Congenital mesoblastic nephroma 50 years after its recognition: A narrative review.

Congenital mesoblastic nephroma (CMN) is a rare pediatric renal tumor with low malignant potential that most commonly occurs early in infancy. Treatment strategies are based on the few published CMN series, while a significant number of CMN patients have been described in case reports. The aim of this narrative review was to create an up-to-date overview of the literature. Complete surgical removal is curative in most cases. The risk of treatment-related mortality (both surgery- and chemotherapy-related) is relatively high in the first weeks of life, indicating that these young patients deserve special attention with respect to timing and type of treatment.

Treatment and outcome of patients with relapsed clear cell sarcoma of the kidney: a combined SIOP and AIEOP study.

BACKGROUND: Clear cell sarcoma of the kidney (CCSK) is an uncommon paediatric renal tumour. Relapses occur in about 15% of the patients. Since detailed clinical information on relapsed CCSK is scarce, the current study aims to describe outcome of patients with relapsed CCSK treated according to recent European protocols. PATIENTS AND METHODS: We analysed prospectively collected data of all CCSK patients who developed a relapse after complete remission at the end of primary treatment, entered onto SIOP and AIEOP trials between 1992 and 2012. RESULTS: Thirty-seven of 237 CCSK patients (16%) treated according to SIOP and AIEOP protocols developed a relapse. Median time from initial diagnosis to relapse was 17 months (range, 5.5 months - 6.6 years). Thirt-five out of thirty-seven relapses (95%) were metastatic; the most common sites of relapse were the brain (n=13), lungs (n=7) and bone (n=5). Relapse treatment consisted of chemotherapy (n=30), surgery (n=19) and/or radiotherapy (n=18), followed by high-dose chemotherapy and autologous bone marrow transplantation (ABMT) in 14 patients. Twenty-two out of thirty-seven patients (59%) achieved a second complete remission (CR); 15 of whom (68%) developed a second relapse. Five-year event-free survival (EFS) after relapse was 18% (95% CI: 4%-32%), and 5-year overall survival (OS) was 26% (95% CI: 10%-42%). CONCLUSIONS: In this largest series of relapsed CCSK patients ever described, overall outcome is poor. Most relapses are metastatic and brain relapses are more common than previously recognised. Intensive treatment aiming for local control, followed by high dose chemotherapy and ABMT, seems to be of benefit to enhance survival. Novel development of targeted therapy is urgently required.

Rare cancers in children - The EXPeRT Initiative: a report from the European Cooperative Study Group on Pediatric Rare Tumors.

The low incidence and the heterogeneity of very rare tumors (VRTs) demand for international cooperation. In 2008, EXPeRT (European Cooperative Study Group for Pediatric Rare Tumors) was founded by national groups from Italy, France, United Kingdom, Poland and Germany. The first aims of EXPeRT were to agree on a uniform definition of VRTs and to develop the currently most relevant scientific questions. Current initiatives include international data exchange, retrospective and prospective studies of specific entities, and the development of harmonized and internationally recognized guidelines. Moreover, EXPeRT established a network for expert consultation to assist in clinical decision in VRTs.

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.

Angiosarcoma in children - still uncontrollable oncological problem. The report of the Polish Paediatric Rare Tumours Study.

Angiosarcoma in children - still uncontrollable oncological problem. The report of the Polish Paediatric Rare Tumours StudyAngiosarcoma is a rare, highly malignant vascular neoplasm with little data available on its clinical course and management in children. Ten children with angiosarcoma (M/F: 6/4; aged 2, 3-16 years) registered in Polish Paediatric Rare Tumours and Soft Tissue Sarcomas Studies between 1992 and 2006. Primary tumour exceeded 5 cm in seven patients and affected mainly deep tissues (heart-2, head/neck, bladder, brain, liver and upper limb - one patient each). Four patients had regional and two metastatic diseases (lungs and bones). Three patients were initially misdiagnosed as haemangioma. Complete primary excision was unfeasible even in local stages. All patients received supplementing chemotherapy with no response in four. Radiotherapy was given to five children, including three after relapse. Three of five secondary tumour resections proved complete. Seven patients experienced relapses (mainly metastatic) and two continuous progression. Relapsed patients received chemotherapy +/- radiotherapy and surgery (three). Nine patients died of disease (overall survival 6-66 months), and one child after mutilating secondary resection is alive. Angiosarcoma in children is highly aggressive with an extremely poor prognosis. Complete primary excision is unfeasible, even in seemingly local stages. The response to chemotherapy is poor and the large number of metastatic recurrences suggests a need for systemic therapy modifications.

