The 54th Annual Congress of the International Society of Paediatric Oncology, Barcelona, İspanya, 28 Eylül - 01 Ekim 2022, ss.1686
Background and Aims
To evaluate the clinical and pathological characteristics and treatment outcome of Wilms tumour (WT) cases treated in our institution.
Methods
Between 1988-2021, patients with WT were reviewed retrospectively. Twentysix patients were treated accoding to the TPOG-WT protocol, remaining patients were treated according to the NWTS protocol.
Results
Sixtyone patients were eligible out of 71. The median age was 44 months, M/F:0.7. Median duration of complaints was one month. There were three familial-WT. Genetic abnormalities were genitourinary abnormalities (n:5), hemihyperplasia (n:3), aniridia (n:1), Denys-Drash (n:1), Beckwith Wiedaman(n:1), DICER 1 syndrome (n:1). Tumour was unilateral in 97% of cases. There was one patient with extrarenal-WT. Complete tumour resection was performed in all except two cases. There were intraoperative tumour rupture (n:6) and partial resection (n:2) in patients without neoadjuvan chemotheraphy (n:26). Stage distribution was stage-1 27%, stage-2 17%, stage-3 20%, stage-4 23%, stage-5 3%. There were 20 metastatic patients (pulmonary:18, liver:6, bone:2). Histopathology revealed 76% FH, 24% UH. Intraoperative complications were not observed in patients who were given preoperative CT, and complete tumor excision could be performed in all of them. Radiotheraphy was performed to the primary tumour site in 22 cases, to whole abdomen in ten cases. Median follow-up-time was 43 months for all cases, and 5-years OS was 83%, 10, 15-years OS were 79%. Nine patients relapsed, three of them primary, others systemic relapse. Median relapse time:10 months. Additional three patients had refractory disease. Ten patients died with disease progression. 5-years EFS was 68%,10, 15-years EFS were 61%.
Conclusions
Obtained survival rates were similar with original protocols. No prognostic
factor could be determined owing to the limited patient number but stage and
presence of anaplasia are important criteria.