Knowledge of School Nurses on the Basic Principles of Type 1 Diabetes Mellitus Self-Control and Treatment in Children.

School nurses should participate in the care of children with type 1 diabetes mellitus. The aim of this study was to assess the level of knowledge of school nurses about the basic principles of self-control and the treatment of type 1 diabetes mellitus and to attempt to determine the factors that influence this level of knowledge. A cross-sectional survey was conducted among school nurses from October 2018 to November 2019 in the Pomeranian Voivodeship. The study was conducted using a self-constructed questionnaire. The survey included questions about the sociodemographic characteristics of the respondents, and a test of the knowledge and skills regarding the principles of self-control and the treatment of type 1 diabetes mellitus (16 test questions). This study included 168 Polish school nurses (mean age ± SD = 55.1 ± 8.9 years). Most of the nurses had a secondary education (81%), worked in a municipal educational institution (78.6%), and provided care to more than one student with type 1 diabetes mellitus at school (70.2%). The average level of knowledge of school nurses was 12.5 ± 2.0 points (maximum 16). The nurses working in a village school and those who worked only in one school had lower levels of knowledge. Only 85.7% of nurses reported that they could independently perform a blood glucose measurement with a glucometer, and as many as 56.5% were unable to determine the level of ketone bodies in the urine with Keto-Diastix test strips. Only 62.5% of nurses had a glucometer and glucometer strips in their nursing office. A total of 19.6% of nurses did not have glucagon (1 mg GlucaGen HypoKit®, Novo Nordisk A/S, Bagsværd, Denmark) or an ampoule with 20% glucose for an intravenous administration. The knowledge of school nurses about the principles of self-control and the treatment of type 1 diabetes mellitus is insufficient. Due to the strong increase in the incidence of type 1 diabetes mellitus among children and adolescents, it is important to organize permanent, continuous, and mandatory training on the principles of self-control and the treatment of type 1 diabetes mellitus for school nurses. The equipment in Polish school nurses' offices should be supplemented with a working glucometer and blood glucose test strips, and the set of obligatory medications in the school nurse's office should be supplemented with glucagon for students with type 1 diabetes mellitus.

Life Satisfaction of Nurses during the COVID-19 Pandemic in Poland.

BACKGROUND: Health care practitioners are at highest risk of COVID-19 disease. They experience an enormous overload of work and time pressures. The objective of the study was to assess nurses' life satisfaction. METHOD: The study included professionally active nurses. The research method was an author's questionnaire and a standardized questionnaire, the Satisfaction with Life Scale (SWLS). RESULTS: The study group included 361 working nurses. The mean raw score and the sten score of the nurses' responses to the statements on the SWLS questionnaire were 21.0 (SD ± 5.6, range = 5-35) and 5.73 (SD ± 1.94, range = 1-10), respectively. It was shown that lower life satisfaction was experienced by nurses aged 51 to 60 (raw score: p = 0.003, sten score: p = 0.005), as well as nurses with secondary and undergraduate nursing education (raw score: p = 0.061, sten score: p = 0.043). Nurses who had a higher self-evaluation of the level of knowledge about SARS-CoV-2 infection experienced greater life satisfaction (raw score: p = 0.008, sten score: p = 0.022). CONCLUSIONS: The majority of Polish nurses surveyed during the COVID-19 pandemic had a low or medium level of life satisfaction. The low response rate to the survey was most likely due to work overloads during the pandemic. Working in a public service profession, a nurse is exposed to stressful conditions related to protecting human health. Constant difficult and stressful situations and total fatigue experienced by nursing professionals can be the cause of a lack of motivation, occupational burnout, listlessness and mental and physical disease. Further research is necessary to assess the factors positively influencing the level of life satisfaction.

Comparison of the Effectiveness of the Ultrasonic Method and Cone-Beam Computed Tomography Combined with Intraoral Scanning and Prosthetic-Driven Implant Planning Method in Determining the Gingival Phenotype in the Healthy Periodontium.

