Kidney Tumors

　

A	Wilms Tumor
B	Grawitz tumor

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Classification of Renal Cell Tumor (1999)

1. Benign: Adenoma

2. Malignant: Renal cell carcinoma

Clear cell carcinoma (Grawitz tumor, hypernephroma, adenocarcinoma)

[proximal tubule origin] (70%)

Granular cell carcinoma (chromophilic) cell carcinama

[proximal tubule origin]) (10-15%)

Chromophobe cell carcinoma [distal tubule origin]

Spindle (pleomorphic) cell carcinoma (Wilms tumor, nephroblastoma)

Cyst-associated renal cell carcinoma

Papillary renal cell carcinoma

3. Collecting-duct (Bellini duct) carcinoma

　

A. Wilms Tumor (nephroblastoma)

Incidence

More than 80% of renal tumors of childhood (ages of 2-4 years)
6-30% of malignant solid abdominal tumors in children

Features

Abnormalities in chromosome 11: deletion of 11p13 containing WT1 gene,

mutations of WT1 gene (transcription factor to suppress gene expression for growth factors or their receptors)

Origin from embrionic tissues

Denys-Drash syndrome: Wilms tumor with nephrotic syndrome (mesangial sclerosis) and psudohemiaphroditism

Pathology

Blastema (nephroblastic),
Epithelium (tubule- or glomerulus-like structure)
Mesenchyma (stroma, striated muscle-like cell)

Miscellaneous structures: (blastema)	Tubule-like structure	Glomerulus-like structure

Striated muscle-like cells

　

B. Clear cell carcinoma (Grawitz tumor)

Genetic background:

loss of chromosome 3p (Von Hipple-Lindau gene in 3p25)

Von Hipple-Lindau disease is an autosomal-dominant multisystem disordor and is characterized by the development of tumors (benign or malignant) in various organs (40% of the patients develop renal cell carcinoma).

Characteristic histology

Alveolar pattern with nest of clear cell separated by a prominent sinusoidal vascular network.

Vascular network

Dark cell / Clear cell

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