RENAL TUMORS

BY: DR HASEEB KHAN

 RENAL TUMORS

BENIGN
 Renal Papillary Adenoma
 Angiomyolipoma
 Oncocytoma

MALIGNANT
 Renal Cell Carcinoma
 Urothelial Carcinoma of the Renal Pelvis

 Wilm’s tumor
 MALIGNANT TUMORS

RENAL CELL CARCINOMA

UROTHELIAL CARCINOMA OF THE RENAL PELVIS

WILM’S TUMOR
 RENAL CELL CARCINOMA

85% of renal cancers in adults.

Approximately 65,000 new cases per year and
13,000 deaths from the disease.

Sixth and seventh decades of life

2 : 1 male preponderance.
 RENAL CELL CARCINOMA

Tobacco

Obesity (particularly in women)

Hypertension
Unopposed estrogen therapy
Exposure to asbestos, petroleum products, and heavy metals.

End-stage renal disease

Chronic kidney disease
Acquired cystic disease
Tuberous sclerosis.
 RENAL CELL CARCINOMA

Von Hippel-Lindau (VHL) syndrome

Hereditary leiomyomatosis and renal cell cancer
syndrome
Hereditary papillary carcinoma
Birt-Hogg-Dubé syndrome
RENAL CELL CARCINOMA
CLEAR CELL CARCINOMA

PAPILLARY CARCINOMA

CHROMOPHOBE CARCINOMA

XP11 TRANSLOCATION CARCINOMA

COLLECTING DUCT (BELLINI DUCT) CARCINOMA

 CLEAR CELL CARCINOMA

70% to 80% of renal cell cancers

They can be familial, but in most cases (95%) are

sporadic.

Deletion on the short arm of chromosome 3

The deleted region harbors the VHL gene (3p25.3)
VHL gene acts as a tumor suppressor gene
Increased Hypoxia inducible factor-1 (HIF-1)
Increased VEGF and IGF-1
 CLEAR CELL CARCINOMA

Arise from proximal tubular epithelium

Solitary unilateral lesions

Bright yellow-graywhite spherical masses

Variable size
Necrosis and hemorrhage

Tendency to invade renal vein

CLEAR CELL CARCINOMA
 CLEAR CELL CARCINOMA

Solid to trabecular (cordlike) or tubular

Rounded or polygonal shape of cells

Abundant clear or granular cytoplasm

Delicate branching vasculature

CLEAR CELL CARCINOMA
CLEAR CELL CARCINOMA
 PAPILLARY CARCINOMA

10% to 15% of renal cancers

Both familial and sporadic forms

Trisomies 7 and 17 and loss of Y in male patients in

the sporadic form
Trisomy 7 in the familial form

MET, a proto-oncogene
GENETICS
 PAPILLARY CARCINOMA

Arise from distal convoluted tubules

Multifocal and bilateral

Hemorrhagic and cystic

Papillae
Cuboidal or low columnar cells
Foam cells are common in the papillary cores
PAPILLARY CARCINOMA
 CHROMOPHOBE CARCINOMA

5% of renal cell cancers

multiple chromosome losses and extreme

hypodiploidy.

Grow from intercalated cells of collecting ducts

Excellent prognosis
 CHROMOPHOBE CARCINOMA

Pale eosinophilic cells

Perinuclear halo

Arranged in solid sheets

CHROMOPHOBE CARCINOMA
 Xp11 TRANSLOCATION CARCINOMA

Young patients

Translocations of the TFE3 gene located at Xp11.2

Clear cytoplasm
Papillary architecture.
Xp11 TRANSLOCATION CARCINOMA
Xp11 TRANSLOCATION CARCINOMA
 COLLECTING DUCT CARCINOMA

1% or less of renal cancers

Arise from collecting duct cells in the medulla

Several chromosomal losses and deletions

Irregular channels
Lined by highly atypical epithelium
Hobnail pattern
Prominent fibrotic stroma
COLLECTING DUCT CARCINOMA
COLLECTING DUCT CARCINOMA
 CLINICAL FEATURES

Costovertebral pain
Palpable mass
Hematuria

Generalized constitutional symptoms, such as fever, malaise,

weakness, and weight loss

Abnormal hormone production

 Polycythemia, hypercalcemia, hypertension, hepatic dysfunction,
feminization or masculinization, Cushing syndrome, eosinophilia,
leukemoid reactions, and amyloidosis

Metastasis at presentation
 MANAGEMENT

5-year survival rate of persons with renal cell

carcinoma is about 70% and as high as 95% in the
absence of distant metastases

Radical nephrectomy has been the treatment of

choice.
Urothelial Carcinoma of the
Renal Pelvis

5% TO 10% OF PRIMARY RENAL TUMORS

EARLY IDENTIFICATION

BAD PROGNOSIS
Urothelial Carcinoma of the Renal Pelvis
 WILM’S TUMOR

1 IN EVERY 10,000 CHILDREN

2 AND 5 YEARS

BILATERAL INVOLVEMENT IS COMMON

 WILM’S TUMOR

11p13 (WT1)
11p15.5 (WT2)

WAGR syndrome
Denys-Drash syndrome
Beckwith-Wiedemann syndrome

Nephrogenic rests
 WILM’S TUMOR

Large, solitary, well-circumscribed mass

Soft, homogeneous, and tan to gray

Hemorrhage, cyst formation, and necrosis

WILM’S TUMOR
WILM’S TUMOR
 WILM’S TUMOR

Triphasic combination

 Blastemal

 Stromal

 Epithelial
WILM’S TUMOR
ANAPLASIA
 PROGNOSIS

Most patients cured

Anaplastic histology = adverse prognosis

Secondary malignancies (related to radiotherapy)

BENIGN TUMORS

Renal Papillary Adenoma

Angiomyolipoma
Oncocytoma
 RENAL PAPILLARY ADENOMA

Found commonly (7% to 22%) at autopsy

Small tumors, usually less than 0.5 cm

Cortex

Pale yellow-gray, discrete, well-circumscribed

nodules.
 RENAL PAPILLARY ADENOMA

Complex, branching, papillary structures

Cells may also grow as tubules, glands, cords, and
sheets of cells.

Cells are cuboidal to polygonal in shape

Regular, small central nuclei
Scanty cytoplasm
No atypia.
RENAL PAPILLARY ADENOMA
RENAL PAPILLARY ADENOMA
 ANGIOMYOLIPOMA

Vessels, smooth muscle, and fat

Originating from perivascular epithelioid cells.

Associated with Tuberous sclerosis

 Lesions of the cerebral cortex that produce epilepsy and mental
retardation
 A variety of skin abnormalities
 Unusual benign tumors at other sites, such as the heart.
ANGIOMYOLIPOMA
 ONCOCYTOMA

Arise from the intercalated cells of collecting ducts

5% to 15% of renal neoplasms

Tan or mahogany brown

Relatively homogeneous
Well encapsulated
Central scar
ONCOCYTOMA
 ONCOCYTOMA

Large eosinophilic cells

Small, round, benign-appearing nuclei

Large nucleoli
ONCOCYTOMA
ONCOCYTOMA