The aim of this study was to compare the effectiveness of two diagnostic methods: ultrasonic gingival thickness measurement (UGTM) and cone-beam computed tomography, intraoral scanning by computer-aided design technology with prosthetic-driven implant planning software (CBCT/CAD/PDIP) in determining the gingival phenotype (GP). Thirty periodontally healthy patients were examined. The ultrasonic device Pirop G® with a frequency of 20 MHz and CBCT/CAD/PDIP were used to measure gingival thickness at upper canines and incisors in three points localized midbuccally, namely free gingival thickness (FGT), supracrestal (SGT) and crestal (CGT). Probing depth (PD), clinical attachment level (CAL) and width of keratinized tissue (WKT) were measured using periodontal probe. Intra-examiner and inter-examiner agreement and agreement between methods were evaluated using Bland-Altman analyses. Comparing both methods in the determination of SGT (bias = 0.17 mm, SD = 0.25 mm, p < 0.000) and CGT (bias = -0.45 mm, SD = 0.32 mm, p < 0.000) 95.0% and 95.6% agreement were found, respectively, and in the FGT range only 93.3% (bias = -0.45 mm, SD = 0.32 mm, p < 0.000). The presence of positive correlations between WKT and SGT was shown. A positive correlation between SGT and WKT confirms the purpose of measuring these parameters for the evaluation of the GP. Both the ultrasonic method and cone-beam computed tomography combined with intraoral scanning and prosthetic-driven implant planning method were useful in determining gingival phenotype, however, the ultrasonic method was more accurate for measuring GT.

Analysis of Strategies for Managing Stress by Polish Nurses during the COVID-19 Pandemic.

BACKGROUND: The COVID-19 pandemic is having a negative impact on the mental health of nurses around the world. The aim of the study was to assess the sense of fear and the degree of exposure to SARS-CoV-2 infection and to estimate the influence of various factors on the fear of SARS-CoV-2. We analysed methods and strategies for coping with stress used by Polish nurses during the COVID-19 pandemic. METHODS: The study was conducted using a diagnostic survey, which included a self-constructed questionnaire and a standardized psychological questionnaire: Brief-Cope. RESULTS: A total of 361 nurses confirmed their participation in the study. Most of the nurses were ages 31 to 50 (48.2%), lived in a city (83.7%), and had a master's degree in nursing (45.7%). Nurses ≥ 51 years of age, with ≥ 21 years of work experience and with secondary education in nursing and master of nursing were more likely than other nurses to agree with the statement, I am afraid of contracting the SARS-CoV-2 (appropriately p = 0.009, p = 0.007, p = 0.014). During the outbreak of COVID-19, nurses most often took action to improve the situation, reflecting on and planning what to do. The most frequent means of coping with stress by Polish nurses during the COVID-19 outbreak were problem-focused strategies. The least frequent strategy was the use of substances (taking substances to alleviate unpleasant emotions), considered to be the least effective, but useful in some situations. CONCLUSIONS: Most nurses were afraid of being infected with COVID-19. The most frequently used strategies for coping with stress by Polish nurses during the COVID-19 pandemic were problem-focused strategies. Nurses should receive psychological support and assistance from the employer in improving their working conditions.

Nutritional status at the moment of diagnosis in childhood cancer patients.

