Haemorrhagic cystitis as a complication after allogenic bone marrow transplantation.

OBJECTIVE: The main objective of the following work is to present our own material and the ways in which we have dealt with haemorrhagic cystitis (HC) following allogenic bone marrow transplantation in children. MATERIALS AND METHOD: From 1994 to 2002, allogenic transplantation of haematopoietic cells was performed in 129 children at the Oncological and Haematological Child Clinic, Wroclaw University of Medicine. The procedure was carried out in patients with neoplastic diseases. In 33 cases, HC symptoms of various intensity were observed. The intensity of the symptoms was evaluated according to Arthur's four-point scale. To confirm the diagnosis USG was carried out in each case. Special attention was given to the ultrasonographic structure of the bladder wall. Cartoni's technique was followed in the examination. RESULTS: Out of 129 children who underwent allogenic transplantation of haematopoietic cells 33 (20.75 %) revealed HC symptoms. The symptoms occurred between the 2nd and the 124th day after transplantation (mean 29 days). The treatment included antiviral medicines, estrogens, reduction of immunosuppression and mechanical urological procedures. The children diagnosed with 2nd grade disease and higher were catheterised and diuresis was forced by the administration of larger amounts of liquids intravenously. Antihaemorrhagic drugs and vitamin K were also given. Blood was substituted if needed as were blood derivatives. Eighteen children with massive haematuria with clots underwent catherisation with a suprapubic catheter so as to continuously rinse the bladder. In 8 cases tamponade of the bladder occurred. The clots were removed from the bladder during cystoscopy under general anaesthesia. Twelve children died from HC. This amounts to 36 % of all the cases identified as HC and 9 % of all the children who underwent allogenic marrow transplantation. CONCLUSIONS: In conclusion it must be emphasised that HC in children after allogenic transplantation of haematopoietic cells is an extremely severe disease, which, if not cured, is terminal. The decision whether to perform embolisation of internal iliac arteries or to remove the bladder when non-radical methods have been exhausted, is worth considering.

Malignant tumours in a horseshoe kidney in children: a diagnostic dilemma.

INTRODUCTION: Although malignant tumours in a horseshoe kidney are fairly common, both diagnosis and treatment are often difficult. The most frequently described tumour in children with this anomaly is nephroblastoma, but renal cell carcinoma and others can also occur. More than a hundred such cases have been presented in the English literature on the subject since Hildebrandt described nephroblastoma in a horseshoe kidney for the first time in 1895. Many of the articles report diagnostic and therapeutic problems. AIM: Our intention is to describe five more cases from our own experience. The cases involve malignant tumours in a horseshoe kidney. MATERIAL: Three children suffered from nephroblastoma, the other two had RCC. The tumours were particularly noted out of a total of 500 cases of nephroblastoma and 22 cases of other malignant tumours treated in Poland between 1993 and 2000. We also report here the treatment outcome of one case treated 30 years ago in our Clinic. CONCLUSION: Diagnostic and surgical procedures involved in the treatment of malignant tumours in the horseshoe kidney are complex and challenging. Only a few oncological centers cooperating with child urologists can provide comprehensive and effective treatment.

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.

[The value of the ultrasonography and computed tomography in diagnosis and monitoring of treatment of renal abscesses in children]. / Wartosc ultrasonografii i tomografii komputerowej w rozpoznawaniu i monitorowaniu leczenia ropni nerek u dzieci.

A retrospective study of 14 patients from 2 to 13 years old with renal abscess was performed. We evaluated the diagnostic value of ultrasonography (US) and computed tomography (CT) in diagnostic investigation and treatment of renal abscess, which have changed during the past ten years from classic surgical treatment to percutaneous drainage. US and CT have revealed the diagnosis by showing a hypoechogenic or hypodense mass in all 14 patients. All had an intensive course of antibiotic treatment. In 10 of them the abscess were drained percutaneously by US guided drainage. Percutaneous drainage was unsuccessful in 2 patients, who subsequently underwent classic surgical exploration, incision and drainage for uncontrolled infection. We conclude that US and CT greatly facilitate the diagnosis and permit the percutaneous drainage of renal abscess in paediatric age group, which benefits from this minimally invasive therapy.

Primary nephrectomy for emergency: a rare event in the International Society of Paediatric Oncology Nephroblastoma Trial and Study no. 9.

