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Lecture 7
Urinary Tract Neoplasm
| Question | Answer |
|---|---|
| If you see a word refering to a disease and it ends in -oma what do you know about that disease | it implies that the disease is benign |
| What renal tumor is grossly brown-orange and sometimes has a central scar | Renal oncocytoma |
| This tumor is small (<.5cm) well circumscribed, pale, and found in the middle of the medullary pyramids | Medullary fibroma |
| How is an angiomyolipoma diagnosed | on imaging |
| Why don't we resect every angiomyolipoma | risk of hemorrhage |
| What tumor is grossly yellow with areas of hemorrhage (benign) | Angiomyolipoma |
| Angiomyolipoma is difficult to distinguish from? | renal cell carcinoma of the clear cell type |
| What is an angiomyolipoma composed of microscopically | adipose tissue, smooth muscle and thick walled vessels |
| Angiomyolipomas ave a strong association with? | Tuberous sclerosis (80% of patients with TS have angiomyolipomas) |
| When is mesoblastic nephroma usually picked up? | in 1st 3 months of life |
| what benign tumor is grossly not very distinct and quite variable in size (1cm to >15cm) | Mesoblastic Nephroma |
| What tumor is microscopically composed of fusiform cells differentiating towards fibroblast or myofibroblasts | Mesoblastic Nephroma |
| Nephroblastoma (not benign) is also referred to as? | Wilms Tumor |
| What are the 3 embryonal elements the compose a wilms tumor | Blastema, Stroma, epithelial |
| When and how does Wilms tumor present | large abdominal mass from 1-3 years of age |
| Wilms tumor is very uncommon after what age | 10 |
| What is the gross appearance and microscopic composition of a Wilms tumor | pale tan on cut surface and microscopically usually a mixture of the 3 embryonal elements |
| what are the congenital syndromes associated with the 5% of Wilms tumors that are not sporadic | WAGR syndrome, Denys-Drash syndrome, or Beckwith-Wiedeman syndrome |
| what gene is responsible for all wilms tumors in the context of WAGR syndrom and 10% of sporadic Wilms Tumors | WT1 tumor suppressor gene |
| what is the most common malignant renal cell tumor in adults | Renal Cell Carcinoma (>11,000 cases / year) |
| about 1/3 of renal cell carcinomas are associated with? | tobacco use |
| what are the syndromes/diseases associated with 5% of inherited Renal Cell Carcinomas | AD renal cell carcinoma syndrome, von Hippel-Lindau disease, hereditary papllary reanl cell carcinoma syndrome |
| Loss of 1 allele of what gene on what chromosome is responsible for the vast majority of sporadic conventional (clear cell) renal cell carcinomas | Loss of one allele of the VHL gene on chromosome 3p |
| what are the 4 main types of renal cell carcinoma | Conventional (clear cell), Papillary, Chromophobe, and Collecting duct carcinoma |
| Prognosis of Clear cell (conventional) renal cell carcinoma is based on? | stage and grade |
| Does papillary renal cell carcinoma have a better or worse prognosis than Conventional clear cell renal carcinoma | for the most part better |
| What renal cell carcinoma stains a gorgeous turquoise color with Hale's colloid iron? | Chromophobe renal cell carcinoma |
| What renal cell carcinoma has a hobnail configuration in a dense stroma | Collecting duct carcinoma |
| what is the most common (70%) renal cell carcinoma | Conventional (Clear cell) type |
| where do Urothelial (transitional cell) carcinomas arise | in the urothlium of the renal pelvis (morphologically identical to the urothelial carcinomas of the bladder) |