Adult renal tumours Flashcards
What are the differentials of clear cell renal cell carcinoma in the kidney?
Other primary renal tumours with clear cell morphology eg: - clear cell papillary rcc - multilocular cystic rcc
What is the pattern of staining of CA9 in clear cell papillary rcc?
Cup shaped basolateral staining (no apical/luminal staining)
List the WHO Classification of renal cell tumours
- Clear cell RCC
- Multilocular cystic renal neoplasm of LMP
- Papillary RCC
- Hereditary leiomyomatosis and RCC-associated RCC
- Chromophobe RCC
- Collecting duct carcinoma
- Renal medullary carcinoma
- MiT family translocation RCC
- SDH-deficient RCC
- Mucinous tubular and spindle cell carcinoma
- Tubulocystic renal cell carcinoma
- Acquired cystic disease-associated RCC
- Clear cell papillary RCC
- Papillary adenoma
- Oncocytoma
What are the benign renal cell tumours according to WHO?
Oncocytoma
Papillary adenoma
Describe the categories of tumours of kidney according to WHO
- Renal cell
- Metanephric
- Nephroblastic and cystic
- Mesenchymal (divided into mainly adults and mainly children)
- Mixed epithelial and stromal tumour family
- Neuroendocrine
- Haematopoetic
- Germ cell
- Metastatic
What are the metanephric tumours of the kidney?
Met. adenoma
Met. adenofibroma
Met. stromal tumour
What are the mesenchymal tumours mainly seen in adults?
- LMS (including renal vein LMS)
- Angiosarc
- Rhabdo
- Osteosarc
- Synovial sarcoma
- EWS
- AML
- Epithelioid AML
- Leiomyoma
- Haemangioma (and lymphangioma)
- Haemangioblastoma
- Juxtaglomerular cell tumour
- Renomedullary interstitial cell tumour
- Schwannoma
- SFT
What are the mixed epithelial and stromal tumours?
Adult cystic nephroma
Mixed epithelial and stromal tumour
What are clinical risk factors of RCC?
Obesity
Smoking
HTN
Acquired cystic kidney disease
Occupational exposure
List syndromes associated with renal tumours (and which tumours they are associated with)
- Von Hippel-Lindau (RCC, haemangioblastoma, cysts)
- Birt-Hogg Dube (RCCs of various types - clear cell, chromophobe, papillary, oncocytoma, hybrid oncocytic tumour -> chromophobe/oncocytoma)
- Beckwith-Wiedemann (Wilm’s tumour)
- WAGR (Wilm’s tumour)
- Denys-Drash (Wilm’s)
- Hereditary papillary RCC syndrome (multiple bilateral type 1 prcc)
- Hereditary leiomyomatosis and rcc syndrome (type 2 papillary rcc and leiomyomas)
- SDH-RCC (RCC with particular histologic features)
- HyperPTH-jaw tumour syndrome (MEST, papillary RCC)
- Tuberous sclerosis (AML)
- Familial PTC syndrome (oncocytomas)
The ISUP grading system is used for which tumours?
Clear cell RCC
Papillary RCC
Describe the ISUP grading system for renal tumours
Grade 1: nucleoli absent/inconspic/basoph at x400
Grade 2: nuc conspic/eosinoph at x400, visible but not prominent at x100
Grade 3: nucleoli conspic/eosinoph at x100
Grade 4: Marked pleomorphism, giant cells, rhabdoid/sarcomatoid differentiation
How does clear cell RCC metastasize and to where?
Haematogenous (renal sinus - important to report, renal vein)
Goes to lungs, liver, adrenal, bone, soft tissue, and elsewhere - often seen in random places
What is the immunophenotype of clear cell RCC?
PAX8+
CAIX - membranous + (complete - vs basolateral of papillary rcc)
CK, EMA+
CK7 rare (isolated cells, helps to diff from chromophobe which is ++)
CD10+ membranous (not specific)
How can the VHL gene be altered in clear cell rcc?
Deletion
Methylation/silencing
Mutation
What are the histologic features of multilocular cystic renal neoplasm of LMP?
Entirely cystic
Multiple cysts - lined by single layer of low grade nuclei
Septa containing individual or groups of clear cells without expansile growth
Can have calcification and ossification
What is the differential diagnosis of multilocular cystic renal neoplasm of LMP?
- Clear cell RCC w/ cystic necrosis
- Tubulocystic carcinoma
- Cystic nephroma
- Clear cell papillary RCC w/ predominantly cystic config
- Benign multilocular renal cortical cysts
What is the underlying genetic alteration in multilocular cystic renal neoplasm of LMP?
VHL deletion (same as clear cell RCC)
What is the etiology of papillary RCC?
ESRD with scarring
Acquired cystic disease
Genetic syndromes (hereditary PRCC syndrome, Birt-Hogg-Dube)
What is the radiologic appearance of papillary RCC?
Can appear hypovascular due to spontaneous tumour necrosis
Often has foci of calcification
What are the histologic features of papillary RCC?
Circumscribed, prominent pseudocapsule
Papillae (can be tightly packed and appear tubular or solid)
FV cores with foamy macrophages and psammoma bodies
Cores can be oedematous or hyalinized
5% show sarcomatoid change
What is the immunoprofile of papillary RCC?
CK+
AMACR+
CD10+
CK7+ (more in type 1 than type 2)
