The prognosis of adults with WT is poor compared with children who have a 85% chance of cure. This success in pediatric WT represents a paradigm shift to multimodal treatment. Historically in the 1980's, most series of adult cases had reported long-term survival of about 25%. Before the report of Arrigo et al. of 27 patients reported to NWTSG 1979-87 with an overall survival at 3 years of 67%, it was believed that this high cure rate can not be achieved in adults. What is Wilms Tumor? you can read in Wilms Tumor ADULT kidney tumors
This series includes six stages I, five stage II, four stage III, eleven stage IV, one patient with stage V-four patients with anaplastic histology. In this series, 26 patients underwent nephrectomy, 25 received chemotherapy and 20 received radiation treatment. This led to recommendations that patients with stage I disease and favorable histology should be treated with surgery followed by 6 months of postoperative chemotherapy with actinomycin-D and vincristine without postoperative radiotherapy, and for stage II, III and IV / FH vincristine, actinomycin-D and doxorubicin for 15 months with 2000 cGy radiation to the tumor bed, from 1200 to 1500 cGy to the lungs, liver 2000 cGy and 3,000 cGy to other sites where appropriate in patients with metastatic at diagnosis. Kattan et al. reported the French experience in 22 adult patients 1973-92.
His series included four stage I, eight stage II, three stage III and seven patients with stage IV. All patients underwent nephrectomy followed by adjuvant treatment modality in only seven patients (radiotherapy and chemotherapy in one in six) and combined modality in 15 patients. The chemotherapeutic agents most often used actinomycin-D, vincristine and doxorubicin. Two of the seven (29%) and 07/15 (47%) patients were free of disease after first-line treatment. salvage chemotherapy had to be given in 13 patients. After a mean of 100 months, 12/22 patients (55%) were alive, including ten who were free of disease (45%). We recommend aggressive treatment, including the three-drug regimen (actinomycin-D + vincristine + doxorubicin), regardless of the stage, and irradiation starting phase II. Terenziani et al. reviewed the Italian experience with 17 adult patients between 1983-2001 who were treated with an Italian protocol and were followed for an average of 131 months.
This included eight patients with stage II, four patients with stage III and five patients with stage IV and included a patient with anaplasia. Sixteen patients undergoing nephrectomy patients, fifteen received chemotherapy (ten with both drugs and five with three drugs) and seven patients received radiation. The 5-year survival free of disease was 45% with an overall survival of 62%. Reinhard et al. reviewed the German experience, which included 30 adult patients in the SIOP 93-01 study. Ten patients (33%) had metastatic disease at presentation. There was a predominance of the upper stage (Stage I, 8 stage II, 7, stage III, 15 patients), histology revealed an intermediate risk in 23 high-risk patients and in 2 patients. Twenty-six patients underwent radical nephrectomy and primary of the other four patients received neoadjuvant chemotherapy before surgery. Nineteen patients received chemotherapy and 11 intermediate risk patients received chemotherapy with high risk according to the protocol. Intermediate-risk chemotherapy includes vincristine, doxorubicin, actinomycin-D ± 18 to 27 weeks, and high-risk scheme etoposide, carboplatin, ifosfamide and doxorubicin for 34 weeks. Four-teen patients received radiotherapy 15 to 35 Gy and three patients received radiation to sites of metastasis. Complete remission was obtained in 24 patients (80%) with event-free survival of 57% and overall survival of 83% with an average observation time of 4 years.
These four modern contemporary series have tended to rest the skepticism with regard to multimodal treatment of PT in adults. Multimodal approach tailored risk similar treatment to pediatric protocols WEIGHT is the current standard of care. In contrast to the pediatric population, where opinion differs as to whether the nephrectomy should be done primarily or after neoadjuvant therapy, there is a consensus view that primary surgery is recommended for adult WT because of the difficulty to establish this rare diagnosis before the operation. Only in cases of primarily inoperable patients the diagnosis is established by biopsy and neoadjuvant therapy in place to try to tumor regression and improve operations. In the absence of bilateral disease, which is rare in adults, primary surgery should include a radical nephrectomy with lymph node sampling.
Despite a complete resection of all viable tumor is the effort desirable surgical can endanger vital organs is not advisable, since the local control can be achieved by adjuvant treatment. Because of the rarity of the disease, there is no established treatment guideline in WT adult. The treatment should preferably be conducted in a tertiary hospital with expertise in this disease. On the basis of the recommendations in the literature, including the series of four cases cited above, current and previous experience NWTSG trial in the pediatric population suggest the following:
- Based on test data and previous multinational experience NWTSG, radiotherapy can be avoided in patients with stage I and stage II patients with favorable histology, when treated with a combination chemotherapy regimen such as vincristine and actinomycin-D. Radiation should be used in the adjuvant treatment of stage III and IV after surgery. Radiation probably also be given to sites of metastasis.
- Chemotherapy should be used in the adjuvant treatment at all stages in patients with adequate organ function and functional status. Stage I can be treated with a regimen of two drugs for 18 weeks. Chemotherapy for Stage II must be adapted to the risk based on histology and can vary from two to four drug regimens in 18 to 24 weeks. Chemotherapy for Stage III and IV also has to be adapted to the risk based on histology and requires three or four-drug regimens 18 to 24 weeks. The chemotherapy regimens used should be based on existing pediatric experience. In the recently closed NWTS-5 study, the two-drug regimen was vincristine and actinomycin-D for 18 weeks, the three-drug regimen was vincristine, actinomycin-D and doxorubicin for 24 weeks, and was the four-drug regimen doxorubicin vincristine, cyclophosphamide and etoposide for 24 weeks.
- Patients with bilateral tumors (stage V) should be given primary chemotherapy for about 6-8 weeks, followed by bilateral partial nephrectomy nephrons in an attempt to preserve normal kidney tissue. Additional chemotherapy and radiotherapy may be needed after surgery. In an earlier series of Byrd et al. , Was observed that adults are at risk of relapse over a longer period of time compared with children. This has not been supported by more recent series. Recurrent disease in children has been successfully treated with radiation, multiagent salvage chemotherapy regimens (etoposide, carboplatin and ifosfamide), 129 or high-dose chemotherapy with stem cell support 130 which leads to long-term remissions (30-60%).