Variations in haematological and biochemical parameters in healthy ponies.

BACKGROUND: Breed specific reference ranges for selected blood parameters are recommended for proper interpretation of blood tests, but there are only few reports dealing with ponies. The purpose of this study was to investigate if blood parameters differ among ponies' classes and to check if general normal values for equine species are applicable to ponies. RESULTS: All, except total protein concentration, biochemical parameter significantly (p < 0.05) differed among ponies' classes. The most pronounced difference was noted in blood lactate concentrations, higher (p < 0.001) in the smallest ponies (class A). In all groups of ponies muscle enzymes (aspartate aminotransferase and creatine kinase) and urea were high when compared to normal values for equine species, but triglycerides and creatinine were low. Blood lactate concentration was high in comparison with normal values for horses only in class A ponies'. CONCLUSIONS: In healthy ponies, blood lactate concentration significantly differs between height classes. Normal values for equine species should not be directly applied to interpret the lactate, triglycerides, aspartate aminotransferase and creatine kinase values in ponies.

A need for cautious interpretation of elevated serum germ cell tumor markers in children. Review and own experiences.

Protocols for pediatric germ cell tumors (GCT) allow for chemotherapy (CHT) initiation without histological diagnosis, based on typical clinical and radiological picture and increased alphafetoprotein (AFP) or beta-human chorionic gonadotropin serum levels. Such strategy may result in misdiagnoses in rare cases. We present two patients with abdominal tumors and high serum AFP levels, diagnosed as GCT. In both, no tumor shrinkage and increasing AFP was observed after first cycles of multidrug CHT for pediatric GCT. Histological examination of biopsied tumor tissues revealed metastatic cholangiocarcinoma in patient 1 and pancreatoblastoma in patient 2, which implicated immediate change of therapy. Presented cases support the necessity to consider the tumor biopsy when patients diagnosed with GCT based on typical clinical presentation and elevated AFP do not respond to CHT with AFP decrease and tumor size reduction.

Safety of intravenous application of second-generation ultrasound contrast agent in children: prospective analysis.

The goal of the work described here was to assess the safety profile of intravenous second-generation ultrasound contrast agents (UCAs) containing sulfur hexafluoride in pediatric contrast-enhanced ultrasound. Between 2010 and 2013, a total of 167 examinations were performed in 137 children referred by the Oncology Department. Approval by an Independent Ethical Review Board on Scientific Research for the intravenous use of an UCA containing sulfur hexafluoride in children with oncologic diseases was obtained. Consent for UCA administration was acquired from the parents or legal guardians. Severe anaphylactic reaction was observed in 0.6% (n = 1). No other adverse events during or after intravenous administration of contrast were observed in the examined group (no changes in heart rate and rhythm, blood pressure, oxygen saturation or respiratory rate). There were no reports of subjective flushing, nausea, transient headaches or altered taste. Although second-generation ultrasound contrast agents are considered potentially safe, all investigators should be prepared for the development of adverse reactions and have provisions in place for all pediatric intravenous contrast-enhanced ultrasound examinations. More multicenter studies are essential to determination of an accurate UCA safety profile.

Ovarian function in female survivors after multimodal Ewing sarcoma therapy.