INTRODUCTION: Children with a neoplastic disease are highly susceptible to malnutrition. The main objective of the study was to assess the frequency of undernourishment and obesity at the time of the diagnosis of the neoplastic disease at children. MATERIALS AND METHODS: The study included 734 patients (58% males) at the age 1-20,25, with the diagnosis of neoplasm in the years 1986-2014. The patients were divided into groups depending on the type of the diagnosis: 1) ALL, 2) ANLL, 3) HL, 4) NHL, 5) NBL, 6) Wilms tumor, 7) mesenchymal malignant tumor. The BMI SDS and the height SDS were evaluated. The difference in the incidence of disorders in each group was examined. RESULTS: In the study group at the time of the diagnosis 21.5% of patients were undernourished while 13.8% presented were overweight. Patients in the ALL group were overweight more often than the rest of the study group (RR 1.82, CI 95%1.26-2.63, p=0.002) - 18.6% of them were overweight. However, children with mesenchymal malignant tumor were less susceptible to overweight than the rest of the patients (RR 0.36, CI 95%0.15-0.87, p=0.021) - only 5.4% of them were overweight. Girls with ALL were malnourished more often than other patients (RR 1.72, CI 95%1.08-2.75, p=0.03). There were no significant differences in the malnutrition/obesity frequency in other neoplasms groups. SUMMARY: ALL patients are less susceptible to underweight than the patients with the solid tumor. Moreover, the high incidence of overweight in children with ALL is noteworthy.

Clinical factors in relapses of Wilms' tumor--results for the Polish Pediatric Solid Tumors Study Group.

BACKGROUND: The risk factors responsible for recurrences of Wilms' tumor (nephroblastoma) are still under discussion. The aim of the study was to analyze the relationship between relapses of Wilms' tumor and the patients' clinical history. MATERIAL AND METHODS: Clinical data from children registered in the Polish Pediatric Solid Tumors Study Group were analyzed. The clinical stages (CS), pathology variants (high risk: HR, intermediate risk: INT, and low risk: LOW) and chemotherapy regimens were correlated with the outcomes. RESULTS: Recurrences developed in 34 out of 288 (11.8%) patients with Wilms' tumor treated in accordance with International Society for Pediatric Oncology 2001 (SIOP 2001) protocols. Of these 34 patients, 11 initially had CS I, seven were at CS II, four were at CS III, 11 were at CS IV and one had CS V. There were eight patients with second recurrences; of these, seven were in the INT risk group and one in the high histological risk group. There was no correlation between age (p=0.256) or gender (p=0.538) and the risk of tumor recurrence. In the study group, seven out of 10 patients with local recurrences are alive; as are 13 out of 22 patients with distant recurrences (p=0.703). Those who died due to disease progression comprised six out of 26 patients with a first recurrence (four HR, two INT), and seven out of eight with a second recurrence (one HR, six INT). CONCLUSIONS: The prognosis after relapse in initially metastatic patients did not differ from that in patients who had primarily localized disease. The pathology variants probably had more significance.

G-CSF therapy and catheter-related Gram-positive sepsis increase serum IL-2 receptor α level and may falsely suggest a relapse in children with soft tissue sarcomas unless serum beta2-microglobulin, lactate dehydrogenase and C-reactive protein levels are determined concomitantly.

Many components of oncologic treatment increase serum sIL-2Rα level, which may falsely suggest a relapse. We tried to establish whether granulocyte colony stimulating factor (G-CSF) and central vein catheter (CVC)-related sepsis increase serum sIL-2Rα level to values on relapse of childhood soft tissue sarcomas (STS) and how to distinguish real relapse from a "false" one. Serum sIL-2Rα, B2-M, LDH, CRP and ESR levels and rates of markers' elevated values were determined prospectively in 18 STS children: pre-treatmently (ST1), in complete remission (CR; ST2), in CR during G-CSF therapy (ST3), in CR during CVC-related sepsis (ST4), on relapse (ST5) and after treatment (ST6) and once in 50 healthy pediatric controls. It appeared that pre-treatment serum sIL-2Rα, LDH, CRP and ESR but not B2-M declined significantly with remission (ST2) achievement. At ST5 sIL-2Rα, B2-M, LDH and CRP increased from ST2 to ST1 values. SIL-2Rα levels at ST3 and ST4 rose significantly in all patients from ST2 to ST1 and ST5 values. At ST3 also serum LDH and B2-M increased to values at ST1 and ST5 and exceeded significantly those at ST2 and ST4. At ST4 CRP but not B2-M and LDH, rose significantly in most patients to values at ST1 and ST5. Thus, serum sIL-2Rα monitoring in pediatric STS reflects well response to chemotherapy unless samples are collected during G-CSF therapy or CVC-related sepsis. Determination of serum B2-M, LDH and CRP together with sIL-2Rα may help to distinguish between "real" relapse and "false" sIL-2Rα increase due to G-CSF administration or CVC-related sepsis.

