Establishing an Internet Based Paediatric Cancer Registration and Communication System for the Hungarian Paediatric Oncology Network

Cancer registration has developed in Europe over the last 50 years, and in the last decade intensive joint activities between the European Cancer Registries, in response to the need of pan-European harmonization of registration practices, have taken place. The Hungarian Paediatric Cancer Registry has been functioning as the database of the Hungarian Paediatric Oncology Network since 1971, aiming to follow the incidence and the treatment efficacy of malignant diseases.The goals of this globally unique open source information system are the following: 1) to raise the quality of the registration system to the European level by developing an Internet-based registration and communication system, modernizing the database, establishing automatic statistical analyses and adding an Internet website, 2) to support clinical epidemiological studies that we conduct with international collaborators on detailed analyses of the characteristics of patients and their diseases, evaluation of new diagnostic and therapeutic methods, prevention programs, and long-term quality of life and side effects.The benefits of the development of the Internet-based registration and communication system are as follows: a) introduction of an Internet-based case reporting system, b) modernization of the registry database according to international recommendations, c) automatic statistical summaries, encrypted mail systems, document repository, d) application of data security and privacy standards, e) establishment of a website and compilation of educational materials.The overall objective of this scientific project is to contribute towards the improvement of cancer prevention and cancer care for the benefit of the public in general and of cancer patients in particular

A gyermekonkológia helyzete Magyarországon

The significance of paediatric oncology is increasing due to its increasing incidence and its leading role among the causes of mortality in children. The epidemiology, prophylaxis and therapy of paediatric malignancies in Hungary are discussed surveying the results and the most important tasks in the future

Az Országos Gyermektumor Regiszter jelentősége a gyermekonkológiai ellátásban.

National Childhood Cancer Registry has been operated since 1971 by the Hungarian Paediatric Oncology Network. This Registry collects data on epidemiology, treatment modalities and effectiveness, as well as late follow-up of childhood cancers. An internet-based paediatric cancer registration and communication system for the Hungarian Paediatric Oncology Network has been introduced in April, 2010. The National Childhood Cancer Registry contains data of all paediatric cancer patients (0-18 yrs) who have insurance covered by the Hungarian Social Security Card. Creation (1971) and operation of the National Childhood Cancer Registry have been very important steps in the field of childhood oncology to evaluate the efficiency of paediatric oncology treatments as well as maximize return on medical investment. Orv. Hetil., 2014, 155(19), 732-739

Improper Supplementation Habits of Folic Acid Intake by Hungarian Pregnant Women: Improper Recommendations

Background: Neural tube defects (NTDs) are some of the most common congenital anomalies. Proper folic acid supplementation is a dominant risk factor, which has been shown to decrease the incidence of NTDs. In Canada, the incidence of neuroblastoma has presented a considerable decrease of 60% as a result of enrichment cereal grain flours with synthetic folic acid. The aim of this study was to investigate the effect of folic acid intake by pregnant women on the incidence of NTDs and neuroblastoma. Methods: Regular folic acid intake has been recommended to pregnant women in Hungary since the eighties of the last century by health visitors eventually raking effect as an official protocol which had been released in 1997. During 2001, 2002 and 2003. folic acid intake habits of pregnant women were evaluated by health visitors, proving to be successful in collecting data front 95.06% of the pregnant women. The incidence of NTDs has been registered by the Hungarian National Centre of Epidemiology, Department of Human Genetics and Teratology. The Pediatric Cancer Registry provided the incidence of neuroblastoma in children. Results: Consistent findings revealed a regular intake of supplementary folic acid products by 68.71% of the pregnant women. Out of these. 93.13% of pregnant women who were taking folic acid, started the supplementation after their 7 weeks of pregnancies, a time designated as the completion period of the development of the neural tube. The dose of folic acid supplementation was evaluated as less than 5 mg/day in 84.75% of the pregnant women. In Hungary, the incidence of NTDs has remained constant, while the incidence of neuroblastoma has shown constant slight increase in spite of the introduction of folic acid supplementation in 1997. Conclusions: Based on our experience, folic acid supplementation was initiated after the recognition of pregnancy and its application in a dose of lower than 5 mg/day neither decreased the incidence of NTDs nor did it have an effect on the neuroblastoma incidence. It is implicated that proper folic acid supplementation, which is started front the conception. can be achieved only with the enrichment of cereal grain flours

