Pathology 8 Flashcards
what are three examples of benign neoplasms of the kidney?
renal papillary adenoma, angiomyolipoma, oncocytoma
what is renal papillary adenoma?
small discrete adenomas arising from the renal tubular epithelium
how might angiomyolipoma present?
with tuberous sclerosis and intellectual disability
how does an oncocytoma appear?
large benign appearing cells with eosinophilic cytoplasms with scattered large nuclei; mahogany brown
when do renal cell carcinomas present?
older individuals- 6th-7th decade of life; usually males
what is the most significant risk factor for renal cell carcinoma?
tobacco
most renal cancer is sporadic, but unusual forms of autosomal dominant familial cancers occur, usually in younger individuals. what are 4 examples of familial variants of renal cancers?
von hippel lindau (VHL) syndrome, hereditary leiomyomatosis and renal cell cancer syndrome, hereditary papillary carcinoma, birt-hogg-dube syndrome
what is von-hippel-lindau (VHL) syndrome?
hemangioblastomas of the CNS, cysts that involve the kidneys, renal cellcarcinomas, and pheochromocytomas
what are the risk factors for renal cell carcinomas?
obesity, hypertension, unopposed estrogen therapy, and exposure to asbestos, petroleum products, and heavy metals; also increased in patients with ESRD, CKD, and acquired cystic disease and tuberous sclerosis
renal cell carcinomas may arise in any portion of the kidney, but more commonly affect what?
the poles
the classification of renal cell carcinoma is based on what?
correlative cytogenic, genetic, and histologic studies of both familial and sporadic tumors
what are the different types of renal cell carcinomas?
clear cell carcinomas, papillary carcinoma, chromophobe carcinoma, Xp11 translocation carcinoma
where do clear cell carcinomas likely arise?
proximal tubular epithelium-usually as solitary unilateral lesions
why are clear cell carcinomas yellow?
there are prominent lipid accumulations
how do tumor cells appear in clear cell carcinomas?
they have a rounded or polygonal shape and abundant clear or granular cytoplasm
what occurs in 98% of clear cell type renal carcinomas?
there is a loss of sequences on the short arm of chromosome 3
what is the purpose of the VHL gene?
it acts as a tumor suppressor gene in both sporadic and familial cancers
what happens when VHL is deleted?
there is increased expression of HIF1, leading to increased expression of VEGF and IGF-1
where is the VHL gene?
3p25.3
buzzwords for papillary carcinoma?
psammoma bodies and interstitial foam cells
what could lead to sporadic clear cell carcinoma?
deletions on chromosome 3 (cytogenetics)
what could lead to hereditary clear cell?
loss of VHL, inactivated mutated VHL, and hypermethylation of VHL (genetics)
what could lead to sporadic papillary carcinoma?
trisomy 7, 17, loss of Y
what could lead to hereditary papillary carcinoma?
trisomy 7, mutated activated MET
what are the characteristic features of Xp11 translocation carcinoma?
young children, translocations of the TFE3 gene at Xp11.2
what are some symptoms of renal cell carcinomas?
flank pain, palpable mass, hematuria, constitutional symptoms
what are the earliest manifestations of renal tumor sometimes?
systemic symptoms not related to the kidney (paraneoplastic syndromes)
in addition to fever and constitutional symptoms, renal cell carcinomas produce a number of syndromes including what?
polycythemia, anemia, hypercalcemia, hypertension, hepatic dysfunction, feminization or masculinization, cushing syndrome, eosinophilia
what type of epithelium lines the renal pelvis?
urothelium
when does urothelial carcinoma of the renal pelvis present?
usually early on because they produce noticeable hematuria
what could urothelial carcinoma of the renal pelvis lead to?
palpable hydronephrosis and flank pain
what is Wilms tumor?
the most common primary renal tumor of childhood (2-5 yo)
germline deletions on what chromosome and what genes in WAGI?
11p13 (WT1 and PAX6)
symptoms of Denys-Drash?
gonadal dysgenesis and early onset nephropathy leading to renal failure
what is the characteristic glomerular lesion in patients with denys-drash?
diffuse mesangial sclerosis
what are patients with denys-drash at increased risk of developing?
germ cell tumors called gonadoblastomas
symptoms of beckwith-wiedemann syndrome?
abnormal large cells in the adrenal cortex, hemihypertrophy, organomegaly, macroglossia, omphalocele
what is observed in the vast majority of wilms tumors?
the classic triphasic combination of blastemal, stromal, and epithelial cell types
what characterizes the blastemal component of the wilm tumor?
sheets of small blue cells
