Clinical factors in relapses of Wilms' tumor--results for the Polish Pediatric Solid Tumors Study Group.

BACKGROUND: The risk factors responsible for recurrences of Wilms' tumor (nephroblastoma) are still under discussion. The aim of the study was to analyze the relationship between relapses of Wilms' tumor and the patients' clinical history. MATERIAL AND METHODS: Clinical data from children registered in the Polish Pediatric Solid Tumors Study Group were analyzed. The clinical stages (CS), pathology variants (high risk: HR, intermediate risk: INT, and low risk: LOW) and chemotherapy regimens were correlated with the outcomes. RESULTS: Recurrences developed in 34 out of 288 (11.8%) patients with Wilms' tumor treated in accordance with International Society for Pediatric Oncology 2001 (SIOP 2001) protocols. Of these 34 patients, 11 initially had CS I, seven were at CS II, four were at CS III, 11 were at CS IV and one had CS V. There were eight patients with second recurrences; of these, seven were in the INT risk group and one in the high histological risk group. There was no correlation between age (p=0.256) or gender (p=0.538) and the risk of tumor recurrence. In the study group, seven out of 10 patients with local recurrences are alive; as are 13 out of 22 patients with distant recurrences (p=0.703). Those who died due to disease progression comprised six out of 26 patients with a first recurrence (four HR, two INT), and seven out of eight with a second recurrence (one HR, six INT). CONCLUSIONS: The prognosis after relapse in initially metastatic patients did not differ from that in patients who had primarily localized disease. The pathology variants probably had more significance.

Therapeutic results in stage I Wilms' tumors in children - 15 years of surgical experience.

INTRODUCTION: Progress in the therapeutic management of Wilms' tumors (WT) in children has resulted in the fact that presently, almost all children with stage I and favorable histology diseases have a chance for a cure. THE AIM OF THE STUDY: The objective of the report is an analysis of therapeutic results in children with unilateral stage I Wilms' tumors depending on the extent of tumor resection. MATERIAL AND METHODS: The analysis included children treated between 1993 and 2008 under the Polish Wilms' Tumor Study, initially following the protocol SIOP 93-01 (patients treated up to 2002) and subsequently SIOP 2001. Following neoadjuvant chemotherapy, depending on tumor size and tumor response to treatment, the children were qualified for a radical nephrectomy, simple nephrectomy, or nephron-sparing surgery. Following surgical treatment, all the children were subjected to adjuvant chemotherapy in keeping with the therapeutic protocol obligatory at the time. RESULTS: Within the 15 years, 111 children with Wilms' tumor were treated; the group included 43 children with stage I disease, which constituted 38.9% of the total number. Radical nephrectomies were performed in three (6.98%) children, simple nephrectomies in 32 (74.42%), and nephron-sparing surgery in eight (18.6%). Regardless of the employed surgical treatment modality, all the children are alive and none have demonstrated recurrent disease. The mean post-treatment completion follow-up period is 73 months (median value, 68 months). CONCLUSION: A simple nephrectomy and, whenever possible, nephron-sparing surgery represents sufficient treatment in 93% of children with stage I Wilms' tumor. To improve qualification for surgical treatment we propose the employment of NSS for stage Ia Wilms' tumors and SN for stage Ib Wilms' tumors.

[Current diagnostic criteria of PHACES syndrome]. / Aktualne kryteria rozpoznania zespolu PHACES.

The PHACES syndrome refers to coexistence of the Posterior fossa brain malformations, (Segmental Haemangiomas) of the face with (Arterial anomalie) and midline raphe defects (Sternal defects or Supraumbilical raphe). The diagnostic criteria have not been precisely established and are still discussed by the experts. The authors present current knowledge on PHACE syndrome and discuss suggested diagnostic criteria of PHACE syndrome.

[Nephroblastoma in children aged less than 6 months at diagnosis]. / Nowotwory nerek u noworodków i niemowlat w pierwszych 6 miesiacach zycia.

UNLABELLED: We present the results of treatment of kidney tumours in newborns and infants aged less than 6 months, in the years 1993-2000, from the Nephroblastoma Committee of the Polish Paediatric Group of Solid Tumours (PPGGL). We have analysed the diagnostic and treatment results in the group of 31 children aged 0 to 6 months. For 19 children registered between 1993 and 1996, event-free survival (EFS) and overall survival (AS) were assessed. Among 450 children registered between 1993 and 2000 by PPGGL and treated for kidney tumours, there were 31 (7.1%) newborns and infants aged below 6 months. The accuracy of diagnosis based on imaging studies was 97%. Only in one child the initial diagnosis of kidney tumour was not confirmed; cystic degeneration of kidney was finally established. The tumours removed during surgery were small, with average size 213 cm3, and in half of the cases the size of the tumour did not exceed 165 cm3. Primary complete excision of the tumour was performed in 21 children (67.7%). In 10 cases histopathology confirmed mesoblastic nephroma, in 19 cases nephroblastoma and in 2 cases sarcoma clarocellulare. In 10 infants (32.2%) with nephroblastoma delayed surgery preceded by chemotherapy was performed. Indications for initial preoperative chemotherapy comprised: tumour in a single kidney, tumour in a horseshoe kidney, preoperative diagnostic biopsy of the tumour and large tumour in neonates older than 3 months. In almost 70% of the children the stage of advancement was low (stage I and IIN-). Histopathology of excised tumours confirmed in 42% of cases low risk, and in 51.6% intermediate risk. Intraoperative complications occurred in 5 infants (16%). The tolerance of reduced chemotherapy by the infants was good. AS was 100%. ESF for the 19 children registered for nephroblastoma between 1993 and 1996 for all stages of advancement and types of histology was 94.75%. CONCLUSIONS: 1) Mesoblastic nephroma and low risk nephroblastoma are the most common tumours in children within the first three years of life. 2) The results of treatment of nephroblastoma in the youngest children (below 6 months of age) are the most favourable and represent world standards.3) Surgical complications in children operated primarily for nephroblastoma indicate the need of performing such operations in academic centres, specialised in newborn surgery. 4) In infants with extensive kidney tumours older than 3 months, primarily considered as inoperative, individual induction chemotherapy should be taken into account.

[Bilateral Wilms' tumour. Results of treatment in 29 children]. / Nerczak obustronny: wyniki leczenia 29 dzieci.

Retrospective analysis of 29 children with bilateral Wilms' Tumour, 14 boys and 15 girls, aged 4m-5 years, from regional centres of paediatric oncology in Poland, treated according to the O1-92 PPGL Protocol of Wilms' tumour, was performed. All patients were treated with two (ACT + VCR) or three (ACT, VCR, DOX) cytostatic drugs preoperatively, and 26 were operated upon after cytoreductive pretreatment. In 12 children nephrectomy was performed. In 28, kidney sparing surgery was possible and made with success. 2 patients who presented disseminated disease and 1 with nephroblastomatosis were given chemotherapy. 19 out of 29 (65.5%) are alive including 1 patient with nephroblastoma. EFS calculated according to Kaplan-Meier analysis for this group, was 58% at 67 months.