Pancreatoblastoma: a report from the European cooperative study group for paediatric rare tumours (EXPeRT).

BACKGROUND: Pancreatoblastoma is a very rare malignant tumour typically occurring in the early years of life. Due to its rarity, standardised diagnostic and therapeutic guidelines are not available for pancreatoblastoma. METHODS: The newborn cooperative group denominated EXPeRT - European cooperative study group for paediatric rare tumours - combined in a joint analysis of all cases registered between 2000 and 2009 by the national groups of Italy, France, United Kingdom, Poland and Germany. RESULTS: Twenty patients <18years old (median age 4years) were analysed: nine had distant metastases at diagnosis. Seventeen patients had tumour resection, at initial or delayed surgery. Eighteen received chemotherapy (response rate 73%), seven received radiotherapy. For the whole series, 5-year event-free survival and overall survival were 58.8% and 79.4%, respectively. Outcome did not correlate with tumour site and size, but was strongly influenced by the feasibility of tumour complete resection. CONCLUSIONS: This international study confirms the rarity of the disease, the critical role of surgical resection both as therapy and as a prognostic variable, and the potential efficacy of chemotherapy. The adoption of an intensive multidisciplinary approach is required, as well as the referral to highly experienced centres. Further international cooperation is needed to collect larger series and stimulate biological studies to improve our understanding of the biology and the natural history of PBL.

Immunohistochemical expression of procaspase-3 and its clinical significance in childhood non-Hodgkin lymphomas.

Previous studies have shown differences in expression levels of apoptosis regulatory proteins in non-Hodgkin lymphomas (NHLs) and indicated the correlation of procaspase-3 (proC-3) and caspase-3 activation to the response of chemotherapy. We investigated whether proC-3 expression in tumor biopsies of childhood NHLs is related to clinical outcome. Formalin-fixed paraffin-embedded tissues from 58 children with NHL were evaluated for proC-3 expression by immunochemistry analysis. The study included 20 cases of Burkitt lymphoma, 7 cases of diffuse large B-cell lymphoma, 18 cases of anaplastic large cell lymphoma (ALCL), and 13 cases of precursor lymphoblastic lymphoma. The highest expression level of proC-3 was observed in ALCL. In the multivariate analysis the higher clinical stage of disease and higher expression level of proC-3 were independent and appear to be significant prognostic factors of treatment failure. Our results suggest that the high expression level of proC-3 may be a powerful independent predictor of response to chemotherapy and progression-free survival in childhood NHLs.

Factors predicting survival in childhood malignant and intermediate vascular tumors : retrospective analysis of the Polish and German cooperative paediatric soft tissue sarcoma study groups and review of the literature.