BACKGROUND: With advances in cancer care, more young women with Ewing sarcoma (ES) survive after treatment. Thus, we sought to analyze the ovarian function in prepubertal, pubertal and postpubertal females and young women receiving multimodal therapy for ES, and to identify patients at risk of infertility on whom fertility preservation would be indicated. PROCEDURES: Twenty-seven female survivors of ES were included in this retrospective multiinstitutional study. Patients were classified into four groups according to the treatment received: chemotherapy (CHT) without pelvic radiation (pRT), chemotherapy and pRT, CHT and autologous hematopoietic cell stem rescue (aHSCT) without pRT, and CHT + pRT + aHSCT. The ovarian function and fertility outcomes were analyzed. RESULTS: At a median follow-up of 5.7 years from diagnosis, and at median age at follow-up of 16.3 years, 67% of the survivors had premature ovarian insufficiency, including all patients receiving pelvic RT and 87.5% of patients who underwent aHSCT, independent of chemoprotection. Thirty-seven percent of patients had a clinical syndrome of premature menopause. The relative risk (RR) of premature ovarian insufficiency of a survivor was 3.9 (p 0.03) for pRT, and 2.4 (p 0.07) for aHSCT. On multivariate analysis, radiation therapy was a significant predictor of higher risk of premature ovarian insufficiency over chemotherapy alone. CONCLUSIONS: A large proportion of women receiving multimodal therapy for ES develop premature ovarian insufficiency. Patients and guardians should be informed about the reproductive potential and strategies for preservation of ovarian function should be considered individually. Pediatr Blood Cancer 2015;62:341-345. © 2014 Wiley Periodicals, Inc.

Internal hemipelvectomy in the management of pelvic Ewing sarcoma - are outcomes better than with radiation therapy?

BACKGROUND: Pelvic Ewing sarcoma (ES) is commonly associated with a worse prognosis. Large size and location limit local control options to radiation therapy, and local recurrences are common. We evaluated the impact of hemipelvectomy and radiation on outcomes, including function. MATERIALS AND METHODS: Thirty-nine patients (median age 13.5years) with ES of the pelvis and sacral bones were treated during the period 2000-2012. Fifteen were treated with definitive radiotherapy (RT), 9 patients underwent hemipelvectomy alone, and 15 were treated with combined hemipelvectomy and RT. RESULTS: Twenty patients (51.2%) are alive with a median follow-up 3.2years from diagnosis. Median time from diagnosis to relapse was 1.3years. Three-year estimates of EFS and OS were 47% and 61%, respectively. Patients treated with surgery or surgery with RT had better outcome than patients treated with RT only (3-year OS 78% or 81% vs. 36%, respectively, p=0.00083). The outcome of patients with pelvic ES treated with hemipelvectomy was not significantly different from the outcome of all patients with Ewing sarcoma treated on the national Polish protocol. CONCLUSIONS: Internal hemipelvectomy offers good chances of cure for patients with pelvic ES, with a reasonable rate of complications and good function.

Validation of a multi-modal treatment protocol for Ewing sarcoma--a report from the polish pediatric oncology group.

BACKGROUND: Ewing sarcoma (ES) is the second most common paediatric malignant bone tumor. Advances in multi-disciplinary care have resulted in significant improvement in cure rates over the last decades. However, the generalization of those results in countries traditionally excluded from large cooperative trials has yet to be demonstrated. We report the results of modern multi-disciplinary care for patients with ES in Poland. PROCEDURES: One hundred and thirty-two patients with ES were treated using modern multi-modal therapy during the period 2000-2009. Overall survival was estimated by Kaplan-Meier methods and compared using long-rank test and Cox models. Factors predictive of outcome in our setting were analyzed to identify distinct risk groups that could help identify areas for improvement. RESULTS: The median age at the time of diagnosis was 12.3 years. With a median follow-up of 5.0 years, the 5-year event-free survival (EFS) and OS estimates for localized disease were 54.88% and 68.29%, respectively. For patients with metastatic disease, 5-year EFS and OS estimates were 36% and 42%, respectively. There was no correlation between age and stage or site. Patients with localized, non-pelvic disease had better outcome than patients with axial tumors (71% vs. 44%, respectively, P = 0.00073). Treatment failure was associated with stage, pelvic primary, poor histological response, and type of local control. CONCLUSIONS: Successful treatment of ES requires optimal systemic and local therapy. We were able to replicate the results of modern multi-modal protocols. Validation of current treatment protocols in countries with more limited cancer treatment resources is required.

