Carcinomas renales quísticos

Tumores renales del adulto:
Diagnóstico diferencial
Carcinomas renales
quísticos
Dr. A López-Beltrán
Córdoba
Tumores Renales Quisticos en
Adultos (3%-14%)
Carcinomas con variable componente quistico
Nefroma quistico/Tumor epitelial y estromal
– Un categoria unica-REST
Oncocitoma quistico
Otros:
–
–
–
–
–
Carcinoma tubuloquistico
ACD-related carcinoma
Cystic clear cell papillary carcinoma
Carcinoma no clasificado quistico
Recent proposals
Carcinomas Renales Quisticos
Carcinomas “habitual”con variable componente
quistico
Carcinomas necroticos con quistificacion
secundaria
Carcinomas sobre pared quistica unilocular
Carcinoma renal quistico multilocular (OMS,
2004)
Otros carcinomas con componente quistico
Mejor pronostico: < masa tumoral
Carcinoma Renal Quistico
Multilocular (OMS, 2004; AJCP, 2006)
Compuesto de quistes delimitados por tabiques
que contienen pequeños grupos de celulas
claras de bajo grado (Fuhrman I/II)
No progresion en casos publicados !BENIGNO¡
– RFS del 100% a los 5 años en nuestra serie
>80% pT1 at diagnosis
IHC: marcadores epiteliales +
Mutacion gen VHL >> TUMOR
“TUMOR RENAL DE BAJO POTENCIAL
MALIGNO” (AJCP, 2006)
Multilocular Cystic RCC
Less than 5% of clear
cells
Non-communicating
cysts separated by
fibrous septa
Dif. Diag: Benign
multiloculated renal
cortical cysts.
– Flat epithelium
– No clear cells
Main pathologic features of MCRCC according to 2004 WHO
classification of kidney tumors ( Lopez-Beltran, Algaba, et al. Am
J Clin Pathol, 2006
GROSS FEATURES
•MULTILOCULAR CYSTIC APPEARANCE AND ENCAPSULATED
•YELLOWISH SOLID COMPONENT LIMITED TO SMALL AREAS
•NO EXPANSIVE NODULES
•TUMOR NECROSIS ABSENT
MICROSCOPIC FEATURES
•CYSTS LINED BY CUBOIDAL CLEAR CELLS OR FLATTENED
EPITHELIUM
•SEPTA CONTAINING AGGREGATES OF EPITHELIAL CELLS WITH
CLEAR CYTOPLASM
•LOW FUHRMAN GRADE 1-2
*None of the reported cases in these series had Fuhrman grade 4, stage T4, renal vein thrombus or metastatic disease at diagnosis.
mcRCC
Summary IHC profile Cystic RCC
Nefroma Quistico/Tumor Epitelial y
Estromal
Nefroma Quistico
Neoplasia quistica benigna con elementos epiteliales y estromales
(OMS, 2004)
>>Mujeres 8:1, todas edades, >30 años, !INCIDENTAL¡
Puede asociarse a blastema pulmonar en niños
Macro: encapsulado compuesto de quistes delimitados por tabiques
No areas solidas ni necrosis
Focal o extenso
Histologia: quistes tapizados por epitelio simple aplanado o
ligeramente cuboidal>> !hobnail¡.
– Citoplasma eosinofilico ó claro
Estroma pauci-celular ó celular simulando estroma ovarico>> en
ocasiones contiene tubulos maduros
Nefroma Quistico / Tumor Epitelial
y Estromal
Tumor Epitelial y Estromal
>>Mujeres adultas, 6:1, 46 años
25% INCIDENTAL, >>Antecedentes TTO hormonal
Algunos se situan en pelvis renal
– Hamartoma de la pelvis renal ó nefroma mesoblastico adulto
Estroma:
Variable celularidad que en ocasiones es mixoide
Celulas fusiformes
Micro-macroquistes
Tubulos ramificados
Puede contener musculo liso y grasa
Celulas epiteliales : cuboideas ó “hobnail”
IHC:
–
–
–
Estroma >> actina/desmina y estrogenos/progesterona
Agunos expresan calretinina inhibina
Epitelio >>CK y Vimentina
MALIGNIDAD?