A Ewing-sarcomás betegek tünetmentes túlélési esélyeinek értékelése a Gyermekonkológiai Szekció eredményei alapján

Correlation between different prognostic factors and the overall survival of Ewing's sarcoma patients has been investigted. In this study data have been selected from the databank of Hungarian Pediatric Oncologist Section (1988-1999) (n=65). Whenever it was possible statistical analysis has been performed. Results: In our patients time interval from the primary symptoms to the diagnosis was 2-16 months. The average event-free survival in patients suffering from Ewing's sarcoma without metastasis is 0.39. Meanwhile, this value in patients with pulmonary or other metatasis is 0.24 (Kaplan-Meier analysis). Conclusion: Our results show a moderate difference between the Hungarian and the international event-free survival. Late detection is one of the answers of this discrepancy

A gyermekkori Ewing szarkómával szerzett magyarországi tapasztalataink

Magyarországon évente kb. 7-9 új Ewing szarkómás gyermeket diagnosztizálunk. Jelen munkánk célja az volt, hogy az Országos Gyermektumor Regiszter adatait felhasználva megvizsgáljuk a magyarországi Ewing szarkómás gyermekek prezentációs tüneteit, a klinikai paramétereket, a prognosztikai faktorokat, a terápiás és a túlélési eredményeket 1992 és 2002 közötti 11 éves periódusban. A fenti időszakban 88 új beteg került diagnosztizálásra, a fiú – leány arány 1,05 : 1 volt, az átlagéletkor 11 év 7 hónapnak bizonyult. A két leggyakoribb prezentációs tünet a lokális fájdalom és duzzanat voltak. 38 betegünkben hosszú csöves csontra lokalizálódott a megbetegedés, 29 gyermekben a Ewing szarkóma a csípő tájékról indult ki és 21 esetben valamely egyéb testtájékról. A betegeink közel egyharmadánál (29/88) már a diagnózis felállításakor áttétek voltak kimutathatóak. A fent nevezett időszakban három kemoterápiás protokollt alkalmaztunk Magyarországon: a CWS, az EICESS/CESS és 1999. decembere óta az Euro-EWING 99 protokollt. A 88 beteg 37,5%-nál (33/88) észleltünk recidívát átlagosan 22,4 hónappal a primer diagnózis felállítása után. A 88 betegből 45 jelenleg is él, az átlag követési idő 28,6 hónap. Az összes beteg 5-éves kumulatív túlélési valószínűsége 48,06±5,9%, a 10 éves 42,91±6,3%. A metasztázissal rendelkező betegek 5 és 8 éves túlélése 19,91±9,4%, a metasztázis nélküliek 5 éves túlélése 60,23±6,9%, míg a 10 éves 52,82±7,8%. A hazai eredmények megközelítik a nemzetközi adatokat, azonban törekednünk kell a diagnózis korai felállítására és ezáltal a kimondottan rossz prognózisú primer metasztatikus esetek számának csökkentésére. The number of newly diagnosed children in a year with Ewing’s sarcoma is 7-9 in Hungary. The aim of our study was to evaluate the presenting symptoms, clinical features, prognostic risk factors and treatment results of children’s Ewing’s sarcoma in Hungary using data from the National Childhood Cancer Registry in a 11 years-long period between 1992 and 2002. In this period, 88 new patients were diagnosed, the male-female ratio was 1,05:1 and the mean age was 11 years 7 months. The two most common presenting symptoms were local pain and swallowing. Tumor was located in the pelvis area in 29 patients, in the extremities in 38 and other sites in 21 cases. Almost one third of our patients (29/88) had metastasis at the time of the diagnosis. In this time period, three different protocols were used for treatment: CWS, EICESS/CESS and since December 1999 Euro-EWING 99. 37,5% of our patients (33/88) had relapse with a mean of 22,4 months after the diagnosis. 45 children are still alive, the median follow-up time is 28,6 months. The overall survival (OS) of all patients (n=88) was 48,06±5,9% at 5 years and 42,91±6,3% at 10 years. Patients with metastasis had OS 19,91±9,4% at 5 and at 8 years. The 5-year OS of children without metastasis was 60,23±6,9%, and 52,82±7,8% at 10 years. The Hungarian data are similar to international results, but we have to try to decrease the number of the primary metastatic cases with early diagnosis

