What are the chemotherapy guidelines for Wilm's tumor?

Chemotherapy Guidelines for Wilms Tumor

The standard chemotherapy regimen for Wilms tumor consists of vincristine, actinomycin D, and doxorubicin, with treatment intensity determined by tumor stage and histology. 1

Staging and Risk-Based Treatment Approach

• Treatment recommendations are based on the National Wilms' Tumor Study (NWTS) schema, which stratifies therapy according to stage and histology 1
• Two major collaborative groups guide treatment protocols: the Children's Oncology Group (COG) and the International Society of Paediatric Oncology (SIOP), with the main difference being the timing of surgery 2

First-Line Chemotherapy Regimens by Stage

Favorable Histology

• Stage I and II: Postoperative "pulsed" or "conventional" dactinomycin (actinomycin D) and vincristine without radiotherapy 1
• Stage III: Triple-agent chemotherapy with dactinomycin, doxorubicin, and vincristine, plus abdominal radiotherapy 1
• Stage IV: Aggressive triple-agent chemotherapy (dactinomycin, doxorubicin, vincristine) with radiotherapy to selected sites 1

Unfavorable Histology

• Stages II-IV anaplastic, clear cell, or rhabdoid histology: Aggressive triple-agent chemotherapy with radiotherapy to selected sites 1

Preoperative Chemotherapy (SIOP Approach)

• The SIOP protocol recommends preoperative chemotherapy before surgery to reduce tumor size and decrease risk of surgical complications 2
• Standard preoperative regimen includes vincristine and actinomycin D 3
• In settings where actinomycin D is unavailable, vincristine and doxorubicin have shown success as preoperative treatment in achieving tumor shrinkage 3

Relapse Treatment

• For patients who relapse after initial treatment with vincristine, actinomycin D, and doxorubicin (VAD):
  • Alternating cycles of cyclophosphamide/etoposide and carboplatin/etoposide 4
  • This regimen has shown approximately 50% event-free survival for children with unilateral Wilms tumor who relapse after initial VAD treatment 4

Special Considerations

• Vincristine is FDA-approved for use in Wilms tumor in combination with other oncolytic agents 5
• Adult Wilms tumor (extremely rare, <1% of renal malignancies) can be treated according to the UMBRELLA SIOP2016 adult guidelines, which include vincristine, actinomycin-D, and doxorubicin with concurrent radiation therapy 6
• Thrombocytopenia is the most frequent toxicity observed in relapse treatment protocols 4

Treatment Goals and Future Directions

• Current survival rates exceed 80% for Wilms tumor patients 1
• Modern treatment aims include:
  • Performing nephron-sparing surgery in selected cases 2
  • Reducing dosage and duration of chemotherapy and radiotherapy in appropriate cases 2
  • Risk-stratified strategies to minimize long-term complications while maintaining high cure rates 2

Common Pitfalls to Avoid

• Delaying treatment initiation can adversely affect outcomes; prompt referral to pediatric oncology centers is essential 2
• Failure to recognize the importance of a multidisciplinary approach involving pediatric surgeons, oncologists, radiologists, and radiation oncologists 2
• Inadequate monitoring for common chemotherapy toxicities, particularly thrombocytopenia in relapse protocols 4