Tumors of the Kidney and Urinary Tract – Flashcards
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Bladder cancer: mostly females or males? what age is commonly affected? What cell type is usually affected?
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Bladder cancer: mainly males mainly 50-80 yo usually epithelial tumors (*urothelial, squamous cell, adenocarcinoma, small cell)
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How does bladder cancer typically present?
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-painless hematuria -irritative symptoms (frequency, urgency, dysuria) -hydronephrosis (if ureteral orifice involved) -at presentation, most are
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Renal cancer - more commonly men or women? Older or younger pts?
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men; older
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What's the most common type of renal cancer?
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renal cell carcinoma
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What is the most important risk factor for both renal and bladder cancers?
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tobacco use
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Is schistosoma haematobium infection a risk factor for bladder or renal cancer?
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bladder
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How does bladder cancer often present? How about renal cancer?
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-Bladder - [presents Badder] - painless hematuria; can have irritative voiding; pelvic/suprapubic pain; pelvic mass. -Renal - often asymptomatic, found incidentally (50%); triad of flank pain/hematuria/mass only in 10%; paraneoplastic; often asymmetric
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What is the pneumonic for hematuria Ddx?
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Pee Pee ON TTTTHIS [Hey ya 1 2 3 4] Obstruction Nephritis Trauma Tumor TB Thrombosis (renal vein) Hematologic Infection/inflammation Stones
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Of the following, which are benign/malignant: renal cell carcinoma renal adenoma oncocytoma angiomyolipoma urothelial carcinoma metastatic tumor abscess infarct ateriovenous malformation pseudotumor
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Benign renal adenoma oncocytoma angiomyolipoma
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Likelihood of renal mass malignancy depends on ________
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size
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Can imaging reliably distinguish benign from malignant?
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NO! (unless intratumor fat - angiomyolipoma)
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What are the significant subtypes of renal cell carcinoma? What is the most common?
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-most common = clear cell carcinoma -papillary carcinoma -chromophobe carcinoma -CD carcinoma -Xp11 translocation carcinoma (mostly in young/ped pops - think young kids and their Xbox 11 listening to Ri Ri - renal cell carcinoma)
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What's the most common familial syndrome associated with renal cell carcinoma? What gene is mutated? What is the inheritance pattern? How does it present?
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Von Hipple Lindau Syndrome (classy name - gonna be classy cancer - renal not bladder) -autosomal dominant mutation of VHL gene on 3p25 -often leads to multifocal, bilateral, multiple renal cell carcinomas at early age [Others: hereditary leiomyomatosis & RCC syndrome; Birt-Hogg-Dube syndrome; Hereditary Papillary Carcinoma]
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Is the VHL gene an oncogene or tumor suppressor gene? How does it work?
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VHL is a tumor suppressor gene (VEHLL THEN = suppressive). It degrades the protein HIF-1alpha which induces VEGF, CA-IX -> formation of vasculature -> progression of tumor
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How does clear cell RCC usually present? Is it solitary? Unilateral? Sporadic?
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-usually solitary and unilateral -usually in kidney poles -usually sporadic -occurs at an earlier age if VHL
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Renal tumor workup
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-H + P - bp, skin, neuro -CT/MRI - evaluate mass, vessel involvement, contralateral kidney, regional lymph nodes, metastasis -CXR -CBC, BMP, Liver function/Alk Phos -look for paraneoplastics (think P close to R for renal rather than bladder); bone scan if elevated alk phos or bone pain
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How do you treat renal tumors? What's the gold standard?
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*Renal tumors are really *Resistant -; chemo and radiation are not effective -; SURG = GOLD STANDARD (radical ;4 cm or partial ;4 cm nephrectomy; cryosurgery; radiofrequency ablation)
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Do you often need biopsies for renal cancers?
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No! majority of solid masses are malignant
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When might you do a renal biopsy?
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if you suspect - lymphoma, bilat masses, multiple masses, diagnostic dilemmas, prior to focal ablative therapy, metastasis
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How does clear cell carcinoma appear grossly?
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[Be guided by the name - CLEAR cell carcinoma bc the cells are clear because of their glycogen and fat content] -circumscribed mass, sharp margins -bright yellow-orange because of lipids and glycogen -large areas of gray/white necrosis -hemorrhage common
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How does clear cell carcinoma appear histologically?