[Chemotherapy in patients with refractory Ewing sarcoma]. / Chemioterapia u dzieci i mlodziezy z opornymna leczenie miesakiem Ewinga

BACKGROUND: Patients with metastatic, progressive or recurrent Ewing sarcoma have a poor prognosis. In addition to increasing the intensity of conventional chemotherapy, the combination of irinotecan and temozolomide has been proposed as an effective salvage regimen for some pediatric malignancies. AIM: To evaluate the effect of two different salvage regimens on the final outcome of patients with refractory Ewing sarcoma. MATERIAL AND METHODS: During the period 2008-2012, twenty-two patients (age between 2.9 -19.3 years) with recurrent or refractory Ewing sarcoma were treated with the combination of vincristine, irinotecan and temozolomide (VIT regimen), and twenty patients were treated with the combination of cisplatin, doxorubicin, cyclophosphamide and teniposide (PACE regimen). All patients had standard tumour imaging and laboratory evaluation. All toxicities were documented. The WHO criteria were used to evaluate response. Statistical analysis was performed using STATA 10.0 for Windows. Results distributions were estimated using the method of Kaplan-Meier. The log-rang test was used to compare the groups. RESULTS: A total of 91 cycles of VIT and 65 cycles of PACE were administered. For VIT therapy the overall response rate was 68.1%. Median time to progression was 3.0 months. Five patients are alive with no evidence of disease with a median follow-up of 10.3 months. For PACE therapy the overall response rate was 75%. Median time to progression was 3.5 months. Four patients are alive with no symptoms of disease with a median follow-up of 17.6 months. The 2 years overall survival probability after recurrence was 29.94%; no differences were detected between therapy groups. Toxicity for PACE was significantly higher. CONCLUSIONS: The effectiveness of VIT regimen in refractory Ewing Sarcoma is comparable to conventional chemotherapy. The VIT regimen has less associated toxicities than the PACE regimen.

Vincristine, irinotecan, and temozolomide in patients with relapsed and refractory Ewing sarcoma.

BACKGROUND: Patients with metastatic, progressive or recurrent Ewing sarcoma (ES) have a dismal outcome. The combination of irinotecan and temozolomide has been proposed as an effective salvage regimen for some pediatric malignancies. Thus, we sought to evaluate this combination with vincristine for patients with relapsed and refractory ES. MATERIALS AND METHODS: Twenty-two patients with relapsed or refractory ES were treated with the combination of vincristine (1.5 mg/m(2) i.v. day 1), irinotecan (50 mg/m(2) /day i.v. days 1-5) and temozolomide (125 mg/m(2) /day p.o. days 1-5) (VIT) during the period 2008-2012. All toxicities were documented. RESULTS: A total of 91 cycles (median 4.1 cycles/patient) were administered. A complete response (CR) was achieved in five patients, partial response (PR) in seven patients, stable disease (SD) in three patients, and progression disease (PD) in seven patients, with an overall response rate of 68.1%. Median time to progression was 3.0 months (range 1.1-37.1 months). Five patients (22.7%) are alive with no evidence of disease with a median follow-up of 10.3 months (range 2.1-46.5); four of them received consolidation with high-dose chemotherapy and autologous hematopoietic stem cell transplant after responding to VIT. Outcome was better for patients with relapsed ES compared with patients who progressed to initial therapy (estimated 2 year overall survival 36.4% vs. 0%, respectively). There were no significant toxicities. CONCLUSIONS: The shorter, 5-day VIT regimen is an active and well-tolerated regimen in refractory ES. This combination deserves further investigation in the upfront management of patients with metastatic disease.

Ultrasound assessment of thrombotic complications in pediatric patients with tunneled central venous catheters.

