[Nephroblastoma -  30-years period of its treatment in the University Hospital Motol, Prague]. / Nefroblastom -  30 let lécby ve Fakultní nemocnici v Motole.

INTRODUCTION: Nephroblastoma (Wilms tumor - WT) is the most common solid tumor of kidney in children. We present treatment development of WT at the Department of Pediatric Hematology and Oncology, Charles University in Prague, 2nd Faculty of Medicine and University Hospital Motol (KDHO) in the Czech Republic over 30 years. Patients that were treated prior to access to the International Society of Pediatric Oncology (SIOP) protocols are considered to be the historical group, then we have patients treated according to SIOP 9, SIOP 93-01 and SIOP 2001 protocols as full participants of SIOP studies. PATIENTS AND METHODS: Between January 1980 and April 2009, we treated 330 patients with WT at KDHO: 91 patients in historical group (1980-1988), 94 pts in SIOP 9 (1988-1993), 80 pts in SIOP 93-01 (1994-2001) and 65 pts in SIOP 2001 (2002-2009). Overall survival (OS) and event-free survival (EFS) were analyzed by Kaplan-Meier test. RESULTS: The overall ten-year EFS was 81.2% and OS 87.6%. Fifty-eight patients from the 330 (17.6%) had metastases at diagnosis, EFS without metastatic process was 84.6% compared to 65.4% with metastasis presented at diagnosis (p = 0.0003), OS was 70.7% compared to 91.2% (p < 0.0001). One hundred and seventy patients (51.5%) were treated with preoperative chemotherapy and/or radiotherapy, whereas 158 patients (47.5%) underwent primary nephrectomy; EFS and OS did not differ: neoadjuvant vs primary nephrectomy EFS was 81.2% vs 80.9% (p = 0.85), OS 89.4% vs 85.4% (p = 0.38). Sixty (18%) patients experienced disease recurrence; OS after relapse was 33%. In the historical group, EFS and OS were 85.7% and 91.2%. In patients treated according to the SIOP 9 protocol, EFS and OS were 68.1% and 74.5%, resp. In patients treated according to SIOP 93-01, it was 83.6% and 93.7%, resp. and in patients treated according to 87 SIOP 2001, it was 7% and 95.4% (p = 0.001 and p = 0.0008), resp. CONCLUSION: WT is a well treatable disease. The aim for the future is to maintain the current very good survival while minimizing the treatment intensity.

Surgical tactics in the treatment of malignant renal tumors in childhood.

The authors report the treatment results of 89 children with renal tumors (excluding carcinoma) seen from 1988 to 1992, 71 (79.8%) with favorable-histology Wilms' tumor (survival 86%) and 18 (20.2%) with unfavorable histology, including anaplastic nephroblastoma, clear-cell sarcoma, and rhabdoid tumor (survival 61%). Preoperative chemotherapy (ChT) was given to 46 patients (survival 84%); none was given to 43 with either tumors in the 1st year of life, massive hematuria, or tumor growth during ChT (survival 78%). There were 3 tumor ruptures in the latter group compared to 1 in the former group.

[Diagnostic imaging methods in neonates with diseases of the urinary tract]. / Uplatnení diagnostických zobrazovacích metod u novorozencu s onemocnením mocových cest.

The actually used examination algorithm of imaging methods was checked in 35 neonates examined in the course of two years (1991 and 1992) on account of suspected diseases of the urinary pathways. It involved sonographic examination of 28 children, voiding cystourethrography (34x) and intravenous urography (18x). Ascendent pyelography and irrigography were performed three times, one neonate was examined by computed tomography. The procedure used as a basic sonographic prenatal screening in 11 children, in 20 clinical suspicion of disease of the urinary pathways, and four neonates were examined on account of an anorectal malformation or abnormality of the external genitalis. The most frequent finding--in 18 children--was hydronephrosis. It was associated with signs of subrenal obstruction (8x), with ren duplex (5x) and with a megaureter (6x). The association of the sonographic finding of dilatation of the intrarenal urinary pathways with absent contrast filling during excretory urography was observed in a child with hydronephrosis and subrenal obstruction and in four children with a multicystic dysplastic kidney. In individual cases the authors detected a valve of the posterior urethra, a sigmoid-vesical fistula, agenesis of the penis and stenosis of the urethra. Eleven neonates had a normal findings of the urinary tract.

[Disseminated histiocytosis X]. / Diseminovaná histiocytosis X.

Three cases of 4-year-, 16-month- and 8-month-old children suffering from a disseminated form of histiocytosis X showed overlapping of Hand-Schüller-Christian syndrome and Letterer-Siwe syndrome in clinical picture, biopsy and autopsy. Morphological lesion does not develop simultaneously in all the organs. Some of them tend to steatosis and fibrosis of histiocytosis infiltrations, which the others do not.