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-round cells with clear cytoplasm bc of glycogen and fat -delicate branching vasculature: chicken wire appearance (all these little clear cages for chickens) - separates tumor nests and tubules
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What's the grading system for renal cell carcinomas? What's it based on?
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-4-tiered system by Fuhrman -based on nuclear size, pleomorphism, nucleolar size;
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What's the staging system used for renal cell carcinomas? What's it based on?
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TNM Stage 1: cancer in kidney only and 7 cm Stage 3: spread to nearby tissue but not past kidney/adrenal Stage 4: metastasis
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Are collecting duct carcinomas malignant or benign? Are they common or rare? What type of carcinoma are they? What are some key features? Are they peripheral or central?
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Collecting duct carcinoma = -very aggressive -rare -an adenocarcinoma -; so the malignant cells form glands -prominent fibrotic stroma [I want to COLLECT you up in my glands!] -central
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What is the most common benign tumor of the kidney?
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angiomyolipoma
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How does fat show up on CT?
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black
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4cm)" alt="Angiomyolipoma: -What type of cancer may it grossly and radiographically mimic? -What is the tumor composed of? -what might it do to the vessels if it gets big (;4cm)">
Angiomyolipoma: -What type of cancer may it grossly and radiographically mimic? -What is the tumor composed of? -what might it do to the vessels if it gets big (;4cm)
4cm)" alt="Angiomyolipoma: -What type of cancer may it grossly and radiographically mimic? -What is the tumor composed of? -what might it do to the vessels if it gets big (;4cm)">answer
Again, use the name to guide you -can mimic RCC -tumor composed of vessels (angio), SM (myo), and fat (lipo) - >4cm -> selectively angioembolize -can hemorrhage extensively
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pics of angiomyolipoma
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creates thick walled vessels that are abnormally shaped
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Angiomyolipoma is often associated with what condition? What are the implications?
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-Angiomyolipoma found in 25-50% pts with tuberous sclerosis -> affects a lot of systems -> -cerebral cortex lesion -> epilepsy, mental retardation -skin abnormalities (sebacious cysts) -other benign tumors, especially in the heart [Think: lot of parts in the name -> can affect a lot of systems]
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If you feel an abdominal mass in kids, what would you think (renally)?
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Wilms Tumor! (lil Willy!) = most common kidney tumor of children, often 2-5 yo, presents as palpable mass
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Is Wilms Tumor usually unilateral or bilateral? How does management change for each situation?
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-usually unilateral -if unilateral -> surg followed by chemo and possibly radiation -if bilateral -> start with chemo (defies the surg first paradigm)
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What's are some of the genetic syndromes associated with Wilms Tumor? What gene is affected in Wilms tumor?
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1) WAGR syndrome = germline depletion of 11p13 on WT1 gene -Wilms Tumor -Aniridia (iris is not fully circumferential) -Genital anomalies -mental Retardation 2) Denys-Drash syndrome -gonadal dysgenesis, tumor -nephropathy -WT1 abnormalities (Those young Willy kids love to go to Dennys and Dash) 3) Beckwith-Wiedemann syndrome -organomegaly, macroglossia (large tongue) -hemihypertrophy -omphalocele -mutation in WT2 (BECKham and his wife - such a nice 2)
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How does Wilms tumor appear grossly?
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-well circumscribed, fleshy -"fish flesh" -may have prominent hemorrhage or necrosis [Something seems fishy...Willy have you been a naughty boy?!]
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Wilms tumor pathology - what are the three phases affected?
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-recapitulates developing kidney -3 phases = blastema (sheets of blue small cells); epithelium (abortive tubules and glomeruli); stroma (mostly fibrotic)
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What's the prognosis for Wilms tumor?
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-good! current cure rate 90%. But beware of secondary malignancy -5% of tumors have anaplasia aka poorly differentiated cells (large, hyperchromatic, pleomorphic nuclei and abnml mitosis) = bad prognostic sign
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Bladder cancer - evaluation
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-H + P -cystoscopy and adequate biopsy with bimanual exam (this is unique to bladder cancers!) -urine cytology -CT abd/pelvic/retrograde pyelogram (for upper tract and metastatic eval) -CXR -CBC, BMP, Liver fx/alk phos (bone scan if elevated alk phos or symptoms to indicate bony metastasis)
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What is bladder lined by (cells)?