Central venous access consists in inserting a vascular catheter to the vena cava and placing its tip in the vicinity of the opening to the right atrium. In the patients of the Clinic of Pediatrics, Hematology and Oncology at the Academic Clinical Centre of the Medical University in Gdansk, such implantation procedures are conducted 40-50 times in a year using Broviac/Hickman catheters that are placed in the subclavian vein. In the Ultrasound and Biopsy Laboratory at the clinic mentioned above, approximately 200-250 examinations have been conducted since 2005 to assess the central venous access. Implantation of a catheter considerably increases the comfort of patients who require a long-term venous access. Nevertheless, it is an invasive procedure, burdened with a risk of numerous, early and late complications. The late complications are associated with implanted catheters and include catheter-related thrombosis. The aim of this paper was to present three patients of the Clinic of Pediatrics, Hematology and Oncology at the Academic Clinical Centre of the Medical University in Gdansk, in whom thrombotic complications occurred as a result of long-term central venous catheters. The paper also discusses the possibilities of using sonography in the assessment of such complications. In the presented patients, it was possible to determine the size and localization of a thrombus which enabled effective treatment in two cases. The pathomechanism of catheter-related thrombosis was explained and the risk factors of such complications were discussed. The attention was paid to the necessity of conducting ultrasound examinations in pediatric patients with inserted catheters as soon as the first symptoms of thrombosis appear. Based on own observations and despite the lack of validation of ultrasound imaging in the assessment of central catheters, we believe that this method is highly promising and can be recommended for the assessment of thrombotic complications in pediatric patients with central venous catheters.

[Characteristics of extracranial malignant germ cell tumours in two age groups of children (0-10 and 10-18 years). Multicentre experiences]. / Charakterystyka pozaczaszkowych zltosliwych guzów germinalnych u dzieci w dwóch grupach wiekowych 0-10 lat i 11-18 lat. Badania wieloosrodkowe.

UNLABELLED: In order to assess if any differences exist in children germ cell tumours depending on age, we compared some features of germ cell tumours in two age groups:younger than 10 and between 11 and 18 years. MATERIAL AND METHODS: Data of 146 patients with germ cell tumours treated in 15 Polish paediatric oncology departments between 1995 and 2005 were evaluated. They were divided into two groups: 76 children 0-10 years old (group I) and 70 patients 11-18 years old (group II). Tumour morphology, sex of patients, primary tumour and metastases localization, disease stage, biochemical markers, treatment response, disease relapse and long survival were analyzed. Every patient was treated according to the TGM 95 protocol. RESULTS: In group 1, 67 tumours were assessed histologically. 64%t tumours had homogenous structure with yolk sac tumour in predominance and 36% were mixed. Yolk sac tumour (YST) or teratoma as components of mixed tumours were the most commonly found. In older group 64 tumours were examined, 41% were homogenous, and seminoma/dysgerminoma predominated. In 59% mixed tumours the most common components were YST embryonal carcinoma and teratoma. The most common primary site in group I was the sacrococcygeal region while in group II - the gonads. Disseminated disease was recognized mostly in older children. Among two evaluated serum markers, AFP was increased mostly in younger patients (76% vs 44%), and 3HCG in older group (40% vs 9%). Treatment response was comparable in both groups. Two relapses were observed in each group. Poor outcome was noted in 17/140 analyzed patients: 9 (12%) in group I and 8 (11%) in group II. In 12 of patients with poor outcome the cause of death was progression and in 5 of them - treatment complications. CONCLUSIONS: 1. Germ cell tumours in younger and older children differ in histology, primary localization and serum level of biochemical markers. 2. In older patients germ cell tumours are recognized more frequently in advanced clinical stages. 3. Treatment response was comparable in both groups. 4. There is a need to analyze the intensity of chemotherapy to precise the adequate risk groups according to primary treatment response.

Potential role of PET-CT in chemotherapy efficacy assessment and recurrence diagnosis in a patient with a Wilms' tumour.

