Adult Wilms' Tumor: Report of 2 Cases and Review of the Literature

Abstract

Wilm's tumor is rare in adulthood. The prognosis is poor when compared with children. Adults more frequently present with advanced disease stages, and chemotherapy has a moderate effect. The various protocols of chemotherapy and indications for surgery and radiotherapy are not yet precisely defined, mainly due to the rarity of the disease. The Society of Pediatric Oncology (SIOP) guidelines suggest that both pediatric and adult patients should have initial chemotherapy followed by surgery. However, the National Wilms' Tumor Stage Group (NWTSG) recommends surgery followed by chemotherapy. The authors present 2 cases of adult nephroblastoma that were highly resistant to presurgical chemotherapy. Nephrectomy was not completed because of rapid disease progression. The poor response of these 2 patients indicates that the alternate NTWSG approach of initial surgery followed by systemic treatment might have better results for this population. The authors review related reports and discuss the diagnosis and management of adult Wilms' tumor.

Adult nephroblastoma

“…According to all available studies and due to the very small number of reported cases, randomized trials cannot be undertaken and results of pediatric trials should be considered. And Until recently, no standardized treatment for AN was found in literature [3,8]. There In 1982, the NWTSG reported 31 patients with AN who were treated between 1968 and 1979 [8,6].…”

“…And Until recently, no standardized treatment for AN was found in literature [3,8]. There In 1982, the NWTSG reported 31 patients with AN who were treated between 1968 and 1979 [8,6]. 51.7% had stage III and stage IV disease.…”

“…These results indicated a need for new therapeutic approaches for patients with AN. In 1990, Arrigo et al [8,11] from the NWTSG studied 27 adult patients who were treated between 1979 and 1987. Their results demonstrated a survival rate of 67% at three year.…”

“…were only a few reports of small series of patients with AN who were treated with different protocols involving chemotherapy, surgery, and radiotherapy [8]. The results of these early studies are somewhat contradictory.…”

“…The results of these early studies are somewhat contradictory. Fortunately, the more current guidelines proposed by the National Wilms Tumor Study Group (NWTSG) and The International Society of Pediatric Oncology (SIOP) have helped to direct physicians [8].…”