Diagnosis and treatment of renal cell carcinoma in children: a report from the Polish pediatric rare tumor study group.

BACKGROUND: Renal cell carcinoma (RCC) in children is rare, accounting for approximately 1.9-6% of all pediatric renal malignancies. The aim of this study was to transmit our experience in the treatment of RCC in Polish children. METHODS: Clinical data from 21 children (6.3-18 years old) with RCC treated between 1992 and 2009 at Polish pediatric oncological centers were analyzed. RESULTS: In 2 patients, RCC developed as a second malignancy after neuroblastoma or astrocytoma fibrillare, respectively. In 6 patients, initial diagnoses based on imaging studies were unilateral Wilms' tumor, leading to preoperative chemotherapy. The remaining patients underwent surgery at the beginning of treatment. According to the AJCC/TNM staging system, 14 patients had stage I, 5-II, 1-III, and 1-IV. Nephrectomy was performed in 19 patients, heminephrectomy in one, and biopsy in another. Histopathological diagnoses were clear-cell RCC (18 patients), papillary RCC (2 patients), and chromophobe RCC (1 patient). 10 patients were treated with chemotherapy, with or without IL-2, INFα, and antiangiogenic agents. 2 patients died due to disease progression. CONCLUSIONS: RCC in children is mostly operable at diagnosis, resulting in good prognosis. The role of adjuvant chemo- and immunotherapies is unclear. Neoadjuvant chemotherapy proven for children with Wilms' tumors is ineffective, but the delay in adequate therapy did not worsen the outcome if complete nephrectomy is done subsequently.

Extrarenal Wilms tumour.

INTRODUCTION: Nephroblastoma is one of the most common solid tumours in children. It also is the most frequent tumour found in the kidneys. In 5 % of cases it affects both kidneys at the same time. About 70 - 80 new cases of Wilms tumour are registered in Poland annually, usually in patients aged from 1 to 7 years. Extrarenal Wilms tumours are extremely rare. Due to its rarity, series with more cases are based upon material collected from many clinical centers. AIM: We would like to present a case of a boy in whom we diagnosed nephroblastoma in the retroperitoneal space 14 years after he had completed a complex therapy for bilateral Wilms tumour. CONCLUSION: The development of an extrarenal tumour 14 years after complex treatment for bilateral nephroblastoma is related to the survival of metanephros located outside the kidney.

[Results of the treatment of myelodysplastic syndrome (MDS)obtained by the Polish Paediatric Leukaemia /Lymphoma Study Group]. / Wyniki leczenia zespolów mielodysplastycznych (MDS) uzyskane przez Polska Pediatryczna Grupe ds Leczenia Bialaczek i Chloniaków u dzieci.

Sixty children with MDS treated in six centres of the Polish Paediatric Leukaemia/Lymphoma Study Group in the period 1975-1999 were included in the study. In 20 children RAEB-T, in 13 RA, in 21 RAEB and in 6 CMML were diagnosed. Our own and literature data showed that BMT is the best therapy for children with MDS. We need a new comprehensive protocol for the diagnosis and treatment of children with MDS in Poland.

[Treatment outcome for myelodysplastic syndromes (MDS) obtained by the Polish Children's Leukemia/Lymphoma Study Group]. / Wyniki leczenia zespolów mielodysplastycznych (MDS) uzyskane przez Polska Grupe ds Leczenia Bialaczek i Chloniaków u Dzieci.

Fourty children with MDS treated in seven centres of The Polish Children's Leukemia Lymphoma Study Group in period 1975-1998y were included to the study. In 16 children RAEB-T, in 2 CMML in 10 RA and in 12 RAEB were diagnosed. Our and literature data showed that BMT is the best therapy for children with MDS. For children, who don't have a donor for BMT. Roacutan therapy seems to be the most effective.

[How to improve the results of treatment of myelodysplastic syndromes in the studies of Polish pediatric leukemia/lymphoma group]. / Co zrobic dla poprawy wyników leczenia zespolów mielodysplastycznych w Polskiej Pediatrycznej Grupie ds. Leczenia Bialaczek i Chloniaków.

Fourty two children with myelodysplastic syndrome (MDS) treated in seven centres of The Polish Paediatric Leukaemia/Lymphoma Study Group in period 1975-1998 were included in the study. In 16 children RAEB-T, in 3 CMML, in 10 RA and in 13 RAEB were diagnosed. BMT is the best therapy for children with MDS. For children, who have not a donor for BMT, Roacutan therapy seems to be the most effective.

[The treatment of central nervous system neoplasms]. / Leczenie nowotworów osrodkowego ukladu nerwowego.

From August 1993 to March 1998 30 children (13 females and 17 males) at the age of 1-14 with central nervous system tumours were treated in Paediatric Hematology and Oncology Hospital in Wroclaw. In 11 patients we diagnosed medulloblastoma, in 4 ependymoma, 6 astrocytoma, 2 oligodendroglioma and in 7 other types of tumours. The histological diagnosis of 2 cases with tumours localized in the brain trunk was not available. All patients were directed to the chemotherapy after the surgery. Complete resection was achieved in 13 out of 30 patients. The chemotherapy was proceeded according to SIOP regimen. Children older than 3 years received additional radiotherapy. The effectiveness of treatment was controlled by constant neurological examinations with computed tomography and ultrasonography. The rate of failures was 10 patients with local recurrences and 1 patient with drug toxicity. 17 children remain still in observation--among them 5 are treated with chemotherapy and 2 present the progression of disease.

[Astrocytoma in a 6-year-old girl after the treatment for acute lymphoblastic leukemia]. / Astrocytoma u 6-letniej dziewczynki po leczeniu ostrej bialaczki limfoblastycznej.

In recent years we observe the increasing number of event-free survivals of patients with acute lymphoblastic leukaemia. It is achieved with multidrug chemotherapy regiment. The important part of protocols is prophylaxis e.g. with radiation therapy of CNS. It is followed by drug toxicity and side effects of treatment (e.g. secondary CNS tumours). In our hospital we observed the case of 6-year-old girl with CNS tumour-astrocytoma. According to the regimen for ALL the radiation therapy of CNS was performed. Three months after neurosurgical operation the local recurrence was observed. The patient died of the progression of disease.