BACKGROUND: Wilms' tumour is the most frequent renal malignancy in children. There is no worldwide consensus regarding treatment and PET-CT role in this neoplasm. The aim of this report is to demonstrate the potential role of PET-CT in chemotherapy efficacy assessment and recurrence diagnosis. CASE DESCRIPTION: a 7-year-old boy was diagnosed with blastemic type Wilms' tumour and underwent neoadjuvant chemotherapy, nephrectomy, and adjuvant chemotherapy in compliance with SIOP protocol. Three years later the patient underwent surgical resection of the metastasis and chemoradiotherapy. Nine months later tomography and PET-CT were performed (during the third month of the treatment due to a second recurrence). The results were equivocal but within four months the boy underwent surgical resection of a third recurrence. Fourteen months later a second PET-CT revealed an active disease with extensive involvement of the left lung and pleura. The patient was referred to oral palliative chemotherapy. DISCUSSION: Equivocal PET-CT results during chemotherapy should be interpreted with caution. The first, during third line chemotherapy, was equivocal; however, an early massive recurrence three months later indicates that treatment was ineffective. The second PET-CT examination fourteen months later, as the only modality, depicted massive progression of the disease. This suggests the value of this examination in recurrence diagnosis. CONCLUSIONS: PET-CT seems to be valuable technique in recurrence detection in patients with histologically unfavourable tumours. Equivocal results of PET-CT should raise suspicion of recurrence even in recently treated patients. The timing of CIM and PET-CT should be considered individually--no universal and reliable schedule exists.

Neuroblastoma and opsoclonus-myoclonus-ataxia syndrome--clinical and pathological characteristics.

INTRODUCTION: Opsoclonus-myoclonus-ataxia (OMA) syndrome is the most common paraneoplastic neurological syndrome in childhood. MATERIALS AND METHODS: We reviewed the literature and reported on clinical and pathological characteristics of four children with OMA and peripheral neuroblastic tumours. In two of the children the onset of neurological symptoms was connected with a vaccination and in one with viral infection. The suprarenal gland was the primary localization of the tumour in 3 of the children and in one the tumour was located in the retroperitoneal area. All cases were in stage II or III of the disease, with no metastases or MYCN amplification. The group included two ganglioneuroblastomas, one ganglioneuroma and one differentiating neuroblastoma. The tumours were characterized by the presence of lymphocytic infiltrations with lymphadenoplasia. Immunohistochemical analysis of inflammatory infiltrations revealed mixed type populations of lymphocytes with prevalence of the cytotoxic type (CD8 and CD56-positive cells). The participation of dendritic cells and macrophages was also detected. All patients were treated by surgery alone or with adjuvant chemotherapy with a positive outcome. In 3 cases persistent neurological disorders were observed with exacerbations during infections. CONCLUSION: In some patients the onset of OMA is related to vaccination or infection. Children with OMA and neuroblastoma despite a good oncological prognosis often present permanent neurological and developmental deficits. The inflammatory infiltrations within the tumours are combined, with predominant participation of cytotoxic cells.

[Therapeutic difficulties in soft tissue sarcoma occurring in children with neurofibromatosis type 1 - own observations]. / Trudnosci terapeutyczne w miesakach tkanek miekkich u dzieci z nerwiakowlókniakowatoscia typu i- obserwacje wlasne.

UNLABELLED: Neurofibromatosis type I (NF1) is one of the most common genetic disorders in man, predisposing to benign and malignant tumours. The most common malignancies comprise nervous system tumours, less frequently soft tissue sarcomas (STS) and leukaemia - myelodysplasia syndrome. Herein we report five cases of STS diagnosed in children affected with NF1 (3 girls and 2 boys, age: 8 months -17 years). Neurogenic tumours were diagnosed in three children (malignant peripheral nerve-sheath tumour in two and malignant triton tumour in one), while soft tissue sarcomas of rhabdomyosarcoma origin were found in two patients. In four cases the primary tumours were highly locally advanced, unresectable and located in pelvis minor. All patients received protocols for soft STS: CWS-91, 96 and 2002. The paper presents the clinical symptomatology, the course and outcome in children with NF1 affected with STS. CONCLUSION: basing on our own observations STS in NF1 seems to have poor prognosis in spite of combined therapy. Since early diagnosis is essential, children with NF1 should remain under the care of the oncologist.