BACKGROUND: The rarity of malignant and intermediate vascular tumors in children means that little is known about their clinical course, optimal treatment, and variables predicting survival. METHODS: A total of 32 children with malignant vascular tumors (14 angiosarcomas [AS], 5 epithelioid hemangioendotheliomas, and 13 intermediate vascular tumors, including other hemangioendotheliomas plus adult-type hemangiopericytomas), registered in the German and Polish Paediatric Soft Tissue Sarcomas Study Groups, were treated following the Cooperative Weichteilsarkom Studiengruppe (CWS)-81, -86, -91, and -96 protocols. RESULTS: Male sex, AS histology, tumor size >5 cm, and T2 invasiveness were independent predictors of inferior 5-year overall survival, while AS histology and T2 invasiveness were predictors of inferior 5-year event-free survival. AS histology was the most important negative prognostic factor for overall survival and event-free survival. Completeness of primary tumor excision was a good prognostic factor for survival in univariate, but not multivariate, analysis. Local therapy (radiotherapy and delayed surgery) were provided to the minority of patients (28% and 38%, respectively) late in the course of disease (after a mean of 9 and 6 months, respectively) and did not prevent local relapses. Response to systemic treatment was poor (44%) and did not prevent local and distant relapses. CONCLUSIONS: The clinical course and outcome in childhood epithelioid HE seems to be similar to intravascular tumors and less aggressive than AS. RTX and delayed surgery should be performed more frequently and earlier in the disease course. An urgent need for modification of systemic therapy is needed because of the development of many metastatic and/or combined relapses and poor response to classic chemotherapy. The problem of effective therapy for childhood AS is the most appaling: 13 of 14 patients died of progression despite multimodal treatment.

The serum levels of soluble interleukin-2 receptor alpha and lactate dehydrogenase but not of B2-microglobulin correlate with selected clinico-pathological prognostic factors and response to therapy in childhood soft tissue sarcomas.

PURPOSE: To establish the clinical utility of serum soluble IL-2 receptor (sIL-2R alpha), lactate dehydrogenase (LDH) [corrected] and B2-microglobulin [corrected] (B2-M) as markers for diagnosis, prognosis and treatment monitoring in childhood soft tissue sarcomas (STS). METHODS: The markers[corrected] were measured prospectively before treatment, in complete remission (CR) during and after therapy and at relapse [corrected] in 35 children with STS and in 50 healthy children [corrected] (once). RESULTS: Serum sIL-2R alpha and LDH [corrected] correlated with age thus they were [corrected] presented as multiplications [corrected] of the upper normal ranges [corrected] for age. Pre-treatment levels [corrected] of sIL-2R alpha and LDH [corrected] but not of B2-M exceeded significantly those of controls. [corrected] Elevated [corrected] sIL-2R alpha levels correlated with more [corrected] advanced stages, poor-risk histology and poor response to chemotherapy, higher LDH with incomplete primary tumour [corrected] resection and increased B2-M with poor-risk histology. [corrected] Patients' age >10 years, male gender and unfavourable tumour localisation were not accompanied by the markers' elevation. [corrected] None of the markers predicted EFS and OS. [corrected] Good response to chemotherapy was paralleled by significant decline of sIL-2R alpha and LDH pre-treatment levels while relapse--by sIL-2R alpha and LDH increase to values similar to those at diagnosis. [corrected] Monitoring of B2-M did not reflect the disease course. [corrected] CONCLUSIONS: sIL-2R alpha and LDH were [corrected] proven to be promising markers [corrected] for diagnosis and treatment monitoring in children with STS. The markers [corrected] correlated also with some [corrected] important prognostic clinico-pathological factors for childhood [corrected] STS; however, they [corrected] failed to predict EFS and OS. Measurements of serum [corrected] B2-M were shown [corrected] to have no clinical value in the diagnostics, prognostics and treatment monitoring in paediatric STS.

Pre-treatment serum levels of interleukin-10, interleukin-12 and their ratio predict response to therapy and probability of event-free and overall survival in childhood soft tissue sarcomas, Hodgkin's lymphomas and acute lymphoblastic leukemias.

