WILMS’ TUMOR

- Nephroblastoma
- Embrioma renale
- Mixed tumor of the kidney
WILMS’ TUMOR

the most common malignant embryonal

neoplasma of the kidney in children

6% of all malignancies in children

8 permillion children < 15 years of age
peaks at 2 – 4 years
sex ratio is 1:1

first described by Rance in 1814

the named is derived from Max Wilms (1899)

Bilateral tumor: 5% in nephroblastoma

inherited
ETIOLOGY

Wilms’tumor derived from pathologic

proliferation of metanephric blastema
(pluripotensial embrionic cell)
no normal stimulation in metanephron duct
to form well differentiation

germinal or somatic mutation:

8 -34 weeks of age intra uterine
hereditary form are rare
7-10%  autosomal dominant inherited

WT1 gene chr 11 p13 WAGR

PATOLOGY
The classic pattern triphasic:
blastemal, epithelial & stromal
 Low, intermediate & high risk tumor

METASTASES
- Percontuinitatum
- Hematogen
- Lymphogen

The most common site:

regional lymph nodes
lungs, liver & bone
CLINICAL PRESENTATION
The most common initial manifestation
is asymptomatic abdominal mass.

Hematuria (25%)
Hypertension (25%)
Anorexia, nausea vomiting,
Fever, constipation
Weight loss

May have associated congenital anomalies:

aniridia, hemihypertrophy and
genitourinary malformation
STAGE OF WILMS’ TUMOR (NWTS)

Stage I : the tumor is resricted to the kidney

& can be totally excised
Stage II : the tumor is not restricted to the kidney
but can be totally excised
Stage III : incomplete excision of the tumor,
no metastases
Stage IV : Metastases (lung,liver, bone)

Stage V : Bilateral nephroblastoma

DIAGNOSTIC PROCEDURES

- Clinical features
- Radiodiagnostic
 USG, CT, IVP
- Tumor markers:
LDH increased
- Pathology  tissue biopsy

IVP: distortion of pyelocalyces

TREATMENT MODALITIES

- Surgery
- Chemotherapy
- Radiotherapy

Dependent on:
Stage
Histopathology characteristics

Almost always begins with surgery

to remove or decrease of the kidney tumor
PROGNOSIS

The main prognostic variables are:

- histopathology
- stage
- age
- tumor size