC1 or TSC2)
• linical importance: susceptibility to spontaneous hemorrhage.

Answer 19

Angiomyolipoma

Question 20

• Epithelial neoplasm composed of large eosinophilic cells.
• Arise from the intercalated cells of collecting ducts.
• Accounts for approximately 5% to 15% of renal neoplasms.
• They may achieve a large size (up to 12 cm in diameter).

Answer 20

Oncocytoma

Question 21

• Account for 85% of renal cancers in adults (3% newly diagnosed)
• The tumors occur most often in older individuals (usually in the sixth and seventh decades of life).
• 2 : 1 male preponderance

Answer 21

Renal Cell Carinoma

Question 22

• Risk Factors:
• Tobacco is the most significant risk factor.
• Obesity (particularly in women)
• Hypertension
• Unopposed estrogen therapy
• Exposure to asbestos
• Petroleum products
• Heavy metals.
• Chronic kidney disease, acquired polycystic kidney disease and tuberous sclerosis.

Answer 22

Renal Cell Carcinoma

Question 23

• Most common 70% to 80% of renal cell cancers.
• 95% are sporadic.
• They arise from the proximal tubule
• Specifically Associated with Von Hippel Lindau syndrome.

Answer 23

Clear Cell Carcinoma

Question 24

Autosomal dominant hereditary neoplastic disorder characterized by the development of tumors in the cerebellum, spine, retina, inner ear, pancreas, adrenal glands, and kidneys).

• Deleted region harbors VHL genes.

Answer 24

Von Hippel Lindau Syndrome

Question 25

- Accounts for 10% to 15% of renal cancers. - It is characterized by a papillary growth pattern and occurs in both familial and sporadic forms. - The most common cytogenetic abnormalities are trisomies 7 and 17. - Mutations in MET

Answer 25

Papillary Carcinoma

Question 26

- Represents 5% of renal cell cancers and is composed of cells with prominent cell membranes and pale eosinophilic cytoplasm. - Grow from intercalated cells collecting ducts (good prognosis)

Answer 26

Chromophobe Carcinomas

Question 27

- May arise in any portion of the kidney, but more commonly affects the poles.

Answer 27

Renal Cell Carcinomas

Question 28

* Most likely arise from proximal tubular epithelium, and usually occur as solitary unilateral lesions. * They are bright yellow-gray white spherical masses * Variable size. * The margins are usually sharply defined and confined within the renal capsule.

Answer 28

Clear Cell Carcinomas

Question 29

- Only 10% of cases - **Costovertebral pain, Hematuria, Palpable mass** - Most reliable clue: **Hematuria Intermittent** - Microscopic; thus, the tumor may remain silent until it attains a large size, often greater than 10 cm. - Associated with generalized constitutional symptoms: fever, malaise, weakness, and weight loss.

Answer 29

Renal Cell Carcinoma

Question 30

* **Polycytemia** from elaboration of erythropoietin. * **Hypercalcemia** with tumor production of parathormone-related peptide. * **Steroid hormone release** with Cushing syndrome, feminization, or masculinization.

Answer 30

Paraneoplastic Syndromes Related To Renal Cell Carcinomas

Question 31

What are the most common locations of methastasis?

Answer 31

1. **Lungs (50%)** 2. **Bones (33%)** 3. Regional lymph nodes, liver, adrenal, and brain.

Question 32

Treatment of choice to Renal Cell Carcinomas?

Answer 32

**- Radical nephrectomy** - Partial nephroctomy if tumor is smaller than 4 cm

Question 33

* Most common renal malignancy in children. * Age 2- 4 * Deletion of WT1 or WT2 chromosome 11 * Clinical Presentation: * Flank mass * Hematuria * Abdominal pain, fever (less common).

Answer 33

Wilms Tumor

Question 34

- 90% of all bladder tumors. 􏰁 - Multifocal at presentation. * *􏰁 Two Precursor lesions:** - Noninvasive papillary tumors (Originate from urothelial hyperplasia). - Flat noninvasive urothelial carcinoma. (Carcinoma in Situ or CIS).

Answer 34

Urothelial/Transitional Tumors

Question 35

- Incidence is higher in men than in women. 3:1 - Higher in developed than in developing nations and in urban than in rural dwellers. - 50 and 80 years of age (not familial, rare exceptions). **Risk Factors:** - Cigarette smoking (the most important). - Industrial exposure to aryl amines (2 naphthylamine) - Schistosoma haematobium infections in endemic areas (Egypt, Sudan). - Chronic inflammatory response - Mucosal squamous metaplasia - Long-term use of analgesics - Heavy long-term exposure to cyclophosphamide, - Radiation.

Answer 35

Urothelial/Transitional Tumors

Question 36

Name the sites to where the aggressive bladders tumors can extend?

Answer 36

Bladder Wall Adjacent Prostate Seminal Vesicles Ureters Retroperitoneum

Question 37

- Painless Hematuria - Frequency, urgency, and dysuria occasionally accompany the hematuria. - When the ureteral orifice is involved, pyelonephritis or hydronephrosis may follow.

Answer 37

Clinical Manifestations of Bladders Tumors

Question 38

What are the prognosis of Bladder Tumors?

Answer 38

- 98% 10 year survival rate - Invasive urothelial carcinoma is associated with a 30% mortality rate once tumor invades into the lamina propria.