NEOPLASMS of the KIDNEY

NEOPLASMS of the KIDNEY

• RENAL CELL NEOPLASMS • MIXED EPITHELIAL AND STROMAL NEOPLASMS• MISCCELANEOUS TUMORS • UROTHELIAL NEOPLASMS• PEDIATRIC RENAL NEOPLASMS

Classification of Epithelial Kidney Tumors(WHO 2004 & AFIP 4th Ed)

Benign tumors Papillary adenoma Metanephric adenoma Metanephric adenofibroma Oncocytoma

Classification of Epithelial Kidney Tumors(WHO 2004 & AFIP 4th Ed)

Malignat tumors (renal cell carcinoma) Clear cell renal cell carcinoma Multilocular cystic renal cell carcinoma Papillary renal cell carcinoma Chromophobe cell carcinoma Collecting duct carcinoma Medullary carcinoma Mucinous tubular and spindle cell carcinoma X:1 Translocation carcinoma Carcinoma associated with neuroblastoma Unclassified carcinoma

Classification of Mixed and Mesenchymal Kidney Tumors(Adults)(WHO 2004 modified, Grignon 2006)

Mixed epithelial and mesenchymal tumors Cystic nephroma Mixed epithelial and stromal tumors Synovial sarcomaMesenchymal tumors (benign) Angiomyolipoma Leiomyoma Hemangioma Juxtaglomerular cell tumor Renomedullary interstitial cell tumor Other benignMesenchymal tumors (malignant) Epithelioid angiomyolipoma Leimyosarcoma Angiosarcoma Solitary fibrous tumor Other sarcomas

RENAL CELL NEOPLASMS

RENAL CELL CARCINOMAS are tumors that arise from the renal tubular epitheliumBefore they were thought arise from adrenal rests, termed “hypernephroma”Accounting for more than 90% of all malignancies arising in the kidney

CORTEX MEDULLA

CLEAR CELL RENAL CELL CARCINOMA

• Definition: - the most common variant epithelial tumors - cells: clear or eosinophilic granular

cytoplasm• Location: renal cortex• Gender: M>F. 2:1• Age: adult, 6 to 7 decade• Clinical feature: hematuria

PATHOLOGY• GROSS - solitary renal cortical mass - well circumscribed - golden yellow, necrosis and hemorrhage• MICROSCOPIC - nests and sheets of clear cells - delicate vascular network - pseudopapillary, tubular, trabecular, microcystic, sarcomatoid

IMMUNOHISTOCHEMICAL• Cam 5.2, AE1-AE3,EMA, RCC, Vimentin

and CD10 – positive • Keratin 34ßE12 – negative• S-100 or CEA – rarely positive

FUHRMAN’S nuclear grading

• Tumors are graded from grade 1 – 4, according to NUCLEAR size and shape, and NUCLEOLAR prominence

• Grading is most useful in clear cell and the other subtype of RCC

FUHRMAN GRADE 1/4

FUHRMAN GRADE 4/4

AJCC-TNM Staging of Renal Cell Carcinoma (2002)

Primary Tumor (T)

TX Primary tumor cannot be assessed

T0 No evidence of primary tumor

T1a Confined to Kidney, ≤ 4.0 cm.

T1b Confined to Kidney, > 4.0 cm and ≤ 7.0 cm

T2 Confined to Kidney, > 7.0 cm

T3a Tumor invades the perinephric fat or the adrenal gland but not beyond Gerota’s fascia

T3b Tumor grossly extends into the renal vein(s) or vena cava below the diaphragm

T3c Tumor grossly extends into the renal vein(s) or vena cava above the diaphragm

T4 Tumor invades beyond Gerota’s fascia

Regional Lymph Nodes(N); Distant Metastases (M); Staging

WILMS Tumor (Nephroblastoma)

Definition• Wilms' tumor is a rare kidney cancer that

primarily affects children. • Also known as nephroblastoma, it's the

most common malignant tumor of the kidneys in children.

• The peak time of Wilms' tumor occurrence is around ages 3 to 4, and it occurs only rarely after age 6.

CLINICAL features• A malignant embryonal neoplasm derived

from nephrogenic blastemal cells• Most of these tumors are found before

metastazise• 1 in 8000 children• Abdominal mass, pain, hematuria,

hypertension• Associated with dysmorphic syndromes

Fate of NEPHROGENIC REST

• Remain unchanged in size and composition

• Maturation, sclerosis and disappearance• Hyperplastic NRs, proliferation of all

susceptible cells. Absence of pseudo-capsule at the interface between hyper-plastic NRs and renal parenchyma

• Neoplastic induction, to represent a clonal event originating in single cells of a rest

PATHOLOGY• GROSS - well-circumscribed - encapsulated - gray-tan, lobulated cut surface• MICROSCOPIC - three patterns:1)blastemal, 2)epithelial, 3) stromal

IMMUNOHISTOCHEMICAL• Vimentin – positive• WT1 – positive: blastemal and epithelial• WT1 – negative: epithelial and stromal• P53 – over expression

UROTHELIAL NEOPLASM (TRANSITIONAL CELL TUMORS

UROTHELIAL NEOPLASM (TRANSITIONAL CELL TUMORS)• 90% of bladder tumors are classified as

urothelial carcinoma, also referred to as transitional cell carcinoma (TCC)

• are believed to originate from transforma-tion of the normal urothelium

• urothelial dysplasia > carcinoma in situ > invasive carcinoma

Bladder: Urothelial carcinomasClassificationHistologic types: • urothelial carcinoma of the bladder • squamous cell carcinoma • adenocarcinoma (2%), rare • poorly differenciated carcinoma/small cell

carcinoma, exceptional

UROTHELIAL NEOPLASM (TRANSITIONAL CELL TUMORS)• Carcinoma cell squamosa 5% in USA,

70-80% in Middle East• M3/F1• Occurs mainly in the 6th-8th decades• Risk factor: cigarette smoking, and

occupational exposure (aniline, benzydine and naphtylamine

• 20-30 yrs latency after exposure

PATHOLOGY Grading and staging• Graded by the degree of cellular atypia

(papilloma, G1->G3) – WHO 1973 • Staged - WHO 2004

- papilloma - papillary tumor of low malignant potential (PTLMP)- papillary urothelial carcinomas low grade- papillary urothelial carcinomas high grade.

Papilloma

Inverted papilloma

Inverted papilloma

Inverted papilloma

SUPERFICIAL BLADDER CANCER ClinicallyNon invasive papillary Ca

Muscle invasive bladder cancer

PATHOLOGICAL STAGING

STAGING OF TRANSITIONAL CELL CARCINOMA OF THE RENAL PELVIS

pTIS Carcinoma in situpTa Non-invasive papillary tumorspT1 Invasion of lamina propria (no muscle)pT2 Superficial muscularis propriapT3 Deep muscularis propria or pelvic fatpT4 Adjacent structures (including kidney)