[Analysis of factors influencing treatment results in children with soft tissue head and neck sarcomas - in the material of the Department of Paediatrics, Haematology, Oncology and Endocrinology, Medical University of Gdansk]. / Analiza czynników wplywajacych na wyniki leczenia miesakow tkanek miekkich okolicy glowy i szyi u dzieci w materiale kliniki pediatrii, hematologii, onkologii i endokrynologii akademii medycznej w gdansku.

UNLABELLED: The aim of the study was to analyze the prognostic factors for the outcome in childhood head/neck soft tissue sarcomas (STS) treated in the Department of Paediatrics, Haematology, Oncology and Endocrinology, Medical University of Gdansk between 1992 and 2007. MATERIAL AND METHODS: Among 23 children with STS in ten it was located in non-parameningeal region, in eight it was in the parameningeal region, and in five in the orbit. Patients were qualified to particular disease stages according to the current diagnostic and therapeutic protocols (Cooperative Weichtel-sarkom Studie-CWS). The qualifications were based on the results of radiologic examinations (ultrasonography, computed tomography and magnetic resonance of the head and neck region, computed tomography of the chest and abdomen and bone scintigraphy) and also on myelogram smears and cerebrospinal fluid investigation. RESULTS: The longest history concerned the parameningeal (8 months), the shortest the orbital soft tissue sarcomas. Six out of ten patients with non-parameningeal STS were referred to the oncologist with a delay of 6.5 months. This was due to the initially false histopathological diagnosis which excluded the neoplastic process. 82.6% of tumours were diagnosed in advanced, inoperable stages. In 16 children rhabdomyosarcomas (including embryonal subtype - RME in 10, alveolar - RMA in five and undifferentiated in one); in seven non-rhabdomyosarcomas (non-RMS) were found. Good response to chemo- and radiotherapy was observed in 60% of children, mainly with RME. Nine children (mainly with non-parameningeal STS) relapsed. 15 patients are alive (including all with orbital, 6/10 with non-parameningeal and 4/8 with parameningeal STS). Eight children died of disease progression. CONCLUSIONS: 1. Poor outcome in our patients with non-parameningeal head/neck STS results from false initial diagnosis cansing a delay in referring them to the oncologist. It is essential to give more training to the histopathologists about neoplasms in children. 2. Because complete tumour resection in parameningeal STS is rarely feasible, the prognosis in this group is uncertain despite intense chemo- and radiotherapy. 3. The prognosis in orbital STS is usually good; however, they need mutilating surgery in selected cases not responding to therapy.

[Second neoplasms in children with solid tumours in the years 1992-2007. Experiences of Gdansk medical academy]. / Drugie nowoteory u dzieci z guzami litymi w latach 1992-2007. Doswiadczenia osrodka gdanskiego

UNLABELLED: The occurrence of a second tumour is a severe complication of neoplastic disease and its treatment, and it reduces the patient's chances to survive. The aim of the study was to assess the frequency of a second neoplasm and its clinical course in children treated in Gdansk in the years 1992-2007. PATIENTS AND METHODS: There were 420 children and young adults included in the study. They were treated for malignant tumours in this period in the Department of Paediatrics, Haematology, Oncology and Endocrinology Medical Academy of Gdansk. The medical records of these patients were analysed. RESULTS: The second neoplasm was diagnosed in 9 patients, aged 9 to 23 years. They were treated for nephroblastoma - 3 cases, soft tissue sarcoma - 2, Ewing's sarcoma - 1, medulloblastoma - 1, retinoblastoma - 1 and neuroblastoma - 1 case. The second neoplasms were: acute non lymphoblastic leukaemia - 2, soft tissue sarcoma - 2, osteosarcoma - 2, chondrosarcoma - 1, renal cell carcinoma - 1 and glioblastoma multiforme - 1 case. Time between the first and second diagnosis was from 3 and 11/12 to 19 years. Treatment failed in 5 out of 9 children treated for osteosarcoma (2/2), chondrosarcoma (1/1), soft tissue sarcoma (1/2) and acute non lymphoblastic leukaemia (1/2). These patients died of progression of neoplastic disease during 2 to 20 months after the diagnosis of the second tumour. CONCLUSIONS: The diagnosis of the second tumour worsens the prognosis. It is difficult to define the factors that predispose to the second neoplasm. In 5 cases the second neoplasm occurred in the region which was previously irradiated.