Prognostic significance of age in 5631 patients with Wilms tumour prospectively registered in International Society of Paediatric Oncology (SIOP) 93-01 and 2001.

BACKGROUND: To enhance risk stratification for Wilms tumour (WT) in a pre-operative chemotherapy setting, we explored the prognostic significance and optimal age cutoffs in patients treated according to International Society of Paediatric Oncology Renal Tumour Study Group (SIOP-RTSG) protocols. METHODS: Patients(6 months-18 years) with unilateral WT were selected from prospective SIOP 93-01 and 2001 studies(1993-2016). Martingale residual analysis was used to explore optimal age cutoffs. Outcome according to age was analyzed by uni- and multivariable analysis, adjusted for sex, biopsy(yes/no), stage, histology and tumour volume at surgery. RESULTS: 5631 patients were included; median age was 3.4 years(IQR: 2-5.1). Estimated 5-year event-free survival (EFS) and overall survival (OS) were 85%(95%CI 83.5-85.5) and 93%(95%CI 92.0-93.4). Martingale residual plots detected no optimal age cutoffs. Multivariable analysis showed lower EFS with increasing age(linear trend P<0.001). Using previously described age categories, EFS was lower for patients aged 2-4(HR 1.34, P = 0.02), 4-10(HR 1.83, P<0.0001) and 10-18 years(HR 1.74, P = 0.01) as compared to patients aged 6 months-2 years. OS was lower for patients 4-10 years(HR 1.67, P = 0.01) and 10-18 years(HR 1.87, P = 0.04), but not for 2-4 years(HR 1.29, P = 0.23). Higher stage, histological risk group and tumour volume were independent adverse prognostic factors. CONCLUSION: Although optimal age cutoffs could not be identified, we demonstrated the prognostic significance of age as well as previously described cutoffs for EFS (2 and 4 years) and OS (4 years) in children with WT treated with pre-operative chemotherapy. These findings encourage the consideration of age in the design of future SIOP-RTSG protocols.

Optimal duration of preoperative therapy in unilateral and nonmetastatic Wilms' tumor in children older than 6 months: results of the Ninth International Society of Pediatric Oncology Wilms' Tumor Trial and Study.

PURPOSE: To determine the optimal duration of preoperative chemotherapy to further increase the proportion of stage I tumors by comparison of two regimens in the treatment of patients older than 6 months who have unilateral Wilms' tumor. PATIENTS AND METHODS: Eligible patients (n = 382) initially received four weekly doses of vincristine (VCR) and two courses of actinomycin D (AMD) and were randomized either to be operated on (4-week group [n = 193]) or to receive 4 more weeks of the same chemotherapy regimen (8-week group [n = 189]). The assessment criterion was the observed percentage of stage I tumors. After surgery, patients were assigned according to tumor stage and histology to four different treatment groups: stage I and favorable histology (n = 5) were to have no further treatment (NFT); stage I and standard histology or anaplasia (n = 244), VCR and AMD for 17 weeks (AV); stages II and III and favorable or standard histology, VCR, AMD, and an anthracycline for 27 weeks (AVE) with no abdominal radiotherapy for stage II N0 disease (n = 75) or with a 15-Gy dose of abdominal irradiation (RTH) in case of stages IIN1 and III (n = 56). Anaplastic tumors staged higher than I or clear-cell sarcoma of the kidney (14), AMD, VCR, an anthracycline, and ifosfamide for 36 weeks (DEVI). RESULTS: No advantage was found in favor of prolonged preoperative treatment. The percentages obtained for the 4-week and the 8-week groups, respectively, were as follows: stage I, 64% versus 62%; intraoperative tumor rupture rate, 1% versus 3%; 2-year EFS, 84% versus 83%; and 5-year OS, 92% versus 87%. Two-year EFS and 5-year OS rates, respectively, of the different treatment groups were as follows: NFT, 100% for both EFS and OS; AV, 88% and 93%; AVE, 84% and 88%; AVE RTH, 71% and 85%; and DEVI, 71% and 71%. The rate of abdominal recurrences in stage II N0 nonirradiated patients was 6.6%. CONCLUSION: The 4-week schedule pre-nephrectomy chemotherapy regimen should be considered the standard treatment. Clinical trials should continue to improve the cure rate of high-risk patients and the quality of life of children with a more favorable prognosis.