Szomatosztatin receptor, mint lehetséges differenciál diagnosztikai, prognosztikai és terápiás faktor a gyermekkori medulloblastomák esetében = Somatostatin receptor Acts as a Potential Differential Diagnostic, Prognostic and Therapeutic Factor in Patients Treated with Medulloblastoma

A gyermekkori agytumorok terápiás eredményei nem kielégítőek. Célunk a medulloblastoma sejtjein kimutatott somatostatin receptorok (SSTR2) expressiojának vizsgálata volt; diagnosztikai-, prognosztikai és terápiás alkalmazhatóságának tanulmányozása. Az általunk vizsgált 21 medulloblastomában a SSTR2 kimutatható volt és recidivában nem változott. Az SSTR2 receptort Octreoscan vizsgálattal mutattuk ki, mely medulloblastomán kívül más agytumoros betegek egy részében is pozitiv volt (56 vizsgálat), tehát nincs differenciáldiagnosztikai jelentősége. Az Octreoscan vizsgálat az MRI nagyobb felbontóképessége ellenére hasznosnak bizonyult a medulloblastoma recidivának korai diagnosztikájában, mivel néhány MRI-vel negatív esetben is mutatott ki tumorszövetet. Szövettenyészetben az octreotid antipoliferativ, mitózisgátló hatással bír 40 ?m-nál nagyobb dózisban; apoptoticus hatása nincsen. Az octreotid vincristinnel, cisplatinnal és etoposiddal nem mutatott synergismust. Medulloblastoma xenograftban az octreotid magasabb dózisa gátolta a xenograft növekedését, de hatása a kezelés után egy héttel megszűnt. Mellékhatást nem észleltünk. Új terápiás eljárást dolgoztunk ki medulloblastomás betegek kezelésére, mellyel a 2 éves követési idő után (28 beteg) az eredmények, mind az előző, mind az átlagos nemzetközi eredményekhez képest jelentősen javultak (össztúlélés 82%); a jó prognozisú csoportban eddig egy betegnél észleltünk recidivát. | The present results of the therapy in paediatric brain tumors are unfavorable. Our aim was the study of the somatostatin receptors in medulloblastoma; the examination of its diagnostic-, prognostic- and antitumor effect. The somatostatin receptor (SSTR2) was shown by octreotid scintigraphy in 21 children with medulloblastoma, however, it was positive in some other brain tumors too. Hence it has no differencial diagnostic value. Instead of the greater sensitivity of MRI the application of Octreoscan my be useful in the early detection of a relapse in medulloblastoma, because in some MRI negativ cases it detected tumor tissue. The somatostatin in a higher dose, than 40 ?g had antiproliferativ effect in tissue culture and showed no synergetic effect with vincristin, cisplatin and etoposid. In xenograft the higher dose of octreotid showed antitumor effect, which lasted one week long after the end of treatmen. No side effect was seen. We set up a new treatment protocol for the patients with medulloblastoma. After 2 years long follow up (28 patients) the results are significantly better (OS 82%), than our previous or the international data: only one patient relapsed in the group of low risk patients