Renal neoplasma Flashcards
1
Q
What is the difference between renal neoplasm and renal cell carcinoma?
A
- Renal neoplasm: Any tumor (benign or malignant) arising in the kidney, it can be benign (e.g., oncocytoma, angiomyolipoma) or malignant (e.g., RCC, Wilms tumor)
- Renal cell carcinoma: malignant renal neoplasm that originates from the renal tubules
2
Q
What are the different renal neoplasms?
A
- Benign
1) Angiomyolipoma
2) Oncocytoma
3) Papillary adenoma
- Malignant
1) Renal cell carcinoma
2) Wilms tumor
3
Q
What are the different renal cell cancer?
A
1) Clear cell RCC
2) Papillary RCC
3) Chromophobe RCC
4) Collecting duct (Bellini duct) carcinoma
5) Renal medullary carcinoma
4
Q
What is angiomyolipoma?
A
- Angio (blood), myo (smooth muscles), lipoma (fat) and these are the components of the tumor
- The majority of the cases are sporadic, while 20% of cases are associated with tuberous sclerosis (autosomal dominant gene mutation in the tumor suppressor genes “TSC1 & TSC2”)
- This mutation distorts a lot of organs in the patient and thus half of the patients will present with autism and epilepsy, and thus if a qs came that a patient has epilepsy and autism we must rule out tuberous sclerosis
- Grossly it is a well-defined encapsulated yellow-whitish mass, in the sporadic cases they are single and unilateral, however, in the cases with TS they are multiple and bilateral
- Microscopically it is triphasic (1. Thick-walled BV, 2. smooth muscle cells, and 3. adipose tissue)
5
Q
What is the cause of angiomyolipoma
A
1) Mainly sporadic
2) 20% TS (tuberous sclerosis) which is due to mutations in TSC1 and TSC2 (TSC “Tuberous sclerosis”), these genes are tumor suppressor genes
6
Q
Describe the gross picture of angiomyolipoma
A
- Well-defined
- Uncapsulated
- Yellow/whitish mass
- Single and unilateral in the sporadic cases
- Multiple and bilateral in the cases with Tuberous sclerosis
7
Q
Describe the microscopic photo of angiomyolipoma
A
- Triphasic tumor
- Thick-walled BV
- Smooth muscle cells
- Adipose tissue
8
Q
From the cystic lecture and till now patients on hemodialysis could end up with what?
A
1) Benign papillary adenoma
2) Acquired cystic disease
3) Malignant carcinoma
9
Q
What is papillary adenoma?
A
- Find in patients on hemodialysis
- It is a cortical papillary tissue smaller than 1.5 cm, it is found incidentally
- Size is VERY IMP here because the size is what determines if it’s malignant or benign, otherwise, it looks the same. If it’s bigger than 1.5 it’s malignant, if it’s smaller it’s benign.
10
Q
What is oncocytoma?
A
- Benign
- Cortical tumor
- Mahogany brown cut surface
- Central fibrous scar
- It has nests of tumor cells with eosinophilic abundant cytoplasm (distinctive red cytoplasm, which is due to the high number of mitochondria)
11
Q
What is the gross picture of oncocytoma?
A
Mahogany brown cut surface with a central fibrous scar
12
Q
Is oncocytoma benign or malignant?
A
Benign
13
Q
What are the microscopic photos of oncocytoma?
A
Nests of tumor cells with eosinophilic abundant cytoplasm
14
Q
When does renal cell carcinoma occur?
A
In the 6th-7th decade of life, and more in males
15
Q
What are the risk factors for renal cell carcinoma?
A
- Tobacco smoking
- Chronic renal failure
- Acquired cystic disease
16
Q
What are the types of renal cell carcinoma?
A
- Sporadic (most cases)
- Hereditary: Multifocal “multiple tumors in one kidney” and bilateral, it occurs at a younger age like (these genetic syndromes increases the risk of RCC):
1) Von Hippel-lindau (VHL) syndrome
2) Hereditary papillary carcinoma
3) Hereditary leiomyomatosis and RCC
4) Birt-Hogg-Dube syndrome
5) Tuberous sclerosis
17
Q
What is the most common clinical symptom that tells us that the patient has renal/bladder cancer?
A
Painless hematuria
18
Q
What is Von Hippel-Lindau syndrome?
A
- Mutation in the VHL gene (named after the disease)
- VHL inhibits hypoxia-inducing factors and thus if VHL is mutated the hypoxia-inducing facto will accumulate putting the cells in a hypoxic state, as a compensatory mechanism the body makes new blood vessels and thus we will ee hemangioblastoma in the cerebellum and the retina (tumors with BV)
- It affects the kidney, liver and pancreas (KLP “Dog”)
19
Q
What is hereditary papillary carcinoma?
A
- The tumor here is over 1.5 cm
- MET gene is mutated which is an oncogene
20
Q
What is hereditary leiomyomatosis and RCC?
A
- Leiomyomatosis is multiple tumors in the smooth muscle that bring with it papillary renal cell carcinoma
- Remember the 3Fs (mutation in the fumarate hydratase, it gives tumors known as fibroids in the uterus and so in Females “but it also affects the males)
21
Q
What is Birt-Hogg-Dubes syndrome?
A
- A type of renal cell carcinoma that is due to the mutation in the BHD gene
- The tumor is a hybrid oncocytic/chromophobe tumor
- It can cause skin lesions and spontaneous pneumothorax as it forms cysts in the lungs
22
Q
What are the different renal carcinomas?
A
- Adult:
- Cortical
1) Clear cell renal cell carcinoma
2) Papillary renal cell carcinoma
3) Chromophobe renal cell carcinoma
- Medullary (known to be very aggressive)
4) Collecting duct (Bellini duct) carcinoma
5) Renal medullary carcinoma
- Children
1) Wilm’s tumor (nephroblastoma)
23
Q
What is clear cell renal cell carcinoma?