Conservative treatment for girls with nonmetastatic rhabdomyosarcoma of the genital tract: A report from the Study Committee of the International Society of Pediatric Oncology.

PURPOSE: To report the results of a conservative multimodal approach in girls with nonmetastatic rhabdomyosarcoma (RMS) of the genital tract, treated in International Society of Pediatric Oncology (SIOP) Malignant Mesenchymal Tumors 84 and 89 protocols. PATIENTS AND METHODS: From 1984 to 1994, 38 girls with RMS of the genital tract (vulva, vagina, uterus) were treated in SIOP protocols. With the exception of patients with rare small tumors, which were resected at the start of the studies, all patients received initial chemotherapy (CHT) (ifosfamide, vincristine, and actinomycin D). Local treatment including surgery, brachytherapy (BT), and external-beam radiotherapy (ERT) was given only to girls who did not achieve complete remission (CR) with CHT or who subsequently relapsed. RESULTS: The primary tumor originated in the vulva or vagina in 27 girls and in the uterus in 11. The overall survival rate (+/- SE) was 91% +/- 6% at 5 years, and the event-free survival rate was 78% +/- 7%. At a median follow-up of 5 years, 30 girls were alive and in first CR and five were alive and in second CR. Four patients treated with complete resection of the tumor at diagnosis received less CHT. Thirteen patients were treated with CHT alone. In 17 patients, local treatment was necessary to achieve complete local control, for a residual mass after initial CHT (10 patients), for viable tumor on biopsy (three patients), or for local relapse (four patients). The local treatment used was radiotherapy (RT) (ERT in three patients, BT in seven), radical surgery with uterine ablation (three patients), RT and radical surgery (three patients), and conservative surgery with RT (one patient). CONCLUSION: Girls with nonmetastatic RMS of the genital tract have an excellent prognosis. We found no difference in outcome between uterine and vulvovaginal RMS. Local treatment does not seem necessary in patients who have a complete response to CHT. When a local treatment is needed, BT may be an alternative to radical surgery or ERT.

Reduction of postoperative chemotherapy in children with stage I intermediate-risk and anaplastic Wilms' tumour (SIOP 93-01 trial): a randomised controlled trial.

BACKGROUND: Present treatment for Wilms' tumour is very successful. Now, efforts are aimed at reducing toxicity and burden of treatment by shortening schedules without loss of effectiveness. The objective of this randomised trial was to assess whether postoperative chemotherapy for patients with stage I intermediate-risk and anaplastic Wilms' tumour could be shortened to only 4 weeks from the standard 18 weeks, while maintaining equivalent event-free survival. METHODS: Between June, 1993, and June, 2000, 410 patients were randomly assigned after four doses of vincristine plus one course of dactinomycin postoperatively either to stop further adjuvant chemotherapy (no further chemotherapy group, n=200), or to receive a further two courses of the same chemotherapy (standard group, n=210). Previous treatment consisted of chemotherapy before nephrectomy of four doses of vincristine and two courses of dactinomycin followed by surgical resection of the tumour. Eligible patients were at least 6 months old and had stage I tumours with either intermediate-risk histology or anaplasia. The primary endpoint of this equivalence trial was 2-year event-free survival. Both per-protocol and intention-to-treat analyses were done. FINDINGS: By 2 years, 18 recurrences were reported in the standard group, and 22 in the no further chemotherapy group. Event-free survival was 91.4% (95% CI 87.5-95.2) for the no further chemotherapy group and 88.8% (84.3-93.2) for the standard group (difference=2.6%, upper 97.5% confidence limit 8.4%). The null hypothesis, that experimental treatment is less effective than standard treatment, could be rejected (p=0.008). CONCLUSIONS: Shortening duration of chemotherapy could reduce acute and late side-effects and inconvenience for patient and parents while maintaining effectiveness, and could be beneficial in terms of health costs.