– Algunos desarrollan sarcomas
REST
CN and MEST share similar mRNA
expression profiles
High expresion of Insulin-like GF
Low expresion CA 2 gene
Main Dif Diag Tubulocystic carcinoma
– Negative for ER/PR
Likely represent morphologic
spectrum of same entity:
REST
Other Cystic Carcinomas
Uncommon and recently
described renal carcinomas
New and emerging
Tubulocystic carcinoma
Carcinoma associated with endstage renal disease
–ACD-associated RCC
– Clear cell papillary renal cell carcinoma
Tubulocystic carcinoma
Not formally
recognized in the
WHO ‘Blue Book’
Most adults in the fifth
and sixth decade
Strong male
predominance
Well circumscribed
Spongy ‘bubble wrap’
appearance
Tubulocystic carcinoma
Tightly packed tubules and cysts separated by
bland fibrous stroma frequently fibrotic
The cells are cuboidal to columnar with
abundant eosinophilic or amphophilic cytoplasm
Nuclei large with prominent nucleoli
(Fuhrman grade 3)
Hobnail cells commonly seen
Initially described as low grade collecting duct
carcinoma
Tubulocystic carcinoma
Immunohistochemistry and
genetics
CD10 and racemase positive in greater
than 90% of tumors
CK7 expressed although the pattern may
be weak and focal
The morphologic features are so distinctive
that the utilization of immunohistochemical
markers is not necessary for diagnostic
purposes ( Amin et al., 2009)
Prognosis and predictive factors
Fuhrman nuclear grading does not seem
to have prognostic value
Significant association ( 5 of 13 patients)
with papillary carcinoma ( Yang et al,
2008)
Biological behavior has not been fully
established although the great majority
behave in an indolent fashion
Other Carcinomas with Cystic
Component
Carcinoma associated with endstage renal disease
Am J Surg Pathol 2006
Clear cell-papillary renal cell carcinoma
Am J Surg Pathol, 2008
Carcinoma associated with endstage renal disease
66 kidneys from 52 patients (15 cases with
bilateral nephrectomy)
39 patients had ACDK
38 received dialysis
Tumors multiple and bilateral
Tumor resembling sporadic adult renal cell
neoplasms (40.9%)
Tumors unique to end-stage renal disease
(59.1%)
Am J Surg Pathol 2006
Tumors unique to end-stage renal
disease
ACD-associated renal cell
carcinoma
Arise in a cyst
Solid acinar, solid sheet-like,
microcystic or macrocystic
Variable % of papillary
architecture
Cribriform pattern
Large cells with granular
eosinophilic cytoplasm
Intratumoral oxalate crystals
Racemase +, CK7 -
Clear cell-papillary renal
cell carcinoma
Prominent cystic
component
Predominantly papillary
Cells with clear cytoplasm
polarized in a linear array
away from the basal
aspects of the cells
Racemase -, CK7 +
Am J Surg Pathol 2006
ACD-Asociated RCC
Clear cell-papillary renal cell carcinoma
Clear-cell papillary RCC: FISH
CK7
FISH
7
17
ICH
Y
normal
3p
CD10
AMACR
CK7
neg
neg
+
Gobbo et al. Am J Surg Pathol.2008;32:1239
CEP3 / 3p
CEP7 / CEP17
CAIX
PAX-2
Cystic Partially Regressed ccRCC
Recent proposal
Cystic Partially Regressed Clear Cell Renal
Cell Carcinoma: Clinicopathologic Features
of 27 Cases
Sean R. Williamson et al.
Definition:
Extensively cystic and sclerotic clear cell renal
cell carcinoma (RCC) with only a minor solid
epithelial component
Solid component 10-30%
Follow-up>> 16 patients: none showed
aggressive behavior.
Oncocytic cystic tumor (Unclassified) in TSC
patient
!GRACIAS POR SU ATENCION¡