B5-081 Renal and Urinary Tumors Flashcards
biggest risk factor for renal cell carcinoma
smoking
familial variants of renal cell carcinoma
- von Hippel Lindau
- Hereditary papillary carcinoma
often bilateral
most common renal cell carcinoma
clear cell
clear cell carcinoma
98% of clear cell RCC is caused by an abnormality on chromosome
3
loss of VHL tumor suppressor
most common subtype of RCC found in dialysis patients
papillary carcinoma
- papillary growth with interstitial foam cells
- multifocal
papillary carcinoma
associated with trisomy 7 and 17, loss of Y
papillary carcinoma
MET locus (proto onocogene)
papillary carcinoma
papillary carcinoma
- tumor cells have prominent cell membranes and pale cytoplasm “vegetable cells”
- halo around nucleus
chromophobe RCC
does chromophobe RCC have a good prognosis?
yes
chromophobe RCC
RCC triad
- flank pain
- palpable mass
- hematuria
why is RCC called a great mimicker?
- variety of systemic symptoms
- paraneoplastic syndromes
polycythemia, HTN, hypercalcemia
most common sites of metastasis of RCC
2
lungs
bones
RCC tends to invade which vessel?
renal vein
can reach vena cava, right heart eventually
treatment RCC
- nephrectomy or partial nephrectomy
- chemotherapy if metastatic
- present with hematuria, urinary obstruction
- 50% of patients have previous or concurrent bladder tumor
urothelial carcinoma of renal pelvis
risk factors for urothelial carcinoma of renal pelvis
- analgesic nephropathy
- lynch syndrome
most common pediatric renal tumor
Wilm’s tumor
WAGR syndrome
- Wilm’s
- Aniridia
- Genital anomalies
- mental Retardation
protein affected by germline 11p13 mutation
WT1 -Wilm’s
PAX6 -aniridia
- critical for normal renal and gonadal development
- encodes DNA binding transcription factor in embryogenesis
WT1 protein
- causes gonadal dysgenesis and early onset nephropathy
- diffuse mesangial sclerosis
- 90% risk of wilms
Denys-Drash syndrome
Denys-Drash syndrome is caused by germline mutations in
WT1
missense mutation
- organomegaly
- macroglossia
- omphalocele
- adrenal cytomegaly
Beckwith-Wiedermann Syndrome
11p15.5 (WT2) imprinting mutation
Beckwith-Wiederman
- caused by loss of imprinting of normally maternally silenced IGF2 allele
- or duplication of active paternal allele
Beckwith-Wiederman
- small blue bastemal cells
- fibrous or myxoid stromal cells
- epithelial cells
Wilms
[…] mutations indicated a worse prognosis for Wilms
p53
cause anaplasia
wilms
precursor for Wilms
nephrogenic rests
- seen in renal parenchyma adjacent to tumor in 25-40% of cases
- seen in 100% of bilateral cases
nephrogenic rests
precursor to Wilms, increases risk of WIlms in other kidney
- large abdominal mass
- hematuria
- abdominal pain
- intestinal obstruction
- hypertension
- may have pulmonary mets
Wilms