Wilm's tumor

  

 Wilms tumor/ nephroblastoma is the most malignant renal and intra abdominal tumor of childhood.

      In the US, about 500 children are diagnosed each year.

       Occurs most often in the first five years of life and accounts for about 5% of all childhood cancers. 

      The five year relative survival rate of children with wilm's tumor is 88%.
Etiology:

  Wilm's tumor probably arise from a malignant, undifferentiated cluster of primordial cells capable of initiating the regeneration of an abnormal structure.
Risk factors:

 - children may have a changed, damaged, or missing gene. About 15% of children with wilm's tumor also have other birth defects. 

- boys with deny-drash syndrome, a condition in which the male sex organs do not develop, have a risk of developing wilm's tumor.

- children with WAGR syndrome have a 33% chance of developing wilm's tumor.
WAGR: wilm's tumor, Ambrosia ( no iris in the eye),  GU abnormalities or gonadoblastoma ( a rare tumor of the reproductive organs), MR. 

Clinical manifestation:

1. Abdominal mass or swelling 

    - firm

    - non tender 

    - confined to one side 

    - deep within the flank 

2. Hematuria

3. Anemia - secondary to haemorrhage within the tumor 

4. Pallor,  anorexia 

5. Fatigue and malaise

6. Hypertension - due to excess secretion of renin 

7. Weight loss, fever

    If metastasis has occurred, symptoms of lung involvement- dyspnea, cough, pain in the chest
Diagnostic evaluation:

-  History and physical examination.

- Radiographic studies 

- Haematologic studies 

- Biochemical studies and urinalysis 

- Inferior venacavogram

   
Staging of wilm's tumor:

  Stage -1: tumor limited to kidney and completely resected 

Stage-2: tumor extends beyond kidney but is completely resected.

Stage-3: residual non-hematogenous tumor is confined to abdomen 

Stage-4: hematogenous metastasis; deposits are beyond stage 3, to lung, liver,  bone and brain 

Stage-5: bilateral renal involvement is present at diagnosis
 Therapeutic management:

 NEPHRECTOMY:

  - tumor, affected kidney, adjacent adrenal glands are removed.

   If both kidneys are involved - radiation and chemotherapy before surgery.

 Partial nephrectomy - on less affected side. 

   

Post- op. Radiotherapy is indicated in case of-

    > large tumor 

     > metastasis 

    > residual post operative disease 

    > unfavourable histologic characteristic recurrence.
    Chemotherapy: 

       > Actinomycin D

      > Vincristine 

      > Adriamycin, with addition of cyclophosphamide for unfavourable histology or advanced disease. 

Nursing management:

   

Preoperative care: 

 - surgery is scheduled with 24-48 hours of diagnosis, hence parents and children should be prepared for the laboratory and operative procedures.

- Do not palpate the tumor.

- monitor BP. 

- Preparation for radiation and chemotherapy- duration, side effects.
Post-op care:

  - monitor BP,  urine output and signs of infection 

- Maintain pulmonary hygiene to prevent post-op pulmonary complications 

  - Play therapy - with dolls, puppets or drawing, helps them to adjust with surgery and side effects.

Precautions: 

- as the child is left with only one kidney, advise to avoid sports or any other activity that has a high risk potential. 

- prevent UTI by good hygiene 

- prompt detection and treatment of any GU signs and symptoms.