Kidney Stuff Flashcards
Disease Angiomyolipoma associated with (also other findings?)
AML benign or maliganant?
IHC?
Treatment?
Tuberos sclerosis: AD neurocutaneous disorder w/ Hamartomas in different organs, TSC1 (9q32) and TSC2 (16p13), 80% develop AML
Benign but invade other organs; hemorrhage complication
Partial nephrectomy and emobloi
HMB45+ and Melan-A+
What is XGP?
What do cultures grow?
Treatment?
Chronic destructive granulomatous process w/ 1. chronic inflammation (plasma cells), 2. Multinucleated giant cells, 3. Foamy histiocytes
Classic central scar and mahogony brown color
E. Coli and Proteus; F>M
Antibiotics
What is a cystic nephroma (histology findings)?
Treatment?
Benign rare tumor in males <4 yrs old and females 40-60. See multiple cysts lined with small, flat cuboidal cells and blue stroma
Partial nephrectomy
What is a congenital mesoblastic nephroma (histology)?
Maligant potential?
Age group?
Pushing tumor with circumscribed border with spindled uniform low N:C ratio cells.
Low but cellular variant more at risk of metastasis
<3 months
Metanephric adenoma (histology)?
Benign or maligant? Associated with what heme issue?
IHC
Pushing circumscribed tumor with small tubules; looks similar to Wilms but lacks mitosis and blastemal compnent
Benign and polycythemia
WT1+, CD56- (Wilms CD56+)
RCC Classic Gross Feature and histology?
How to grade; what makes auto grade 4?
What genetic disorder is associated?
IHC?
Yellow golden mass; lace-like vascular pattern with clear cytoplasm and numbers thin walled vessels
By nuclei size (2: see nuclei at 40x, 3: 10x, 4: low power): sarcomatoid features
VHL: Clear cell, pheos, retinal angioma, pancreatic cysts, CNS hemangioblastoma, epindymal cystadenomas: Auto dom: loss of VHL on Chr3 ; 25-60% have RCC
PAX8+, CD10+, RCC+, Vimentin+, CK7-
Chromophobe renal cell carcinoma (histology and differentiate from RCC)?
Mortality vs Clear cell and papillary RCC?
What genetic disease is it associated with?
What electon microscope finding and special stain?
IHC?
Eosinophilic tumor with a pushing border and some clear cells (less vascular than RCC); “plant like cell walls”
Lower; can have sarcomatoid features
Brit-Hogg-Dube: Benign cutaneous leasions, pulmonary cysts, spontaneous pneumothorax, multicoal bilateral RCC, FLCN on Ch17, Auto Dom
Hybrid onocytic/chromophobe and chromophobe tumors common
Intracytoplasmatic microvesicles and Hale’s collooidal iron
CK7+, EMA+, E-cadherin+, CD10-, RCC-
Papillary RCC (histology; type 1 vs 2)?
Better prognosis I or II?
Is it associated with dialysis?
Papillary w/ high NC ratios with scant cytoplasm;
Type 1 has foamy histiocytes and foamy papillae (better prognosis); type II have abundant cytoplasm and are more pink with think fibrocores
Yes
What is Hereditary Papillary Renal Cell Carcinoma (gene involved?)
Hereditary Leiomyomatosis and Renal Cell Carcinoma?
PRCC, MET onogene 7q31, auto dom, 70% of carriers develop paillary RCC type 1
FH gene on 1q42-43, Auto dom, Skin an duterine leiomyomas, RCC Type II with aggressive features and mets
Wilms tumor (3 microscopic findings and gross)?
Patients who get this?
Chromosome association?
Treatment?
Survival and biggest risk factor for poor prognosis?
Gross: Smooth tumor w/o necrosis
Micro:Small blue cells, tubules, fibrous stoma
2-4 yrs old. 4th most common peds malignancy, painless abdominal mass
11 WT1; W11LM’s
Nephrectomy stage I, Chemo III/IV
90% survival/ Anaplasia predicts chemo resistance
Wilms associated syndromes (3 of them)?
Bechwith-Wiedmann: Macroglossia, abdominal wall defects, visceromegalcy, hemihyperplasia, ear lobe creases, high birth weight
WAGR: Wilms, Aniridia, genitourinary abnormalities, and mental retardation
Denys-Drash: Early nephrotic syndrome to ESRD and peudohermaphorism