Experience of the International Society of Paediatric Oncology (SIOP) Trials and Studies indicates that the preoperative chemotherapy in Wilms' tumour improves stage distribution, decreases complication rate and reduces postoperative treatment. However, some situations may lead to prompt primary surgery. The aim of the study is to assess reasons leading to primary emergency nephrectomy. Records of 720 patients with non-metastatic unilateral nephroblastoma who were registered in the SIOP Trial and Study 9 were reviewed. Twenty-four (3%) cases of primary emergency nephrectomy were identified. Reasons leading to emergency nephrectomy were massive bleedings from ruptured tumours in 13 patients, suspicion of an "acute abdomen" in 7, bowel occlusion in 2 and other in 2. Postoperative treatment included radiotherapy in 71% of cases and anthracyclines in 92%. Complications were frequent and happened in 25% of patients, the outcome however, was favourable and 22 of 24 patients are alive (from 9 to 79 months). The 7 patients with a suspicion of an "acute abdomen" probably constitute the group which could have been markedly reduced if adequately diagnosed and observed prior to surgery.

Multicenter retrospective analysis of various primary pediatric malignant hepatic tumors--management in a series of 47 Polish patients (1985-1995).

Forty-seven children treated in various Polish centers between 1985 and 1995 for primary malignant liver tumors were retrospectively analyzed. Hepatoblastoma (HB) prevailed--it was found in 39 cases. There were 6 hepatocarcinoma (HCC) cases and 2 cases of undifferentiated sarcoma (UDS). In 44% of HB patients the tumor involved both liver lobes. 18% of children with HB presented with pulmonary metastases at diagnosis. Chemotherapy was applied in 92% of cases (preoperatively in 67%). Tumor resection was performed in 56% of HB patients. Overall survival of patients with hepatoblastoma was 43.6%, while it was 50% for hepatocarcinoma and 100% for undifferentiated sarcoma (2 cases only). Mean observation time was 58 months. The hepatoblastoma subgroup, being the largest (83% of all cases), was analyzed separately for prognostic factors. Completeness of tumor excision strongly influenced survival. Involvement of both lobes of the liver and multifocality of the tumor were other adverse prognostic factors.

Screening for germline p53 mutations in pediatric and adult patients of high-risk groups in Poland.

Germline mutations of the p53 gene lead to cell transformation in various tissues. Such a complex cancer phenotype makes it difficult to recognize the carriers of the defective allele. Several studies undertaken to identify high-risk groups found germline p53 mutations in familial cancer aggregations and in patients with multiple tumors. We screened 189 pediatric and 48 adult patients. The high-risk groups comprised 41 patients with a family history of cancer and 35 with multiple neoplasms. Furthermore, 124 tumors were screened for somatic mutations. p53 exons 2 to 11 were analyzed by polymerase chain reaction and single strand conformation polymorphism (PCR-SSCP) followed by direct sequencing of abnormal DNA fragments. No germline p53 mutations were found and somatic mutations were detected in 5 of 59 sarcomas, globally, in 8 of 124 tumors. In conclusion, in Poland, p53 alterations do not seem very important for the predisposition to malignancy and development of sarcomas.

Venoocclusive liver disease (VOD) as a complication of Wilms' tumour management in the series of consecutive 206 patients.

UNLABELLED: In 4 years (1993-1996) 206 pts. with nephroblastoma were treated. All children were treated according to SIOP 93-01 protocol. Overall survival was 92%. In 27 cases hepatotoxic events occurred. In 10 cases, venoocclusive liver disease (VOD) was diagnosed. VOD is a syndrome associated with hepatomegaly, sudden weight gain or ascites and jaundice. It results from damage to the endothelium of hepatic venules and necrosis of central hepatocytes with subsequent proliferation of fibrous tissue and occlusion of the central hepatic veins. Dactinomycin is one of the drugs considered responsible for its development. Mean age of VOD patients was 4 yrs, however 3 of them were below 1 yr. In all cases, VOD occurred during postoperative chemotherapy (mean 16 th week of treatment). All patients received dactinomycin and vincristine. Five children with right kidney tumors underwent post-operative abdominal irradiation. Main VOD symptoms were hepatomegaly and ascites (80%). Hypertransaminasaemia, as well as, on ultrasound, gallbladder wall thickening and/or free abdominal fluid were observed. Median VOD duration was 27 days and its course was usually temporary and self-limiting. However, in 2 cases recurrent VOD episodes were noted. All children received supportive treatment only. In 6 cases, VOD resulted in chemotherapy delay or drug reductions, while in 4 others chemotherapy was completed preliminarily. Nevertheless it did not affect patients' outcome overall survival in VOD group was 90%. CONCLUSIONS: Total 5% VOD frequency is similar to other reports. Infants and children receiving abdominal irradiation seem to be at special risk of VOD development.

