B/2. Renal tumors Flashcards
BENIGN Renal tumors
- Angiomyolipoma
- Oncocytoma
Angiomyolipoma def
benign renal tumors that arise from perivascular epithelioid cells
and consist of:
* blood vessels,
* smooth muscle, and
* mature fat cells
most common benign renal tumor
angiomyolipoma
Epidemiology Angiomyolipoma
most common benign renal tumor,
F > M(4:1)
angiomyolipoma etiology
: sporadic
Angiomyolipoma symptoms
mostly asymptomatic;
large tumors can present with
* hematuria,
* retroperitoneal hemorrhage,
* impaired renal function
Angiomyolipoma Pathology, hows growth rate?
slow-growing;
epithelioid angiomyolipoma have a greater number of epithelioid
cells, acidophilic and granular cytoplasm, less fat than classic type
Angiomyolipoma diagnostics
- US
- CT
Angiomyolipoma treatment
Treatment: surgical resection if >4 cm
Oncocytoma def
benign epithelial tumor arising in the collecting duct from the intercalated tubular cells
Oncocytoma symptoms
- painless hematuria,
- abdominal mass,
- flank pain
Oncocytoma gross pathology
smooth, clearly defined brown tumor
with central radial scar;
large acidophilic cells,
excessive amount of mitochondria resulting in granular cytoplasm
without perinuclear clearing (unlike chromophobe RCC)
Oncocytoma diagnostics
- US
- CT
Oncocytoma treatment
often resected in order to exclude RCC
Oncocytoma prognosis
may transform into malignant oncocytic RCC
Oncocytoma prognosis
may transform into malignant oncocytic RCC
painless hematuria can be symptom of ?
Oncocytoma
MALIGNANT renal tumor
RENAL CELL CARCINOMA
RENAL CELL CARCINOMA epidemiology
gender
age
%
- Incidence increases continuously during the last years
- RCC accounts for >90% of malignant kidney neoplasms
- More common in males
- 50-70 years but can present at any age
what tumor accounts for >90% of malignant kidney neoplasms
RCC
RENAL CELL CARCINOMA more common in which sex
males (2:1)
peak age incidence of RCC
ages 50-70
but can present at any age
RENAL CELL CARCINOMA risk factors
- Smoking
- Renal cystic diseases
- end-stage kidney disease
- Von-Hippel-Lindau syndrome (AD mutation, tumor suppressor gene, located on chromosome 3);
bilateral RCC, hemangioblastoma, angiomatosis, pheochromocytoma
Von-Hippel-Lindau syndrome
- (AD mutation, tumor suppressor gene, located on chromosome 3);
bilateral RCC, hemangioblastoma, angiomatosis, pheochromocytoma
Von HIPPEL-Lindau syndrome:
Hemangioblastoma, Increased risk of renal cell carcinoma, Pheochromocytoma, Pancreatic lesions (cysts, cystadenomas, and neuroendocrine tumors), Eye Lesions (retinal angiomas or hemangioblastomas)
VHL normally inhibits
VHL normally inhibits HIF-1 alpha (Hypoxia Inducible Factor alpha ) > mutation results in increased activity of VEGF and PDGF pathways
RENAL CELL CARCINOMA clinical findings
- Up to 70% of cases are asymptomatic
- Classic triad: hematuria, abdominal mass, flank pain
- Constitutional: fever, weight loss, anemia
- Paraneoplastic syndromes:
*PTHrP > hypercalcemia
*EPO > erythrocytosis
*ACTH> cushing syndrome
*Renin > hypertension
How is RCC usually discovered?
- Up to 70% of cases are asymptomatic; most commonly detected as an incidental finding on radiograph or other abdominal imaging
List Paraneoplastic syndromes in RCC
- PTHrP > hypercalcemia
- EPO > erythrocytosis
- ACTH> cushing syndrome
- Renin > hypertension
classic triad in RCC
- hematuria,
- abdominal mass,
- flank pain
histopathological classification of RCC
- Clear cell RCC 60% Arises from PCT cells
- Papillary (chromophilic) RCC 5-15% Arises from PCT cells
- Chromophobe RCC 5-10% Indolent clinical course
- Oncocytic RCC 5-10% Considered benign neoplasm
- Collecting duct (Bellini) RCC < 1% Arises from medullary collecting ducts; aggressive
RCC Diagnostics
- The standard evaluation of patients with suspected renal cell tumor includes:
1. Renal US
2. CT scan of the abdomen and pelvis
3. CXR
4. Urinalysis
5. Urine cytology - If metastatic disease is suspected from the chest radiograph, a CT of the chest is warranted
- MRI is useful in evaluating the IVC in cases of suspected
tumor involvement or invasion by thrombus.
when is MRI useful in RCC
MRI is useful in evaluating the IVC in cases of suspected
tumor involvement or invasion by thrombus.
In clinical practice, any solid renal mass should be considered
malignant until proven otherwise
staging of RCC
T1a, T1b
T1a Tumor < 4 cm in largest diameter, confined to the kidney
T1b Tumor 4-7 cm in largest diameter, confined to the kidney
staging of RCC
T2
T2 Tumor > 7 cm in largest, confined to the kidney
staging of RCC
T3a, T3b, T3c
- T3a Tumor invades adrenal gland or perinephric tissue, but not beyond Gerota’s fascia
- T3b Tumor invades renal vein or IVC below diaphragm
- T3c Tumor invades IVC above diaphragm
staging of RCC
T4
T4 Tumor invades beyond Gerota’s fascia
metastasis of RCC
lymphatics,
lung,
bone,
brain,
liver
which LN get involved in RCC
renal hilar
lymph nodes
Treatment of RCC
T1, T2
- Radical nephrectomy en-bloc removal:
*of Gerota’s fascia including kidney,
*adrenal gland, and
*hilar lymph nodes - Nephron-sparing approach/Partial nephrectomy (laparoscopic or open surgery):
*appropriate choice if T1a
*or for patients who have only one functional kidney (depending on the size and location of the lesion)
when is nephron sparing approach/partial nephrectomy used in RCC
(laparoscopic or open surgery)
1. appropriate choice if T1a
or
2. for patients who have only one functional kidney (depending on the size and location of the lesion)
Advanced/
metastatic RCC
treatment
- Consider surgery to alleviate hematuria/pain from primary tumor, or cytoreductive nephrectomy before systemic treatment is initiated
- Targeted therapy
-Sunitinib, sorafenib, pazopanib, axitinib (oral TKI targetting VEGF and PDGF)
-Bevacizumab Anti-VEGF monoclonal Ab
-Everolimus, temserolimus (mTOR inhibitors, used if patient fails to respond
to TKI therapy - Immunotherapy
-Pembrolizumab, nivolumab (anti-PD1 monoclonal Ab)
-Ipilimumab (anti-CTLA4 monoclonal Ab)
-Cytokines : IL-2 (Aldesleukin), IFN-alpha; not commonly used nowadays
when to consider surgery in advanced metastatic RCC
Consider surgery to alleviate hematuria/pain from primary tumor,
or
cytoreductive nephrectomy before systemic treatment is initiated