OBJECTIVES: Deregulated serum IL-10, IL-12 and their reciprocal balance have been stated in malignancies of adults. In children with cancer the issue has not been investigated so far. DESIGN AND METHODS: To determine the diagnostic and prognostic roles of pre-treatment serum levels of IL-10 (Th2 cytokine), IL-12 (Th1) and their ratios (measured by the IL-10 and IL-12p70 ELISA kits; Endogen) in 91 children with soft tissue sarcomas (STS), Hodgkin's lymphomas (HL) and acute lymphoblastic leukemias (ALL). RESULTS: Median IL-10 and IL-12 levels were significantly higher in cancer patients than in healthy controls. Increased IL-10 indicated presence of general symptoms in HL and high risk group in ALL. Elevated IL-10 and IL-10/IL-12 ratios and decreased IL-12 correlated with poor-risk histology in STS, poor response to therapy, relapse and death from cancer. Multivariate analysis identified IL-10/IL-12 ratio>0.14 and IL-12<40 pg/mL as significant predictors for shorter EFS and OS, respectively. CONCLUSION: Pre-treatment serum levels of IL-10, IL-12 and IL-10/IL-12 balance in children with STS, HL and ALL may be of value as additional prognostic tools to predict the response to therapy and probability of EFS and OS.

[Surgical treatment for malignancies in children--is prolonged bone marrow suppression an absolute contraindication for surgical procedures?]. / Leczenie chirurgiczne dzieci z choroba nowotworowa--czy przedluzajaca sie aplazja jest bezwzglednym przeciwwskazaniem do interwencji chirurgicznej?

AIM: To analyze the perioperative period in children who required surgery anticipated in the protocol and suffered from prolonged bone marrow suppression. MATERIAL AND METHODS: The surgical treatment of 15 children with prolonged bone marrow suppression, resistant to GM-CSF for 5-15 weeks (M=10), was retrospectively analyzed. Criteria to submit a patient to the group were: neutropenia <1000/ml or/and thrombocytopenia <70,000/ml. Four had thrombocytopenia, 8 neutropenia and 3 cytopenia of both cell lines. Neutropenic patients were divided into two groups: severe neutropenia--<500/ml (2), and moderate--500-1000/ml (7). Children with thrombocytopenia were divided into two groups: with thrombocytes <30,000/ml (2) and between 30,000-70,000/ml (5). RESULTS: Complications (pneumonia and sepsis) were noted in 2 out of 4 children with severe neutropenia, remaining patients had uncomplicated perioperative period. Patients with moderate neutropenia did not show any alarming signs after surgery. As a major haemorrhage we assumed loosing > 30 percent of circulating blood. This was observed only in 1 patient (with thrombocytes <30,000/ml). 13 patients were discharged from our Department between 3-10 days after surgery in good general condition. Two had to be treated for a longer time because of advanced cancers (18 and 21 days). CONCLUSIONS: Neutropenia does not seem to be an absolute contraindication for surgical procedures needed in complex cure of malignancies. In our opinion thrombocyte level > 30,000/ml appears to be safe enough to conduct surgery.

[Prognostic value of Jass classification of rectal cancer compared to original Dukes system]. / Skutecznosc prognostyczna klasyfikacji raka odbytnicy wedlug Jassa w porównaniu z klasycznym systemem Dukesa.

UNLABELLED: The aim of this study was to assess the prognostic value of Jass and Dukes classification of rectal cancer. MATERIAL AND METHOD: The impact of these staging systems on five-year disease-free survival has been estimated in univariate and multivariate analysis for the group of ninety-two curatively treated patients. RESULTS: In univariate analysis a relationship between survival and stage of disease has been found with a high degree of statistical significance for both Jass (I: 89.3 +/- 5.9; II: 54.2 +/- 10.2, p< 0.005; III: 14.3 +/- 9.4, p< 0.001; IV: 12.0 +/- 6.5, p < 0.001) and Dukes (A: 88.1 +/- 6.4; B: 41.0 +/- .9, p < 0.001; C: 15.4 +/- 7.1, p < 0.001) classifications. Cox regression model has identified Jass system as the best predictor of prognosis (II: p < 0.05; III: p < 0.001; IV: p < 0.001) and has shown the lack of significance for Dukes classification. In multivariate analysis of specific pathological features constituting Jass scale a significant and independent influence on survival (p < 0.05) has been noticed for the depth of penetration through the bowel wall, type of tumour growth and lymphocytic infiltration but not for the number of positive lymph nodes. CONCLUSION: Jass classification of rectal cancer is a prognostically effective modification of Dukes system as the result of addition of histological parameters significantly affects the prognosis.