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urothelium aka transitional epithelium; 3-8 layers of cells with top umbrella layer that covers a couple cells
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What are the most common types of bladder cancers?
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epithelial tumors -mainly urothelial -squamous cell -adenocarcinoma -small cell
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In addition to stage and grade, what in particular about bladder cancers is important to consider in evaluation?
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architecture! flat vs. papillary = 2 distinct precursor lesions
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How would you describe papillary architecture? Flat?
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-papillary = finger-like projections, cells radiate from a fibrovascular core; can progress to invasive carcinoma -flat = in situ carcinoma; almost always progresses to invasive carcinoma
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Which pattern - flat or papillary - is more common?
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-papillary is more common (think more projections -> more common) -
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Urothelial tumor staging
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TNM T - presence or absence of invasion; if it invades into the bladder wall muscle it's worse
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How do you treat bladder cancer? What do you need to determine first?
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First, determine if muscle invasive or not. -non-invasive - transurethral tumor resection +/- intravesical therapy (BCG, mitomycin, etc.) -Invasive: NEED multimodal treatment - cystectomy with urinary diversion +/- chemo -like renal cancer, surg moe effective than chemo/radiation
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Is metastatic bladder cancer curable?
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no!
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What is intravescial therapy? Why is it used?
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-intravescial therapy = chemo or immunotherapy -helps reduce relapse, eliminate residual tumor in certain kinds of bladder cancers (Ta, T1, CIS) since high recurrence and progression rates for non-muscular invasive bladder cancer
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Intravesical therapy: what's an example of immunotherapy? Chemotherapy?
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-immunotherapy = BCG -Chemo = mitomycin C
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What is mitomycin C? How does it work?
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mitomycin C is a chemotherapy. it's an alkylating agent -> interferes with DNA synthesis
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What is BCG?
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Bacillus Calmette Guerin vaccine -By binding fibronectin, BCG activates T cells which revs up the body's immune response to the cancer
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How is BCG administered?
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-induction -maintenance ^hard regimen to complete
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Neither metastatic renal nor bladder cancers are curable. Here are some treatment options
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In renal, rather than doing a nephrectomy, often give systemic therapy first rather than surg.
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Hematuria is _______ until proven otherwise
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cancer
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Grading for urothelial tumors
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WHO/ISUP 2004: papilloma, PUNLMP, low grade, high grade
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Following slides are from prework only - lower priority What are papillomas? Who do they usually affect?
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-papillary bladder tumor; before grade 1 even -small -affect young pts -almost never recur
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Papillary cortical adenoma Is it a kidney or bladder tumor? Benign or malignant? What's the usual size (size based definition)?
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kidney tumor benign usually size < 5 mm
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Renal oncocytoma -benign or malignant? -gross appearance?
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benign -circumscribed tumor, deep brown or mahogany and homogenous, can have central scar [ONCOcytoma - think of old rusty mahogany ANCHOR that's scarred]
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Renal oncocytoma - histo
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keeping with the red anchor - red eosinophilic cytoplasm -big cells - big anchors
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Papillary Renal cell carcinoma: -What is it? -how does it usually present (focally and laterally)?
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Papillary renal cell carcinoma -papillary (cells radiate from a fibrovascular core) tumor growing in a cystic space -cores may have foamy macrophages -often multifocal and bilateral -typically hemorrhagic and cystic
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Chromophobe RCC -prognosis compared to clear cell and papillary? -gross appearance? -histo
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Chromophobe RCC -excellent prognosis compared to clear cell and papillary (Think chromophobe germophobe I'm paranoid so I have good prognosis) -gross: tan circumscribed mass (I'm a chromophobe and I'm tan) -Histo: may mimic oncocytoma. Cells with eosinophilic cytoplasm and a halo around raisinoid nuclei (little germophone eating my raisins and staying anchored)
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Urothelial carcinoma of renal pelvis = a renal cancer -does it have symptoms? -better or worse prog than baldder urothelial carcinoma?
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Urothelial carcinoma of renal pelvis -has more symptoms than renal cell carcinomas -prognosis worse than bladder urothelial carcinoma - more commonly infiltartive
