MANAGEMENT OF WILMS

TUMOR
Literature Review

[NAMA]
 STAGING
COG STAGING FOR WILMS TUMOR
Stage I • tumor is limited to kidney and has been completely resected
• tumor was not ruptured or biopsied prior to removal
• no penetration of renal capsule or involvement of renal sinus vessels
Stage II • tumor extends beyond capsule of kidney but was completely resected
with no evidence of tumor at or beyond margins of resection
• there is penetration of renal capsule or invasion of renal sinus vessels
Stage III • gross or microscopic residual tumor remains postoperatively, inc.
inoperable tumor, positive surgical margins, tumor spillage, regional
lymph node metastases, positive peritoneal cytology, or transected
tumor thrombus
• tumor was ruptured or biopsied prior to removal
Stage IV • hematogenous metastases or lymph node metastases outside
abdomen, e.g. lung, liver, bone, brain
Stage V • bilateral renal involvement is present at diagnosis and each side may be
considered to have a ‘local’ stage
 SURGICAL CONSIDERATIONS
Pediatrics
• recommended initial therapy: radical nephrectomy via
transperitoneal approach
• exploration of abdominal cavity >> to exclude local tumor
extension, liver and nodal metastases, peritoneal seeding
• adrenal gland can be spared if not in close proximity to
tumor
• selective sampling of suspicious nodes is essential
• responsibility: complete removal of tumor without
contamination of operative field
• gentle handling is mandatory to avoid tumor spillage
 SURGICAL CONSIDERATIONS
Pediatrics
• laparoscopic nephrectomy
• feasible with preoperative chemotherapy and
reduction in tumor size
• partial nephrectomy (parenchymal-sparing)
• may be considered for unilateral tumors
• to avoid late occurrence of renal dysfunction
(higher risk in patients with genitourinary
anomalies, DDS, WAGR)
 SURGICAL CONSIDERATIONS

Pediatrics
•risk factor for local tumor recurrence
•tumor spillage
•unfavorable histology
•incomplete tumor removal
•absence of lymph node sampling
 SURGICAL CONSIDERATIONS
Pediatrics
• surgical complications
• hemorrhage
• small bowel obstruction
• risk factors for increased complications
• higher tumor stage
• tumor size > 10 cm
• incorrect preoperative diagnosis
• thoracoabdominal incision
• intracaval tumor extension
• resection of visceral organs
 POST-OP IRRADIATION

Pediatrics
• postoperative irradiation is given for
patients with positive lymph node and
diffuse tumor spill (increased risk of
abdominal relapse)
• irradiation with 10 – 20 Gy is successful
in reducing tumor recurrence rates
after tumor spillage
 POST-OP CHEMOTHERAPY

Pediatrics
• postoperative chemotherapy is not needed for
very-low-risk Wilms tumor (VLRWT)
• definition: children < 2 y.o with stage I
favorable-histology tumors weighing < 550 gr
• high risk Wilms tumor: tumor with relapse after
chemotherapy with ≥ 3 agents (survival 40-50%)
• very-high-risk Wilms tumor: recurrent anaplastic
or blastemal type Wilms tumor (survival 10%)
COG RECOMMENDATION FOR POST-OP IRRADIATION & CHEMOTHERAPY
Stage I-II favorable histology, LOH 1p and • VCR, AMD, DOX
16q • no radiotherapy
Stage III favorable histology • VCR, AMD, DOX
• irradiation of flank or abdomen
Stage I-III focal AHWT (or) stage I diffuse • VCR, AMD, DOX
AHWT • abdominal irradiation
Stage II-IV diffuse AHWT (or) stage IV focal • new chemotherapy regimen
AHWT (or) stage IV clear cell sarcoma (or)
stage I-III malignant rhabdoid tumor
 PRE-OP CHEMOTHERAPY

Pediatrics
• recommended for
• children planned for renal-sparing
surgery
• tumor inoperable at surgical exploration
• tumor extension into IVC above hepatic
veins
 INOPERABLE TUMORS
• should not be based on preoperative imaging

• decision must be made by surgeon

 BILATERAL WILMS TUMOR
• goal of chemotherapy: tumor shrinkage and renal-sparing surgery

• renal tissue is preserved to avoid renal failure

• tumors not responding to therapy require bilateral open biopsy to

determine histology

– open biopsy more recommended than percutaneous needle

biopsy >> more accurate in anaplasia assessment

– bilateral biopsy is recommended >> anaplasia is commonly

discordant
 BILATERAL WILMS TUMOR
• all patients should proceed to surgical resection within 12
weeks of starting therapy
– continuing therapy >> lack of benefits
– partial nephrectomy or wedge resection is preferred in attempt
to achieve negative margins
– tumor enucleation may be considered
• if renal-sparing surgery is not feasible, additional
chemotherapy is given based on biopsy finding
 MANAGEMENT FOR ADULTS
• outcome for Wilms tumor in adults is poor >> require
more intensive therapy
• general recommendation: stage-appropriate combined
modality therapy
THANK YOU