[Prognostic significance of clinical and pathomorphological factors in colorectal cancer: a uni- and multivariate analysis]. / Ocena wartosci prognostycznej czynników klinicznych i patomorfologicznych w raku okreznicy i odbytnicy metoda analizy jedno- i wielowariantowej.

The aim of this study was to estimate the prognostic value of clinical and pathomorphological factors in cancer of the colon and rectum. We studied seventy-seven patients subjected to a radical surgery with a five-year follow-up. Survival was calculated using the Kaplan-Meier method and compared by log-rank test. Multivariate analysis was performed using Cox's regression model. Overall 5-year survival was 68%. Primary tumour spread beyond the bowel wall and regional lymph node involvement appeared to be independent prognostic factors, significantly influencing the survival of patients. Other variables including patient's age and sex, tumour location, symptom duration before treatment onset, grade of differentiation and mucinous histology had no significant impact on prognosis. Tumour spread beyond the bowel wall and metastases to regional lymph nodes are the most useful prognostic factors in patients with colorectal carcinoma. In routine practice the clinical stage of cancer should remain a reference against which other clinical and pathomorphological variables are assessed.

[Malignant vascular tumours in children -- report from the Polish Pediatric Rare Tumors Study]. / Charakterystyka kliniczna naczyniopochodnych nowotworów zlosliwych u dzieci -- raport Programu Guzów Rzadkich PPGGL.

UNLABELLED: Malignant vascular tumours represent a rare group of neoplasms, usually treated according to protocols for soft tissue sarcomas. THE AIM OF THE STUDY: To assess the clinical characteristics, disease course and outcome in Polish children with malignant vascular neoplasms. MATERIAL AND METHODS: The Polish Paediatric Rare Tumours Study retrospectively analysed multicentre data on 32 children with vascular tumours, registered between 1992 and 2002. On the basis of the histological type of the neoplasm these patients have been divided into three groups: group I -- 10 patients with angiosarcoma (ASA), group II -- 7 children with haemangioendothelioma (HE) and group III- 15 patients with haemangiopericytoma (HP), of both infantile (7 children) and adult-types (8 patients). RESULTS: Group I: 5 patients presented with local, 3 with systemic and 2 with regional disease. Primary complete resection (PRC) was performed in 7 patients, not resulting in local control in any of them. Radiotherapy (RTX) was administered in 5 patients, chemotherapy (CHT) in all. 7 patients relapsed, 3 never entered clinical remission. 9 of the 10 patients of group I, died of disease progression. Group II: PRC was performed in 5 patients and led to local control in 2. Adjuvant RTX was used in 2 and CHT in 4 patients. 2 children relapsed and died of the disease. Infantile HP: PRC was performed in 3 children, remaining 4 patients received adjuvant CHT All children entered complete remission and are disease-free. Adult-type HP: PRC was performed in 5 patients, resulting in local control in 4. Five children were given adjuvant RTX and six CHT Three patients relapsed and died of disease progression. CONCLUSIONS: The effectiveness of primary complete resection in all groups was doubtful. The high rate of metastatic relapses suggests that the currently given systemic therapy is not satisfactory. The only tumour with excellent prognosis was infantile type HP (all patients are alive and free of disease). Adequate treatment for children with angiosarcoma remains still unknown -- 9 of 10 patients died of disease progression. Prognosis in patients with haemangioendothelioma is intermediate, however the role of immunotherapy should be further investigated.

[Rare tumours--are they really rare in the Polish children population?]. / Nowotwory rzadkie--czy rzeczywiscie rzadkie w populacji polskich dzieci?

