general surgery

1. WILM’S TUMOUR
BY DAKSHAYANI KONNUR
INTERN

2. • This is a malignant tumor of the kidney occurring in children.
• Can involve one or both kidneys.
• Most common pediatric renal Malignancy .
• 2nd most common pediatric abdominal malignancy.
• Usually unilateral but 5% Cases may be bilateral.
• Affect children between 3-5yr Of age.
• Left kidney is more involved than right one.

4. PATHOLOGY
• The tumour is composed of epithelial and mesothelial elements. Thus, it may
contain bone, cattilage, muscle, etc. Hence, it is called nephroblastoma
(immature embryonic tissue).
• The tumour arises In one of the poles, distorting the reniform shape of the
kidney.
• Rapidly growing and friable in consistency
• It is greyish white or pinkish white in Colour. At places, there may be areas
of haemorrhage/necrosis.

6. • Microscopic features include connective tissue elements cartilage, spindle cells,
smooth striated muscle cells and epithelial elements.

7. CLINICAL FEATURES
• Common in female children around 2-4 yrs.
• Less than I year of age carries good prognosis.
• Upper limit of age is 7 years.
• Rarely it may occur in adolescents.

8. • The child is brought with abdominal distension, due to hugely enlarged kidney
which on palpation feels nodular.
• Haematuria is a bad prognostic symptom. It is an indication of rupture of tumour
into the pelvis of kidney. Most of such children die by 2 years of age.
• Low grade fever can occur in rapidly growing tumour due to tumour necrosis,
which releases pyrogens.
• Rapid deterioration of health is characteristic.

9. INVESTIGATIONS
• Abdominal USG can detect a solid tumour in the kidney. It also rules out opposite
kidney tumour.
• CT scan to know extent oflesion and spread to the adjoining structures.
• IVP is done to study distortion of calyces and to evaluate the function of the
opposite kidney.
• FNAC is done to confirm the diagnosis preoperatively.

11. STAGING (NTWS)
• Stage 1- Tumour confined to the kidney and Completely excised.
• Stage 2- Tumour outside the kidney but completely Excised.
Local tumour spillage during surgery.
Lymph nodes negative.
• Stage 3- Non hematogenous disease confined to the abdomen.
Perioperative rupture of renal capsule.
Peritoneal implants
Positive lymph nodes
• Stage 4- hematogenous metastasis to lungs or liver.
• Stage 5- bilateral wilms tumour

13. DIFFERENTIAL DIAGNOSIS
• Neuroblastoma arises from adrenals. This is more common than
nephroblastoma.
• Retroperitoneal tumours
• Adrenal tumour

14. • Calcification-foci of calcification seen in NBL (85%). Less common in Wilms’ (15%).
• Intraspinal extension-seen in NBL.
• Aorta and IVC invasion by Wilms’ tumour.
• Location
1. Wilms’ tumour–intrarenal
2. Neuroblastoma-seen above kidney pushing it downwards and outwards.
• Crossing midline neuroblastoma
• Homovanillic acid (HVA) and Vanillylmandelic acid (VMA) increase in neuroblastoma.
DIFFERENTIATING FEATURES BETWEEN WILM’S
TUMOUR AND NEUROBLASTOMA

15. SPREAD
• Direct infiltration of the capsule.
• Lymphatic spread- occurs to the hilar lymph nodes, paraaortic lymph nodes,
mediastinal and left supraclavicular lymph nodes.
• Haematogenous spread- occurs to the lungs, liver, bones, brain, etc. The tumour
thrombus can extend to the renal vein and inferior vena cava.

16. TREATMENT
• Anaemia has to be corrected at the earliest.
• For tumours confined to renal capsule or perirenal soft tissue not infiltrating the
adjacent organs, radical nephrectomy followed by chemotherapy with
actinomycin D and vincristine are given for 6 months.
• For tumours which have gone beyond renal capsule and perirenal soft tissue,
local infiltration to adjacent tissue,lymphatic metastasis, nephrectomy followed
by local radiotherapy and chemotherapy is given with actinomycin D and
vincristine for 15 months.

17. • If the tumour is found to be unresectable by CT scan or magnetic resonance
imaging (MRI), preoperative FNAC to confirm diagnosis is indicated followed
by preoperative radiotherapy ( l 000 cGy) or chemotherapy. Once the tumour
regresses in size, nephrectomy has to be done.
• Postoperative chemotherapy is given with actinomycin D, vincristine and
doxorubicin.

18. • Bilateral Wilms’ tumour: Radical nephrectomy on the side of the larger tumour
and partial nephrectomy on side of the smaller tumour should be done. As much
of renal tissue as possible should be preserved after leaving a tumour free
margin. Postoperatively the patient has to be treated with chemotherapy. If the
surgery is not feasible, only radiotherapy and chemotherapy has to be given.
Growthdisturbances, cardiac and pulmonary toxicities are complications of
radiotherapy.

19. Thank you