L16- Renal Cancer Flashcards
What are the 3 malignant and 4 benign renal tumors we are going to review in this notecard set?
Malignant: RCC (most common), Urothelial Carcinoma, Nephroblastoma (Wilms - kids)
Benign: Adenoma, Oncocytoma, Angiomyolipoma, Simple cyst
What are the different subtypes of Renal Cell Carcinoma?
Clear cell - 75%
Papillary 15%
Chromophone <5%
Sarcomatoid <5%
See picture attached!
Who gets RCC? What are Risk Factors? What are symptoms/Classic Triad?
Epidemiology: Older people, MEN!
Risk Factors: SMOKING, obesity, HTN, estrogen therapy, asbestos/exposures, CRF or Acquired PKD, and Von Hippel Lindau Syndrome w/ Clear Cell
Symptoms: Classic Triad: CVA Tendernes, Hematuria, Flank Mass
Often no pain and incidentaloma found on CT scan
_Paraneoplastic Syndromes: _
- Hypercalcemia - PTH-like hormone - most common
- Polycythemia - EPO
- HTN from renin
- Cushings from ACTH
- Eosinophilia, Lukemoid reactions
What are features of Von Hippel Lindau Syndrome? what causes it?
RCC - bilateral and multiple
Retinal Angiomatosis
Cerebellar Hemangioblastoma
Adenomas and cysts throughout the body
Caused by mutations in VHL Tumor Suppressor Gene on 3p25
Clear Cell RCC: Gross appearance? Histology? and Genetics?
Gross: Bright yellow/white/grady solid and well-demarcated lobular mass w/ lipid and glycogen in cells - can see necrosis and hemorrhage too
Histo: Polygonal cells w/ Abundant clear to eosinophilic granular cytoplasm –> CLEAR CELLS THAT FORM TUBULAR STRUCTURES
Genetics: Mutation/loss of VHL gene 3p25 that encodes a protein that functinos in degradation Hypoxia Inducible Factor 1 HIF1 –> HIF1 elevated then increased VEGF and TGFbeta and IGF
Papillary Cell Carcinoma: Grossly? Histology? Genetics?
Gross: BIG, encapsulated and eccentrically located in cortex, bilateral (vs clear cell unliateral)
- tiny yellow specks from histiocytes
- Reddish brown or tan appearance (vs yellow in clear cell)
Histology: Eosinophilic cuboidal/columnar cells arranged on **Fibrovascular stalks **
- **Lipid-laden Macrophages (Foam Cells) **
- Psammoma bodies w/ basophilic color
Genetics: Sporadic trisomy 7, 16, 17, and loss of Y in males
Hereditary Papillary RCC: Germ-line mutation in C-Met gene 7q31
Chromophobe RCC: Gross? Histo? Genetics?
Gross: well-circumscirbed slightly lobulated large mass that is pale yellow, tan or brown
Histology: cells w/ prominent membranes - Plant Cell Like and PALE (but not clear) to pink cytoplasm and halo around nucleus
**EM: microvessels around nucleus **
Genetics: Hypodiploidy
What is Sarcomatoid RCC?
Poorly differentiated spindle cell component arises in context of other cell types and when you see it then automatically MUCH worse pronosis
Usually means already metastatic and survival <1 year
What is a danger w/ RCC to the heart?
All RCC have propensity to invade renal vein or perinephric fat invasion which decreases survival bc tumor can climb into IVC and into heart!
Prognosis and treatment of RCC?
Stage of disease most important prognostic factor!!!
Tend to metastasize to Lung and Bone and then liver, adrenals
TX = NEPHRECTOMY
or partial nephrectomy
or cryotherapy for ablation
What is a random RF for Urothelial carcinoma of kidney?
Analgesic Nephropathy
Urothelial carcinoma of kidney - how does it occur? Symotoms?
Histo?
Arises from urothelium of Renal Pelvis and grows into kidney
Symp: Hematuria and Hydronephrosis
Histo: thin walled papillary w/ multilayered epithelium
Wilms Tumor: Presentation? Gross? Histo?
Presentation: most common primary renal tumor of kids, ages 2-5 yo
Palpable Mass, Hematuria, Intestinal obstruction, HTN
Gross: Solitary, spherical sharply demarcated pale tan to gray
some bilateral
Histology: **Triphasic Morphology: **
- Blastema - sheets of small blue cells
- Epithelial - abortive tubules and glomeruli formation
- stroma - fibrous, skeletal, fat, cartilage bone
Can also see Anaplasia and Large, Hyperchromatic, pleomoprhic nuclei
What are precursor lesions for Wilm’s Tumor?
What are genetics?
Precursor Lesion = Nephrogenic Rests - small focl of persistient primitve blastemal cells
- signal increased risk for developing Wilms tumor in contralateral kidney!!!
Genetics = mutations/deletions in Chromosome 11 (WT1 and WT2 genes)
ASsociated w/ Syndromes - WAGR, Beckwith-Wiedemann etc
PRognosis and treatment of Wilms tymor?
Pulmonary mets common at presentation
good prognosis
2 year survival implies cures bc low recurrence after that
Tumors w/ diffuse anaplasia have least favorable outcomes
Treatment: Surgery and CTX