Sarcomas in children with neurofibromatosis type 1-poor prognosis despite aggressive combined therapy in four patients treated in a single oncological institution.

OBJECTS: Patients with neurofibromatosis type 1 (NF1) are predisposed to developing soft tissue sarcomas (STS). MATERIALS AND METHODS: We report on four cases of STS diagnosed in locally advanced, unresectable stages in children with NF1 (three girls, one boy; age = 8 months-14 years). All patients received protocols for STS: Cooperative Weichteilsarkomstudie 91, 96 and 2002. One patient with limb rhabdomyosarcoma entered complete remission but developed late metastatic relapse and died of progression despite complete excision and autologous bone marrow transplantation. The other patient with bladder rhabdomyosarcoma died of neutropenia-related sepsis without remission. Patients with malignant peripheral nerve sheet tumour and malignant triton tumour located in the pelvis did not respond to therapy. One of them died of disease progression, while the other is disease-free 6 years post-therapy after mutilating tumour resection. CONCLUSION: STS in NF1 seem to display poor prognosis in spite of combined therapy; thus, children with NF1 should remain under detailed control of the oncologist.

[Neurofibromatosis type 1 in children. Experiences of the Gdansk Paediatric Oncohaematology Centre. Preliminary results]. / Nerwiakowlókniakowatosc typu 1 u dzieci. Obserwacje osrodka gdanskiego. Doniesienia wstepne.

INTRODUCTION: Neurofibromatosis type 1 (NF1) is a frequent genetic disorder of autosomal-dominant pattern. The incidence is about 1 per 3000 live births. Patients with NF1 are predisposed to malignancies including soft tissue sarcomas and leukaemias. The aim of the study was assessment of the most frequent symptoms on the basis of long term observation of children with NF1 and presentation of implemented diagnostic and therapeutic procedures. MATERIAL AND METHODS: In our department there are 149 children (71 boys and 78 girls) aged from 7 months to 18 yrs with diagnosed or suspected NF1. Each child is carefully followed up every 6 months on outpatient basis. Paediatric, neurological and opthalmological examinations are performed during the first visit and in cases of any new symptoms. Number of Lisch nodules, vision field, audiogram, dermatological evaluation of skin abnormalities as well as orthopaedic examination are also investigated. In any case of NF1 without neurological symptoms, MRI of the brain and spine is carried out every 2 years. Moreover, each child is consulted in the Genetic Clinic. RESULTS: Cafe-au-lait spots were observed in all 149 children, freckling of the armpits in 40, peripheral neurofibromas in 30, Lisch nodules in 2 patients. Secondary symptoms and complications such as mental retardation (9 cases) and epilepsy (10 cases), cognitive disorders and learning disabilities (21), abnormalities in MRI examination (53), benign or malignant CNS tumours (9), scoliosis (99) were diagnosed. In 5 patients malignant neoplasms occurred (3.4%) including: RMS--2 cases, Triton tumour--1 case, MPNST--1 case. Two children died of disease progression, one of treatment complications (sepsis) and two children are alive. CONCLUSIONS: 1. Patients with NF1 need regular specialist medical care. 2. Continuous education of the families with this disease is necessary. 3. Diagnostic and therapeutic procedures recommended for patients with NF1 need to be implemented at different levels of health care.