Outcome of localised blastemal-type Wilms tumour patients treated according to intensified treatment in the SIOP WT 2001 protocol, a report of the SIOP Renal Tumour Study Group (SIOP-RTSG).

Blastemal-type Wilms tumour (BT-WT) has been identified as a high risk histological subgroup in WT assessed after pre-nephrectomy chemotherapy in trials of the International Society of Paediatric Oncology (SIOP) Renal Tumour Study Group. Therefore, in SIOPWT2001, post-operative chemotherapy for BT-WT was intensified aiming to improve survival. Survival analysis of all unilateral BT-WT patients (SIOPWT2001) (n=238), was compared with historical BT-WT controls (SIOP93-01) (n=113). 351/4061 (8.6%) unilateral non-metastatic BT-WT patients (SIOP93-01/SIOPWT2001) were studied. Median age at diagnosis was 43 months (Inter Quartile Range (IQR) 24-68 months), stages: I (n=140, 40%), II (n=106, 30%), III (n=105, 30%). BT-WTs were higher staged, showed greater volume decrease after pre-operative chemotherapy and were diagnosed at an older median age compared to other WT patients. Patient characteristics did not differ substantially between SIOP93-01 and SIOPWT2001. Univariate analysis showed a 5-year event-free survival (EFS) of 80% (95% confidence interval (CI): 75-86%) (SIOPWT2001) compared to 67% in SIOP93-01 (95% CI: 59-76%; p=0.006) and overall survival (OS) of 88% (95% CI: 83-93%) (SIOPWT2001) compared to 84% (95% CI: 77-91%; p=0.4) in SIOP93-01. 95% of relapses were distant metastases (SIOP93-01/SIOPWT2001). Treatment protocol, age at diagnosis, tumour stage (III versus I/II) and volume (at surgery), were prognostic variables for EFS (uni- and multivariate Cox regression analysis). Independent prognosticators for OS were age at diagnosis, tumour stage and volume (at surgery). The most significant survival benefit of intensified treatment, was observed in Stage I (EFS 96% in SIOPWT2001 (OS 100%), 71% in SIOP93-01 (OS 90%)). BT-WT derived benefits from more intensive chemotherapy as reflected by a reduction in relapse risk. However, the benefit of the more intensive chemotherapy to improve OS was only observed in stage I BT-WTs, by adding doxorubicin.

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.

Midfacial fractures in children.

The purpose of the study was to present a modern classification and discuss the treatment of midfacial fractures in children. From the beginning of 1 January 1998 to 31 October 2000, 147 children were treated for different craniofacial fractures. Among them 28 patients (19 %) had extensive midfacial fractures complicated by impaired vision and/or CNS dysfunction and were treated surgically. The fractures were divided into: zygomatico-orbital (1 pt) and zygomatico-orbito-maxillary (5 pts), isolated orbital wall fractures (14 pts) and naso-orbital dislocations (2 pts), upper facial portion dislocations (2 pts) and fronto-naso-orbital (2 pts) or cranio-orbital fractures (2 pts). Clinical examination revealed mainly dysfunction of facial morphology and aesthetics (enophthalmos, telecanthus), CSF leakage, impairment of vision and restricted eyeball movements. Ophthalmological, neurological and radiological (Waters view, CT, NMR) examinations were performed in all cases. The treatment consisted of bone repositioning, reconstruction with autologous bone grafts and three-dimensional stabilisation using titanium plates. Twenty-three pts were operated on before and 5 pts after 7th day after trauma. The restoration of normal facial morphology was achieved in 26 patients. In 2 cases it was impossible because of the destruction of soft tissue and an extremely extensive bony trauma. Better reposition of bony fragments, prompt healing and better final results were achieved in patients operated before day 7 after trauma. Achieving good results in midfacial fractures requires prompt and adequate treatment and a multidisciplinary (paediatric, maxillofacial, plastic surgeons, neurosurgeon, ophthalmologist) team.

