Renal system carcinomas and cyst Flashcards
What is a cyst?
Sac filled with fluid
what are the characteristics of a cyst?
1- ALL cysts are lined with CUBOIDAL EPITHELIUM ( Except one type and gives malignancy potential )
2- Some Cysts ENLARGE the kidneys, some have no effect and some even shrinks it
3- Knowing the site of cyst is very crucial for diagnosis purposes ( Medullary or cortical or both )
4- what are the conditions associated with Cysts :
Asymptomatic , patient on dialysis , carcinoma, neonatal death, RENAL FAILURE
imp to know the age of renal failure ( some come in 20-30 and some 40-50 )
what are 3 cysts disease where the cyst is found in BOTH cortex and medulla?
Infantile AR polycystic kidney disease
Adult (AD) polycystic kidney disease
Renal dysplasia –> multicystic renal dysplasia
so cysts in both medulla and cortex
do mixed cysts ( cortical and medullary ) affect the kidney size?
yes
when we have cysts in both it tends to ENLARGE THE KIDNEY ( tends to not all cases )
what is infantile AR polycstic kidney diseaes?
poly cyst = multiple cysts in the kidney
Infantile =in infant
So multiple cysts in the kidney of an infant
AR? = AUTOSOMALL RECESSIVE
what is the clinical presentation of infantile AR polycystic kidney disease ?
Stillbirth
Neonatal death
whenever u read stillbirth, neonatal death and multiple cysts in an infant = infantile AR polycystic kidney disease
what is the gene mutation in infantile AR polycystic kidney disease ?
PKHD1
way to remember = infant = boss baby = has big head = PK (PIK ) HD ( head )
PKHD1= PK HEAD1 gene mutation
what are the grossly features infantile AR polycystic kidney disease ?
Massively enlarged kidney by :
small cysts in cortex
small cysts in medulla ( BOTH )
the cysts ae perpendicular to the renal capsule
what is the histological feature of the cyst in infantile AR polycystic kidney disease ?
Cyst lined by cuboidal cells
( REMEMBER ALL CYSTS ARE LINED BY CUBOIDAL CELLS EXCEPT ONE TYPE )
what are the extra RENAL manifestations of infantile AR polycystic kidney disease ?
Congenital hepatic fibrosis in patients who survive the infancy
what is adult AD polycystic kidney disease ?
multiple cysts of kidney in adults
AD = autosomal dominant so ADULT has AD =autosomal dominant
What is the clinical presentation of adult AD polycystic kidney disease ?
renal failure at the age of 40-50 ( OTHER CYST WILL BE 20-30)
What are the genetic mutations that lead to Adult AD polycystic kidney disease?
PKD1
PKD2
to remember –> an adult person PKED fi al 3amal or an adult persons is tired cuz his schedule is PACKED ( PKD )
so adult = PKD1 and 2
what are the grossly features of adult AD polycystic kidney disease ?
Starts of as :
Few cysts in cortex and medulla
THEN PROGRESS to :
Massively enlarged kidney with many cysts
thats why renal failure comes at 40-50 cuz progression take time
what are the histological features of the cysts of adult AD polycystic kidney disease ?
Cuboidal epithelium like always except the 1 case
INFLAMMATION :
interstitial fibrosis , Lymphocyte infiltrate , tubular atrophy
what are the extra renal manifestation of adult AD polycystic kidney disease ?
Intracranial berry aneurysms
Pancreatic cyst
Hepatic cysts
Mitral valve prolapse
what is renal dysplasia ?
Multi cystic renal dysplasia
Dysplasia here doesnt mean cancer cells proliferation
IT MEANS MALFORMATION ( abnormal development of the kidney
MOST COMMON PEDIATRIC CYSTIC RENAL DISEASE
what are the grossly features of renal dysplasia ( multicystic renal dysplasia )?
Enlarged cystic kidney
Numerous variable sized cysts
COULD BE UNILATERAL OR BILATERAL
what are microscopic features of multicystic renal dysplasia ?
Disorganized kidney parenchyma
Cysts lined by cuboidal epithelium ( like always except for 1 case )
Cartilage
IMMATURE TUBULES surrounded by BLASTEMAL CELLS
blastemal cells = small round blue cells that are immature
TO REMEMBER : BLUE TUBE CCs
Blue = blastema cells
Tube = immature tubules
C = cyst with cuboidal epithelium
C= cartilage
what is acquire cystic disease ?
condition that happens to patients on hemodialysis or chronic renal failure
THEY START DEVOLPING CYSTS
what is the location of the cysts in acquired cystic disease ?
CORTICAL CYSTS
no medulla cysts
what could these cyst contain?
calcium oxalate crystals
what type of epithelium lines these cysts?
ATYPICAL HYPERPLASTIC EPITHELIUM
could be come malignant and become tumor = becomes solid
its the only exception where the cyst doesnt have cuboidal epithelium
what is medullary sponge disease ?
condition where we have cysts in the medulla
Children or adolescents
CYSTS ARE IN THE MEDULLA ONLY NOT CORTEX from the name