Renal Pathology & Neoplasms

darodri6's version from 2016-10-09 14:59

Cystic Kidney Lesions

NameTypeEtiologyCyst LocationKey Desc
AD PKD (adult)GeneticMutation in polycystin 1 (PKD1 chrom 16p) & polycystin 2 (PKD2 chrom 4q) --> altered renal tubulogenesis/proliferationALL parts of NEPHRON (clear-to-brown fluid). Enlarged kidneyPain in abdomen, flank + HTN

Multisystemic (renal tubues, liver ducts, pancreas, lungs, etc)
AR PKD (infant)GeneticMutation in PKHD1 gene (fibrocystin) --> altered CD and biliary (liver) differentiationBL @ renal CD at cortex& medulla PERPENDICULAR to SMOOTH cortex surface (enlarged kidney)oligouria @ birth --> oligohydramnios --> Potter sequence (defects in limbs, facial features, & pulm hypoplasia)
+ hepatic fibrosis w/exp portal area & inc bile ducts
Juvenile NephronopthisisGenetic (AR)-Mutation in NPHP1 (2q13; nephrocystin --> cilia function in kidney, resp & RETINA)
-NPHP2 (9q13; inversin --> tubular development & left-right axis detemrinaiton)
-both lead to --> altered renal tube development/dunction & ciliar function
CORTICOMEDULLARY JUNCTION cysts (normal to small kidneys)Child with polyuria/polydipsia, anemia, short stature & RETINITIS PIGMENTOSA
Medullary Cystic Kidney DzGenetic (AD)n/asimilar to nephronopthisispresents during adulthood with NO EXTRARENAL involvement
Medullary Sponge KidneyAcquiredassoc. w/hemihypertrophy of body, Marfans or Ehlers-DanlosBL small cysts in MEDULLARY CDs (medullary pyramids & papillae). Normal sized kidney + NORMAL CORTEXnormal renal fxn; may see calcification/stones, hematuria & infection (if 30+ years)
Acquired Cystic DzAcquiredassoc w/prlonged dialysis for ESRD or long-term uremiaCORTICAL /MEDULLARY cysts that may contain OXALATE CRYSTALS. Enlarged kidney.due to uremia, obstruction by oxalate cystals, fibrosis or hyperplasia
Simple CystsAcquiredassoc. w/increase in ageCORTICAL cysts (thin-walled w/clear-yellow fluid)presents as mass lesion --> hemorrhage may cause pain
Multicystic Renal DysplasiaDevelopmentalabnormal METANEPHRIC differentiation (by wk 20) --> assoc w/ureteropelvic obstruction, ureteral agenesis, atresia or refluxenlarged cystic kidneys w/ISLANDS of CARTILAGE & fibrotic & disorganized intervening parenchymahealthy newborn with only an abdominal mass in the flank

Kidney Neoplasms

Renal Cell Tumor = Epithelial cell = lines the cavities & surfaces of blood vessels & organs throughout the body
Mesenchymal Cell Tumor = stem cells for bone, muscle, fat, cartilage, blood vessels, etc.)
Malignant Tumors = -nomas & -comas (also lymphoma/leukemia)....most other -omas are Benign
Carcinoma = Epithelial origin & Malignant
Sarcoma = Mesenchymal origin & Malignant


Renal Cell Carcinoma = Males >60, SMOKERS!!!!, obesity, HTN, long-term NSAIDs or dialysis, kidney transplant and several genetic disorders (see 'Related Systemic Dz & Genes' section). Clinical = FLANK PAIN, HEMATURIA, FLANK MASS + Constituional (wt loss, fever, HTN)
NameTypeKey Histo Desc.Key ClinicalKey Associated Dz or Gene
Renal Papillary Adneoma (aka Renal CORTICAL adenoma)Benign Renal Cell Tumor (Epithelial)Gross = well-circumscribed, UNENCAPSULATED
Micro = small cytoplasms (AMPHOBILIC-to-BASOPHILIC), psammoma bodies (concentric calcified structures), Xanthomas (fat cells), NO INVASION
found in SUBCAPSULAR Kidney, 5mm or less
Renal OncocytomaBenign Renal Cell Tumor
Gross = well-circumscribed, UNENCAPSULATED, mahongany or brown-yellow, STELLATE CENTRAL SCAR
Micro = EOSINOPHILIC, GRANULAR cytoplasm w/uniform round/polygonal cells & uniform/centralized nuclei
Males > 50 yo
Renal AngiomyolipomaBenign Tumor (Mesenchymal)Micro = BVs w/ THICK WALLS, myoid spindle cells, Islands of mature adipose tissue-can occur in LIVER, LUNGS & RETROPERITONEAL SOFT TISSUE
-dx should express HMB45 (Ab used for dx of melanocytic tumors)
Tuberous Sclerosis & TSC2/PKD1 Contiguous Gene Syndrome
Clear Cell TypeMalignant Renal Cell Carcinoma (RCC)Gross = Orange/yellow, Well-circumscribed & possible NECROSIS, HEMORRHAGE and CALCIFICATION
Micro = CLEAR cytoplasm arranged in NESTS w/INTERVENING BLOOD VESSELS
dx = cells co-express CYTOKERATIN & VIMENTINVHL gene (3p25) & TOBACCO!!!!!PCT
Papillary TypeMalignant Renal Cell Carcinoma (RCC)Gross = THICK capsule
Micro = Papillary or Tubulopapillary tumor w/intracellular HEMOSIDERIN & FOAMY MACROPHAGES
multifocal & common in males-mutations in the MET proto-oncogene
-Trisomy 7 & 17
Xp11 Translocation TypeMalignant Renal Cell Carcinoma (RCC)Micro = eosinophilic & clear cells-dx via FISH or PCR
-usually women < 35 yo
gene fusion that involves TFE3 trx factor
Collecting Duct TypeMalignant Renal Cell Carcinoma (RCC)Gross = gray-white tumor in MEDULLA & POORLY circumscribed
Micro = glands lines by HIGH GRADE HOBNAIL cells & marked FIBROSIS
-mostly males-affects CD of renal MEDULLA
Chromophobe TypeMalignant Renal Cell Carcinoma (RCC)looks like onchocytome.
Gross = TAN-BROWN & Well-Circumscribed
Micro = GRANULAR & PALE EOSINOPHILIC cytoplasms w/ PERINUCLEAR HALOS (main diff from onchocytoma)
-dx = positive stain for HALES COLLOIDAL IRON stainBHD SyndromeCORTICAL CD (Intercalated cells)
Urothelial CarcinomaMalignant at Renal PelvisGross = EXOPHYTIC (grows outward, beyond epithelial surface) PAPILLARY mass
-hematuria & flank pain & multifocal
-occurs with lower UT Dz & majority are PAPILLARY (high grade)
-TOBACCO & Industrial carcinogens (COAL, ASPHALT, PETROCHEMICALS, TAR), Radiocontrast
Wilm's Tumor (Pediatric)Malignant NephroblasticGross = well-circumscribed & soft, homogenous & gray, HEMORRHAGE, MECROSIS, CYSTS & LOBULAR PATTERN common
Micro = Triphasic (non-developed/differentiated BLASTEMA + Epithelial elements + Fibroblasts-like stroma)
Large abdominal mass, NEPHROGENIC RESTS (fragments of embryonal cells that remain --> precursor for this type of tumor)
-may invade renal vein, metastasize to renal lymph nodes, lung, liver, or peritoneum
WAGR Syndrome, Denys-Drasg syndrome, Beckwith-Wiedeman syndrome, Nephroblasmatosis