[High risk Wilms' tumor in children. Clinico-pathological correlation based on 320 patients registered in the Polish Paediatric Solid Tumor Study Group]. / Nerczak plodowy u dzieci z Wysokim ryzykiem zlosliwosci. Korelacje kliniczno-patologiczne u 320 dzieci zarejestrowanych w Polskiej Pediatrycznej Grupie ds. Leczenia Guzów Litych.

The records of 320 patients treated for Wilms' tumour in the first Wilms' Tumour Study (01-92 schedule) were reviewed and 42 children (13,86%) with unfavourable histology (UH) tumours were identified. There were 18 boys and 24 girls. Diffuse anaplasia was found in 26 patients (61,9%), focal anaplasia in 10 children (23,8%), CCSK and MRT were diagnosed in 3 patients each (7,1%). Clinical stages were: CS I - 5 (11,9%) patients, CS II N(-) - 7 (16,7%), CS II N(+) - 9 (21,4%), CS III - 15 (35,7%), CS IV - 5 (11,9%) and CS V - 1 patient (2,4%). Local and metastatic relapses of the disease occurred in 18 patients (43%). Seven of the 42 patients died, in 2 cases due to complications and in 5 from progression of the disease.

Nodular goiter and thyroid carcinoma in children and adolescents in a moderate endemic area (lower Silesia-Sudeten endemia) in the last twelve years.

Although thyroid carcinoma is more common in the adult population, the risk of a nodule being malignant is greater in children. The aim of our present investigation was to ascertain the percentage of malignancy in nodular goiter observed in patients from the Lower Silesia region in the last 12 years. The examination included 60 children (12 boys and 48 girls) treated in our clinic from 1987 to June 1998. Age varied from 7 to 18 years (mean 14.8 +/- 2.4), most of them in the age group between 13 and 18 years. The following investigations were performed: TSH, T3, T4, thyroid ultrasonography, fine needle aspiration biopsy and Tc99 scintigraphy of the thyroid. Most of the patients were euthyroid; two children demonstrated pressure symptoms. All the patients were treated by operation. Histological examinations revealed the following: nodular goiter in 19 patients, cystic nodular goiter in 5, follicular adenoma in 20, fetal adenoma in 3, nodular goiter and follicular adenoma in 6, papillary carcinoma in 6, and follicular carcinoma in 1 patient. We concluded that an increased incidence of thyroid cancer has been noted in children with nodular goiter in Lower Silesia during the last 12 years. Thyroid cancer was observed mostly in patients with single nodules and was associated with a high risk of malignancy.

Rarity of surgical complications after postchemotherapy nephrectomy for nephroblastoma. Experience of the International Society of Paediatric Oncology-Trial and Study "SIOP-9". International Society of Paediatric Oncology Nephroblastoma Trial and Study Committee.

The aim of the study was to assess rates and types of nephrectomy-related complications in children nephrectomized for nephroblastoma after preoperative chemotherapy. Records of 598 Wilms' tumour patients registered in the International Society of Paediatric Oncology Trial & Study No. 9 (SIOP-9), and pretreated correctly according to the protocol with vincristine + actinomycin D +/- epirubicine or adriamycin prior to nephrectomy, were retrospectively reviewed. Forty-nine patients (8%), who suffered from 54 complications, were identified. Most frequent events were small-bowel occlusions (3.7%) and tumour ruptures (2.8%). Other complications were registered in 2.0% of cases. The low rate of nephrectomy complications and no deaths related to registered ones, are another argument for preoperative chemotherapy in Wilms' tumour patients.

[The role of chemotherapy in treatment of nephroblastoma in children. Report of the Polish Pediatric Group re. treatments of solid tumors]. / Udzial chemioterapii w leczeniu nerczaka zarodkowego u dzieci. Raport Zespolu Polskiej Grupy Pediatrycznej ds. Leczenia Guzów Litych.