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.

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.

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.

[Update on kidney tumors in children: role of the pediatric surgeon]. / Actualités dans les affections tumorales du rein del'enfant: rôle du chirurgien pédiatre.

The surgical treatment of malignant renal tumours in children (nephroblastomas) is part of a multimodal therapeutic strategy, defined by the International Society of Paediatric Oncology protocol. Resection of the primary tumour and determination of the post-operative stage determine subsequent treatment. The modalities of the surgical procedure are described together with the particular cases concerning infants under the age of 6 months, extension to the renal vein and inferior vena cava, emergency surgery, bilateral, tumours or tumour in a solitary kidney, lymph node involvement, metastatic nephroblastomas and complications of surgery.

[The surgical treatment of advanced stages of solid tumors in children]. / Leczenie chirurgiczne w zaawansowanych stadiach guzów litych u dzieci.

Hope for cure in children with advanced cancer came with introduction of chemo- and radiotherapy, however surgery is still important as a part of the multidirectional treatment. The aim of the review was to assess the impact of surgical treatment in children with advanced cancer. From 1991 to 1997, 30 patients aged from 6 months to 17 years were treated for soft tissue sarcomas (STS: stage III/8 pts, stage IV/2 pts), nephroblastoma (WT: stage IV/5 pts, stage V/3 pts), PNET/Ewing sarcoma (locally advanced/4 pts, metastatic/2 pts) and others (stage III/4 pts, stage IV/2 pts). All patients received pre- and postoperative chemotherapy, all but 6 were irradiated. Twenty one of 30 patients entered remission (CR) after radical surgery for local control: 12 relapsed locally, of whom 7 entered IICR after re-treatment and next surgery and 5 died. 3 of 9 patients who never had any local relapse, died of metastases. Nine of 30 patients never had any radical surgery, 8 died (including 2 toxic deaths) and 1 (stage V Wilms tumour) is in CR after chemotherapy and radiotherapy (12 Gy including both kidney with unresectable tumours). Advanced cancer does not imply the fatal outcome: 14/30 patients are in CR. Possibility of surgical resection of the disease focuses brings some hope for final cure even in initially disseminated disease: 13 such cases of 21 are in ICR or IICR (follow-up: 6 months-7 years) whereas only 1 of 9 those who have never had any radical surgery.

[Alternative methods of central venous access insertion in pediatric oncology]. / Alternatywne sposoby wprowadzania centralnego dostepu zylnego w onkologii dzieciecej.

UNLABELLED: Introduction of the central venous access (CVA) in children treated for malignancies is usually planned procedure. Some patients however require the CVA in emergency. Also in emergency, insertion of CVA should be feasible and safe. The standard technique of CVA insertion is through surgically approached internal jugular vein. This technique requires correct blood coagulation and general anaesthesia in younger patients. Alternative ways of the CVA insertion (via cephalic vein, cubital vein or external jugular vein) seem safe also in case of thrombocytopenia (< 40,000/mm3) and coagulopathy. Aim of the report is to compare efficacy (central tip of the catheter in the superior vena cava on X-ray) and safety (complication rate) of both techniques. PATIENTS: 166 children treated for malignancies aged from 0 to 16 years. The standard technique was used in 109 and in 107 was successful. The alternative technique was used in 57 and in 50 was successful. There were no acute complications, the late ones were observed in 7 of 107 in the standard technique group and in 9 of 50 in the alternative technique group. The alternative ways of the CVA introduction are effective and safe, however the longer X-ray monitoring is necessary.