Ask the Experts - Advanced Stage IV Wilms Tumor in an Adult?
What is the best treatment for a 39-year-old man with a stage IV adult Wilms tumor with lung metastases, para-aortic adenopathy, and performance status (PS) 0 after a nephrectomy?
Wilms tumors are rare in adulthood, but the histology and staging are identical to those seen in pediatric oncology. In children, the histologic classification defines prognosis stage by stage. Because of this, the Fifth National Wilms Tumor Study (NWTS-5) has 5 treatment arms, based upon specific histologic groupings, including focal anaplasia, diffuse anaplasia, clear cell sarcoma, and rhabdoid tumor.
For children with stage IV disease, 4-year survival rates (and probably therefore cure) are between 50% and 70%. However, there is a feeling that adult patients do not do as well. The optimum management of an adult patient should best be decided by a multidisciplinary team with experience in treating pediatric Wilms tumors.
Treatment guidelines for adult patients are based on small series of patients and case reports, and investigators differ on how aggressive treatment should be. Some advocate aggressive treatment for all stages and histology, whereas others adopt the risk-adaptive protocols (using favorable vs unfavorable histology) used for pediatric patients.
What all experts agree on is that patients with stage IV disease should receive radiotherapy, which, for this particular patient, should involve both whole abdominal irradiation and whole lung irradiation. The timing of radiotherapy should be integrated into the administration of a multiagent chemotherapy regimen consisting of vincristine, doxorubicin, cyclophosphamide, and dactinomycin. In this patient, who had a nephrectomy, radiotherapy should probably follow completion of chemotherapy. I would suggest that in the United States, this patient be treated on the appropriate stage IV pediatric NWTS protocol.
What is the best treatment for a 39-year-old man with a stage IV adult Wilms tumor with lung metastases, para-aortic adenopathy, and performance status (PS) 0 after a nephrectomy?
Wilms tumors are rare in adulthood, but the histology and staging are identical to those seen in pediatric oncology. In children, the histologic classification defines prognosis stage by stage. Because of this, the Fifth National Wilms Tumor Study (NWTS-5) has 5 treatment arms, based upon specific histologic groupings, including focal anaplasia, diffuse anaplasia, clear cell sarcoma, and rhabdoid tumor.
For children with stage IV disease, 4-year survival rates (and probably therefore cure) are between 50% and 70%. However, there is a feeling that adult patients do not do as well. The optimum management of an adult patient should best be decided by a multidisciplinary team with experience in treating pediatric Wilms tumors.
Treatment guidelines for adult patients are based on small series of patients and case reports, and investigators differ on how aggressive treatment should be. Some advocate aggressive treatment for all stages and histology, whereas others adopt the risk-adaptive protocols (using favorable vs unfavorable histology) used for pediatric patients.
What all experts agree on is that patients with stage IV disease should receive radiotherapy, which, for this particular patient, should involve both whole abdominal irradiation and whole lung irradiation. The timing of radiotherapy should be integrated into the administration of a multiagent chemotherapy regimen consisting of vincristine, doxorubicin, cyclophosphamide, and dactinomycin. In this patient, who had a nephrectomy, radiotherapy should probably follow completion of chemotherapy. I would suggest that in the United States, this patient be treated on the appropriate stage IV pediatric NWTS protocol.
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