A
- The most common type of renal cell carcinoma
- It arises from the PCT
- Most are sporadic, but some are familial; with a mutation in the VHL gene in both
- Grossly it is yellow to orange mass
- Microscopically it forms nests of clear cells (lipid+glycogen) which are separated by the vascular network
24
Q
What is the cause of clear cell renal cell carcinoma?
A
It can be sporadic or familial, but VHL gene mutation is found in both
25
From which part of the nephron does the clear cell renal cell carcinoma arise?
From the proximal convoluted tubule
26
What is the most common renal cell carcinoma?
Clear cell renal cell carcinoma
27
Describe the gross features of clear cell renal cell carcinoma
1. Yellowish to orange mass
2. Spherical tumor at the upper pole of the kidney
- It like a snake and it loves to invade the veins
28
Describe the microscopic photo of clear cell renal cell carcinoma
Nests of clear cells (lipid and glycogen) that are separated by a vascular network
29
What is papillary renal cell carcinoma?
- Arises from the distal convoluted tubule
- It can be familial or sporadic but in both mutations, the MET gene is seen
- Grossly there is a yellow to brown mass
- Microscopically the papillae are lined by epithelial cells, psammoma bodies, and foamy macrophages
30
From which part of the nephron does the papillary RCC arise?
The distal convoluted tubule
31
What is the gene mutation seen in papillary RCC?
MET gene mutation
32
What can we see microscopically in the papillary RCC?
1. Papillae are lined by epithelial cells
2. Psammoma bodies
3. Foamy macrophages
33
What is the chromophobe RCC?
- Arises from the intercalated cells of the renal collecting ducts
- It has the best prognosis
- It can be sporadic or familial but in both it is associated with the Birt-Hogg-Dube syndrome
- Grossly it forms a yellow-brown mass
- Microscopically we can see sheets of granular cells with prominent cell membranes and perinuclear halo
34
Where does the chromophobe RCC occur
In the cells of the collecting ducts
35
Which RCC has the best prognosis?
The Chromophobe RCC
36
What is the collecting duct (Bellini duct) carcinoma?
- Arises from the medullary collecting duct
- Grossly it has a medullary mass
- Microscopically it has tubules that are lined with atypical tumor cells
37
Which renal cell cancer arises from the medullary collecting duct?
The collecting duct (Bellini duct) carcinoma
38
What is the renal medullary carcinoma?
- Arises from the renal papillae
- It is associated with sickle cell disease in patients under 40 years old (only when the patient has sickle cell disease)
- Grossly we can see a medullary mass
- Microscopically the tubules are lined with atypical tumor
39
Renal medullary carcinoma occurs where in the kidney?
Renal papillae
40
In renal medullary carcinoma, what other disease must the patient have?
Sickle cell disease
41
What is the clinical picture of the renal cell carcinomas?
1. Dull loin pain (PAINLESS HEMATURIA & abdominal mass)
2. Some of the tumors secretes:
- Parathyroid-like hormone (leads to hypercalcemia)
- Erythropoietin (leads to polycythemia)
- Renin (leads to hypertension)
42
How does RCC spread?
1) Direct: to the renal pelvis, renal vein, and then adjacent tissues
2) Lymphatic: To the para-aortic lymph nodes
3) Blood: to the lungs and bones
43
Which cancer has a yellowish cut section?
Clear cell RCC
44
What is Wilm's tumor (Nephroblastoma)?
- It is the most common primary renal tumor during childhood (2-5 years)
- Pathogenesis:
1. Associated genetics are
- WAGR: Deletion of WT-1, the baby will have wilm's tumor + Aniridia (no iris) + mental retardation
- Denys-Drash: Dominant negative (mutation that formed a bad copy of WT-1 which suppresses the good/normal ones) WT-1 mutation, the child will suffer from Wilm's tumor + Glomerulosclerosis + Gonadal dysgenesis (testis/ovaries are not formed)
- Beckwith-Wiedemann syndrome: Mutation in WT-2, the child will suffer from Wilm's tumor + exophthalmos + macroglosia + gigantism (the mutation will result in the activation of IGF-2 of the mother "normally only the father one is active"
- They present clinically with an abdominal mass (big abdominal mass has two differential 1. Wilm's tumor 2. Neuroblastoma "a tumor that secretes catecholamines") and we measure serum catecholamines to differentiate between them
- Microscopically we will see:
1) The blastemal component would be sheets of small blue cells (small round blue cell tumors)
2) Epithelial component where the tubule/glomeruli are poorly formed
3) Spindle cells or myxoid tissue, skeletal muscle, fat, cartilage or bone are the stromal tissue
45
A patient comes in with RCC, hemangioblastoma in the brain, cysts in the kidney, liver & pancreas and he has pheochromocytoma what is this?
Von hippel landau so VHL genetic mutation
46
Patient with papillary RCC, multiple & bilateral?
Hereditary papillary RCC = ME
47
Patient with multiple fibroids in the kidney & uterus with RCC?
Hereditary leiomyomatosis RCC = FH
48
Patient with hybrid oncocytic with chromophobe RCC?
Bert Hogg Dube or tuberous sclerosis
49
What are the histopathological types of renal pelvic carcinoma?
1. Noninvasive urothelial (the epithelium) carcinoma:
1) Low-grade papillary urothelial carcinoma
2) High-grade papillary urothelial carcinoma
3) Urothelial carcinoma in situ
2. Invasive urothelial carcinoma:
1) Conventional urothelial carcinoma
2) Urothelial carcinoma variants
