Pathology of Kidney Tumours

(ID#3422)
Tuesday, 2 Dec, 2014, 10:10 - 11:00
Dr Salah Al-Waheeb
Department of Pathology
 Weekly Learning Objective

Describe the clinical and pathologic features of

the most common kidney Tumours.(WLO6292)
 Objectives

1. Describe the clinical features of the main

types of kidney tumours
2. Describe the pathologic(gross and
microscopic) features of the main types of
kidney tumours
3. Describe the molecular gene mutations of
the main types of kidney tumours
Benign
 Papillary adenoma
Commonly found at autopsy: 10% at age 21-
40 years, 40% at age 70-90
Increasing numbers during life are being
detected with more widespread whole body
imaging
Low-grade (usually) neoplasm also known as
renal cortical adenoma
 Small less than 0.5 cm firm mass.
Cortex of kidney
Associated with pyelonephritis
 Angiomyolipoma
Usually benign neoplasm composed of thick-
walled vessels, smooth muscle and adipose
tissue with spindle and epithelioid cells
The amount of each component varies
May occur outside of the kidney
May be sporadic but is associated with
tuberous sclerosis.
Firm grey well circumscribed

Yellow soft predominantly fat

 FAT
SMOOTH MUSCLE
BLOOD VESSELS
 Oncocytoma
4-7% of adult renal epithelial tumors
Adults age 50+; 2/3 men; usually incidental
May co-exist with renal cell carcinoma
 Large Mahogany colour
Central white scar
Polygonal cells
Pink cytoplasm
Abundant Mitochondria on EM
 Renal cell Carcinoma
Clear Cell
Chromophobe
Papillary
 Clear Cell RCC
Also called conventional / classic type
Historical names: hypernephroma due to
belief that it derives from adrenal gland
70% of adult renal epithelial tumors
 Origin: tumor cells are derived from proximal
convoluted tubule
3p- (considered the initial mutation) in 98%
(3p25 is von Hippel Lindau [VHL] gene)
VHL is tumor suppressor gene
Loss of VHL causes an increase in VEGF, which
may cause the increased vascularity of these
tumors
 Risk Factors
Adult polycystic disease,
Smoking (RR = 2.0)
Obesity in women
Hypertension,
Von Hippel Lindau (VHL) disease
 Nodular,solid,cystic,haemorrhagic
yellow,brown
 Clear Cells
Pink Cells
Low grade vs High grade nuclei.
 Chromophobe RCC
5% of adult renal epithelial tumors; ages 45+,
no gender preference.
Cell of origin: intercalated cell of cortical
collecting duct
 Birt-Hogg-Dube syndrome
Multiple tumors, usually bilateral chromophobe
carcinomas, oncocytomas or hybrids
Syndrome is autosomal dominant and features
small dome-shaped papular fibrofolliculomas of
face, neck and upper trunk, renal tumors, lung
cysts and spontaneous pneumothorax
Due to mutations in folliculin gene (FLCN) at
17p11.2
 Solid brown tumour.
 Pink cytoplasm.
Perinuclear Halo.
 Papillary type, RCC
10-20% of adult renal carcinomas
Origin: proximal or distal convoluted tubule
Epidemiology: 75% male
Tends to present at early stage
5 year survival is 82-90%, may be better than
clear cell carcinoma.
Metastasizes to regional lymph nodes
 Papillary or tubulopapillary tumor with foamy
macrophages and intracellular hemosiderin
Size greater than 5 mm distinguishes tumor from
papillary adenoma; frequently trisomy 7, 17
Hereditary papillary renal carcinoma:
Due to activating mutation of MET oncogene at
chromosome 7q31
Autosomal dominant, late onset bilateral
papillary renal tumors
 Multifocal, nodular, tan coloured.
 Wilms tumor of children
Most common pediatric renal tumor, affecting
1 per 8-10,000 children (500 new cases/year
in US)
90% diagnosed prior to age 6 years; rarely
congenital; occasionally diagnosed in
teenagers / adults
M=F
Most are sporadic but some are associated
with several syndromes
 WAGR syndrome: Wilms tumor (1/3), Aniridia,
Genitourinary anomalies, mental Retardation; due to
11p13 deletion (WT1)
Denys-Drash syndrome: gonadal dysgenesis (male
pseudohermaphroditism), glomerulosclerosis and
Wilms tumor most develop Wilms; have WT1
dominant negative missense mutation)
Beckwith-Wiedemann syndrome: exophthalmos,
macroglossia and gigantism; also hemihypertrophy,
renal medullary cysts, adrenal cytomegaly and
hypoglycemia; have 11p15.5 abnormality, may be due
to genomic imprinting.
Abdominal mass
White firm mass
 Triphasic elements-
Blastema-round blue cells
Stroma
Epithelial elements-glomeruli,tubules
 Urothelial carcinoma Renal Pelvis
10% of all renal tumors and only 5% of all
urothelial tumors of the urinary tract.
Tobacco and occupational exposure
 Solid mass in the renal pelvis

Urothelial cells
Squamous cell carcinoma of the renal
pelvis
Associated with squamous metaplasia of renal
pelvis, renal calculi or infection.
Especially with Schistosoma Hematobium in
Egypt.
 Squamous cells
Keratin