Kidney trivia Flashcards
How is RCC graded?
FUHRMAN GRADING: based on nucleoli
- 400x: nucleoli basophilic and inconspicuous
- 400x: nucleoli eosinophilic and conspicuous, but inconspicuous at 100x
- 100x: eosinophilic and conscpicuous
- Nuclear pleomorphism; sarcomatoid; rhabdoid.
what are the histologic features of chromophobe RCC?
Architecture: alveolar or multi-cystic
Two histologic patterns:
Plant-cell like
Eosinophilic
Nuclear features: Irregular nuclear borders (raisinoid) PERINUCLEAR halo Binucleation Hyperchromatic
NOT graded
What are key histologic features of FH-deficient RCC?
PERI-NUCLEOLAR halo
Eosinophilic cytoplasm
Large, high-grade nuclei (CMV-like)
Variable architecture (papillary, tubulopapillary, others) ; intracystic papillary formation common
What are characteristic features of collecting duct carcinoma of the kidney?
Medulla
Desmoplasia
Lymphocytic response
looks like a high-grade adenocarcinoma
Variable architecture, can be tubulopapillary, solid, nests, cords, cribriform, infiltrating glands.
Which kidney tumor has Perinuclear Halos, and which one Perinucleolar?
Perinuclear: Chromophobe
Perinucleolar: FH-Deficient (HLRCC)
Which tumor type stains with Hale’s colloidal iron, and what is staining?
Chromophobe
HCI stains acid mucopolysaccharides in microvesicles (seen on EM/ultrastructurally)
What are some characteristic features of MiTF RCC?
Clinical (1)
Histologic (features of Xp11 type)
Molecular (2)
Pediatric (40% of peds RCCs)
High nuclear grade
Prominent papillary &/or solid alveolar (because dyscohesive) growth patterns
Clear cells (eosinophilic also seen)
Psammoma bodies
TFE3 or TFEB mutation
What is a strict criterion to diagnose a Multilocular Cystic Renal Neoplasm of Low Malignant Potential?
NO SOLID tumor nodules, MUST be entirely cystic
What is the classic gross characteristic of Tubulocystic carcinoma?
“Bubble wrap”
Micro: look like variably-sized, cystically dilated tubules lined by single layer of eosinophilic epithelium. Cuboidal, flat or HOBNAIL.
What are characteristic clinical, histologic and immunohistochemical features of Renal Medullary carcinoma?
Sickle-cell anemia; Aggressive, rapidly fatal
Micro: desmoplastic stroma, neutrophils, margin of lymphocytes
Immuno: INI-1 loss !!!!!
Also OCT3/4+
Loss of INI-1 is requisite for diagnosis.
What are characteristic features of SDH-deficient RCC?
Clinical & syndromic
Histologic
Immuno
Young patients (median 38) SDH deficient syndrome: paraganglioma, pituitary adenoma, SDH-deficient GIST usually indolent unless high grade (rare)
Eosinophilic
Solid, nested or tubular , cystic areas
Histo: round, bland nuclei
intracytoplasmic vacuole or inclusion with EOSINOPHILIC MATERIAL
loss of SDH-B
PAX8+
What are characteristic features of Eosinophilic, Solid & Cystic RCC?
Associated with Tuberous Sclerosis
EXCLUSIVELY WOMEN
eosinophilic, abundant cytoplasm
granular stippling (looks like leishmaniasis)
prominent nucleoli
CK20+/ CK7- phenotype
Which renal tumor classically has a “sieve-like” architecture?
Acquired cystic kidney disease-associated RCC
List 3 (out of 7 possible) syndromes associated with Wilms Tumor
WAGR
Denys-Drash
Beckwith-Wiedemann
Perlman familial nephroblastosis syndrome
Trisomy 18
Neurofibromatosis
Bloom syndrome
Which Wilms-associated syndrome is associated with Perilobar nephrogenic rests?
Beckwith-Wiedemann
