Renal

Question 1

Clear cell renal cell carcinoma mutation?

Gross color?

Defining feature?

IHC?

Answer 1

VHL inactivation and upregulation of hyoxia-inducible factor–> increased IHC carbonic anhydrase CA-9

Yellow/golden

Lots of small thin walled blood vessels

CK7-, PAX8+, Her2, CA-9 (complete membrane!)

Question 2

Clear cell renal genetic association?

Multilocular cystic neoplasm of low malignantt potential findings?

Answer 2

VHL 3p25-26

Numerous cystss with thin speta lined with clear cell like cells. NO NECROSIS or OVERGROWTH

Question 3

Papillary RCC Type 1 vs 2.

CK7 status?

Genetic profile?

Prognosis?

Answer 3

Type 2 is more “pink” and plump with more and worse looking nuclei”; Type 1 cores more classic

T1+ CK7+, T2 CK7+ ~50%

Trisomy 7, 17, and loss of Y; 13% RET

Better than ccRCC, and urothelial

Question 4

Hereditary leiomomatosis and RCC nuclei contain?

IHC stain?

Answer 4

Inclusion in neucli with perinuclear clearing!

Fumerate hydrase LOSS and icnrease of 2-succinocyteine and CK7- and Racemase-

Question 5

Chromophobe gross findings?

Micro findings?

IHC?

Answer 5

Brown with possible central scar (more seen in oncocytoma)

Wrinkled nuclei and irregular and binucleated: PLANT CELL MORPHOLOGY solid sheet

Vimentin negative! CK7+ (more positive than oncocytoma), E-cadherin +, Hale’s Colloidal Iron

Question 6

Oncocytoma gross finding?

Hybrid Chromo and Onco tumor associated with?

Answer 6

Brown with Central scar!

Birt-Hogg-Dube; AD folliculin gene

Question 7

Renal medullary carcinoma is associated with?

Looks like?

Survival?

Answer 7

Sickle cell trait and hemoglobinopathies;

Neutrophils and poorly differentiated adeno apperence. OCT3/4+ and INI1 loss, CK7+, PAX8+

Poor, metastasis common.

Question 8

Collecting duct vs urothelial carcinoma (IHC)?

MiT family transolcation genes?

Answer 8

CD: PAX8+, HW+, P63-, GATA3-
Uro: PAX8-, HW-,P63+, GATA3+

TFE3: Xp11 (indolent and peds)

TFEB: t(6;11) MALAT1-TFEB (adults)

Question 9

Xp11 translocation common histofinding?

t(6,11) histologic findings?

Answer 9

Papillary tumor with epithelial and clear cells; psammoma bodies

Biphasic; nests of epithelioid cells and smaller cell clustered around basement membrane

Question 10

Succinate dehydrogenase deficent RCC, histo finding?

Associated with?

Answer 10

Vacuolated eosinophilic to clear cells with cytoplasmic vacoules

Loss of IHC expression of SDHB

SDH-deficent RCC, paragangliomas, SSDH-deficent GIST

Question 11

Mucinous tubular spindle cell carcinoma?

Prognosis?

Acquired cystic disease assocaited RCC has what type of crystal?

Answer 11

Need to see spindle cellss with mucinous stroma

Good

ACD-RCC: OXALATE, microcysstic architecture

Question 12

Clear CEll Papillary RCC/ High or low grade?

IHC?

Answer 12

Low grade with reverse polarity (away from bassement membrane) with tubulopapillary architecture

PAX8+, CK7+, CA-9 CUP SHAPED (Vs Clear Cell full +), AMACR-

Question 13

Metanephric adenoma histo and IHC?

Wilms triphasic?

What needs to be reported?

Associated with?

Answer 13

Tight single lined cells; WT1 +, CD57+, AMCAR -, EMA-, CK7 focal +

Blastemal, epithelial and stromal

Anaplasia! Associated with bad prognosis and chemo resistance

WAGR, Deny-Drash (WT1 muctation), Beckwidth Weideman