Clear statement that pediatric neoplasms are really rare is not easy. Thus the incidence of rare tumours in children has not been defined so far. The paper efforts to assess the topic of rare tumours of childhood in the Polish population. Following two categories are proposed: tumours typical for adults, but possible in children (neoplasms of epithelial origin--mainly carcinomas, melanomas, carcinoids) and paediatric tumours consisting less than 10% of cases in corresponding clinical groups according to the ICCC classification. Data on 317 patients aged 0-18 years treated in centres associated in the Polish Paediatric Group for Solid Tumours (PPGST) were analysed. Classical adult malignancies were registered in 130 patients: carcinomas in 90 (mean age 12.6 +/- 4.5 years), melanomas in 25 (mean age 9.4 +/- 4.9) and carcinoids in 9 (mean age 14.5 +/- 1.2 years). Non epithelial neoplasms were registered in 187 patients (mean age 10.4 +/- 5.5). That group included rare tumours of soft tissue, CNS, bones and other organs. Treatments of certain groups were specified by separate therapeutic protocols within PPGST. Rare malignancies of adult-type among children under 18 years of age in Poland comprised 1.5% of all pediatric neoplasms. The incidence of adult-type neoplasms increased with age until 14 years. In patients over 15 years of age the number of registered cases decreased. It may suggest a first peak of incidence in early adolescence or an underestimation of number of patients with carcinoma aged over 15 years. In the analyzed group, the mean age of patients with carcinomas and other epithelial and unspecified tumours significantly exceeded the age of children with rare neoplasms of non-epithelial origin (12.1 +/- 4.7 vs 10.4 +/- 5.5 years; p<0.05). A very young age at diagnosis of malignant melanomas (mean 9.4 years) and numerous cases of carcinomas affecting the digestive tract (n=24; 27% of all carcinomas), especially those located in colorectal region (n=10), seem surprising. The preliminary analysis of the collected data on rare neoplasms in Poland encourage to undertake a prospective study, meant to link the epidemiology and characteristics of rare epithelial tumours in childhood with diagnostic and therapeutic suggestions for these types that are not coordinated within Polish Paediatric Group of Solid Tumours.

[Ewing's sacroma / PNET--the possibility to assure local control]. / Miesak Ewinga / PNET -- mozliwosc uzyskania kontroli miejscowej.

UNLABELLED: Ewing's sarcoma and PNET are more and more frequently submitted to surgical treatment, as this method of local therapy seems to offer a better chance for cure. Which method surgery or radiotherapy (RTx) is more mutilating in childhood, should be carefully considered. The aim of the report is to review the possibility of local resection and reconstruction in various sites of the tumour. PATIENTS: 12 patients with Ewing's sarcoma / PNET were submitted to the site-specific surgical procedures and assessed for local control and function. All were pre-treated with CHT and continued that treatment after surgery. Four patients had fibular Ewing's sarcoma and all underwent subtotal fibulectomy. Long term complete remission (CR) was achieved in all. Two patients had Askin tumours; both developed pleural relapses distant to the primary tumour site. One patient had total scapulectomy (CR for 7 years). One patient had hemipelvectomy supplemented with RTx because of micro residue (CR for 10 years). Two patients were amputated: one for PNET of tibia invading nerves and vessels, another one for Ewing's sarcoma of femur. The first one developed pulmonary metastases and died one year later, the second one has been alive for 13 years but has badly accepted the prosthesis. Two patients with very extensive tumours of pelvis could never be operated on, they never achieved CR despite chemo- and radiotherapy and died. SUMMARY: Of 10 patients submitted to surgery 7 patients are alive and 4 have an excellent functional result. Of the 2 patients in whom the tumour was inoperable neither is alive. It seems justified to recommend surgical resection of Ewing's sarcoma / PNET; satisfying reconstruction is usually feasible. RTx seems to have value rather as a method supplementing minor failures of surgery than as the only local treatment.