[Soft tissue sarcomas of the parameningeal region in children--own observations]. / Miesaki tkanek miekkich okolicy okolooponowej u dzieci--obserwacje wlasne.

UNLABELLED: Soft tissue sarcomas (STS) non-Hodgkin's lymphomas and less frequently nasopharyngeal carcinomas are the most common malignancies located in the parameningeal region in children. AIM: To assess diagnostic and therapeutic problems in children with parameningeal STS treated in the Departments of Paediatric Oncology in Gdansk and Lublin between 1992 and 2006. MATERIAL AND METHODS: The study includes 17 patients with parameningeal STS; mean age of patients was 5.6 years. In one boy an undifferentiated STS was diagnosed 7 years after treatment of retinoblastoma. RESULTS: Initial symptoms lasted from 2 weeks to 24 months, mean 5.5 months. Symptoms associated with parameningeal location of the tumour (snoring, breathing through the mouth, epistaxis, chronic purulent rhinitis, dysphagia and earache) predominated and were treated initially as upper respiratory tract infections. All analysed patients presented with highly advanced stages of STS. Oncological treatment was conducted according to the schemes approved by the Polish Paediatric Solid Tumours Study Group. Good response to therapy was stated only in 24% children with STS. These patients (all with embryonal subtype) entered complete remission after standard I line therapy. 13 children required more aggressive II line treatment because of poor response to therapy (NR - 5 children) or relapse (8 children). Seven of the analysed patients (41%) are in lasting complete remission, from 32 months to 13 years 2 months (mean 5 years) after therapy discontinuation. In four children (23%) persistent complications of oncological treatment occurred, including postradiation defect of the orbital bulb, postsurgical facial nerve palsy and cranio-nasal fistula complicated with pneumocephaly. A patient with STS of maxillary sinus developed a second neoplasm 2 years after first therapy. This was a glioblastoma multiforme located in the left parietal lobe (outside the radiation field). At present, the boy is in complete remission nearly 4.5 years after treatment for the second tumour. Ten patients died, all in the phase of disease progression. In two of them myelosupressive, gastrotoxic and infectious complications of antitumour therapy were the direct cause of death. CONCLUSIONS: 1. Non-specific initial symptoms of soft tissue sarcomas located in parameningeal region in children suggesting inflammatory process result in diagnostic dilemmas and proper diagnosis delay. 2. Because complete resection of the parameningeal STS is unfeasible, the prognosis is poor in spite of aggressive chemo- and radiotherapy. 3. Complex therapy carries a risk of severe complications, thus it should be conducted in highly specialized oncological centres.

[Initial results of the treatment of six patients with high risk neuroblastoma in the years 2002--2006]. / Wstepne wyniki leczenia szesciu chorych z rozpoznaniem zwojaka zarodkowego wspólczulnego z grupy wysokiego ryzyka w latach 2002--2006.

UNLABELLED: Between the years 2002 and 2006 in the Department of Paediatrics, Haematology, Oncology and Endocrinology, Medical University of Gdansk, six patients with neuroblastoma were qualified for the high risk group. Aim of the study was the evaluation of treatment results after using new methods of therapy. MATERIAL AND METHODS: Intensive multimodal therapy according to the new European protocol HR-NBL-1/ESIOP, was implemented in 6 children. In two cases the high risk group qualification was delayed. Mean time of observation was 2 years 4 months. RESULTS: After the end of therapy, three of six children remain in complete remission. The histopathology of the tumour was assessed according to Shimada classification. Number of NMYC copies was estimated by the FISH method. One patient died of infection after high dose chemotherapy. Relapses occurred in two patients. One of them died of progression and the other is alive, in the third relapse, with progression of disease. CONCLUSIONS: The preliminary analysis shows good results of the new protocol in half of the patients. Further additional cooperative studies with bigger groups of patients are required.