Retrospective analysis of chemotherapy results of children with nephroblastoma was performed in 220 patients aged from 1 yr to 14 yrs of live in 12 centers. Stage I nephroblastoma was documented in 24.5% but stage II--in 55.3%. Histologically 74.6% cases were diagnosed as medium malignant and 12.7%--high malignant. Therapy results were similar to observed in other centers.

[Renal neoplasms in children with tuberous sclerosis]. / Nowotwory nerek u dzieci ze stwardnieniem guzowatym.

Two cases of 14 and 11 years old children with tuberous sclerosis were admitted to Department of Paediatric Surgery in Wroclaw and Department of Paediatric Hematology and Oncology in Lublin because of renal tumors. Radical nephrectomy was performed in both cases. Histopathological diagnosis revealed malignant rhabdoid tumor (MRT) in a 14 years old boy and benign angiomyolipoma in the latter case. The authors stress the rarity of MRT in older children and successful treatment due to nephrectomy and maintenance chemotherapy.

[Atypical sarcomas localized within a head and neck in children]. / Nietypowe miesaki wieku dzieciecego zlokalizowane w obrebie glowy i szyi.

Seven children treated surgically because of non-rhabdomyosarcomatous soft tissue sarcoma (NRSTS) localized extrameningeally on the head and neck were presented. Three of the patients were operated on haemangiopericytoma, two--fibrosarcoma, one child--neurofibrosarcoma and one--liposarcoma. The pre- and postoperative TNM classification was employed as a staging system. The surgical resectability--R was used to establish tumour margins. Four patients (two with fibrosarcoma and two with haemangiopericytoma) survived free of disease. The influence of the complete surgical resection on the outcome of the head and neck extrameningeal NRSTS was proved.

[Early and late complications of the use of life-cath type catheters in children with malignancies requiring urgent surgical intervention]. / Wczesne i pózne powiklania stosowania cewników typu life-cath u dzieci z choroba nowotworowa, wymagajace pilnej interwencji chirurgicznej.

Permanent access to the centrally positioned veins is necessary for the treatment of children with malignancy. The Broviac-Hickman's catheters and venous port system fulfill this require. Since January 1992 to February 1998, 276 intravenous life-cath devices were installed. In 7 patients life-threatening complications after catheter implantation occurred, which demanded urgent surgical intervention. The complications appeared in both early and late periods after catheter insertion. Pneumothorax (three children), bleeding to the mediastinum (one patient), fragmentation, disconnection and migration of a separate part of catheter into the right heart (two patients) were observed. The way of treatment and its results were described.

[Pigmented nevus in children as a diagnostic and treatment problem]. / Znamiona barwnikowe u dzieci jako problem diagnostyczny i leczniczy.

Congenital and acquired nevi are common cutaneous malformation occurring in childhood. 315 children were cured in 1990-1995. 62% of nevi did not show any abnormalities of growth pattern and typical appearance. In 119 nevi there were danger signals present, that suggested unusual activity or possible malignant transformation. Changes of pigmentation were present in 36%, enlargement and abnormal texture in 21%, satellite nevi in 11%. Histologically, they were dermal in 28% and epidermodermal in 24%. Only in 3 children melanoma malignum was found, in 2 melanoma juvenile, in 4 dysplastic nevi. All of the children were operated. The very good final result was obtained in 289 children (92%).

[Concurrent goiter and vitiligo in a 13-year old boy]. / Wspólwystepowanie wola koloidowego oraz bielactwa nabytego u 13-letniego chlopca.

The coincidence of goitre and vitiligo in 13-year-old boy is reported. Skin changes appeared two years before the thyroid disease was diagnosed. Immunological investigation revealed the presence of anti-thyroid antibodies, which may suggest an autoaggressive etiology of both disorders.

Paediatric surgical oncology. 5--Nephroblastoma. International Society of Paediatric Oncology (SIOP) Nephroblastoma Trial & Study Committee.

The favourable results from the treatment of Wilms' tumour are an example of the success of multimodal therapy in paediatric oncology. The epidemiology, methods of diagnosis, benefits of pre-operative chemotherapy, basic principles of surgery and post-operative treatment modalities are presented. The approach to the management of Wilms' tumour considered in this paper is mainly that of the International Society of Paediatric Oncology.