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Brain tumours are the commonest childhood neoplasm, with a worldwide incidence of 29.9-47.1/million (1). Childhood brain tumours carry substantial morbidity/mortality and are the largest cause of paediatric cancer deaths (2). Historically, classification was largely based on histological features. In recent years, expansion of high-resolution genomic, epigenetic and transcriptomic profiling has led to improved molecular understanding and categorisation, as well as targeted therapies (3). Consequently, the 2016 WHO classification incorporated molecular features in some brain tumour entities (4). According to this classification, astroblastomas are extremely rare, not formally graded, and listed under 'other gliomas'. Astroblastomas are generally treated by surgery alone but can display intermediate behaviour with high recurrence rates and unpredictable behaviour (5, 6). Controversy exists as to whether astroblastomas are a truly distinct entity as they have histological features in common with both astrocytomas and ependymomas (7-9). Diagnosing astroblastomas is therefore challenging and misclassification can alter subsequent management (8). The present case demonstrates how recent molecular advances identified a novel gene fusion for this patient, confirmed a more precise tumour diagnosis and guided subsequent management decisions. The findings here have general importance to other rare paediatric brain tumour entities.

The possible impact of interpregnancy interval (IPI) on perinatal outcomes has long been studied, however, a definition of the optimal interval is still not clear. Both short and long IPIs have been associated with obstetrical syndromes and short and long-term complications. In this study, we sought to explore the impact of IPI on the hazard for neoplasm of the offspring, thus contribute to the present literature in determining the preferred birth spacing. We aim to investigate the association between short and long IPIs and the hazard for childhood neoplasm of the offspring. A population-based retrospective cohort analysis comparing offspring neoplasm hazard following three different IPIs. Exposure was defined as short (60 months) IPIs, whereas intermediate IPI (6 months − 60 months) served as the comparison group. The study included singleton live births in a tertiary regional hospital between 1991 and 2014. Offspring were followed for 18 years, and all hospitalization records for neoplasm diagnoses were collected. Kaplan–Meier survival curves were used for the cumulative incidence of neoplasm morbidity, and Cox proportional hazards models were used to control for confounders. During the study period, 144,397 deliveries met the inclusion criteria. Of those, 18,947 (13.1%) occurred in women with short IPI, 114,012 (79%) in women with intermediate IPI, and 11,438 (7.9%) in women with long IPI. 61 benign neoplasms and 80 malignant neoplasms were registered in offspring born after long IPI. The total percentage of neoplasm were the highest in the long IPI group versus the intermediate and short IPI groups (malignant − 0.7%, 0.6%, 0.5% respectively, benign − 0.5%, 0.4%, 0.3% respectively). Controlling for maternal age, diabetes mellitus, preterm delivery, birth weight, smoking, cesarean section, and fertility treatments, long IPI was found to be independently associated with high hazard for long-term pediatric neoplasm related hospitalizations (adjusted HR 1.39, 95% CI 1.09, 1.77). Short IPI may be associated to decreased hazard for childhood neoplasms (adjusted HR 0.74, 95% Cl 0.59, 0.92). Long IPI is associated with a high hazard for childhood neoplasms, compared with intermediate and short IPIs. Short IPI may be associated with decreased hazard for childhood neoplasms.

Acute lymphoblastic leukaemia (ALL) is a relevant form of childhood neoplasm, as it accounts for over 80% of all leukaemia cases. T-cell ALL constitutes a genetically heterogeneous cancer derived from T-lymphoid progenitors. The diagnosis of T-ALL is based on morphologic, immunophenotypic, cytogenetic, and molecular features, thus the results are used for patient stratification. Due to the expression of surface and intracellular antigens, several subtypes of T-ALL can be distinguished. Although the aetiology of T-ALL remains unclear, a wide spectrum of rearrangements and mutations affecting crucial signalling pathways has been described so far. Due to intensive chemotherapy regimens and supportive care, overall cure rates of more than 80% in paediatric T-ALL patients have been accomplished. However, improved knowledge of the mechanisms of relapse, drug resistance, and determination of risk factors are crucial for patients in the high-risk group. Even though some residual disease studies have allowed the optimization of therapy, the identification of novel diagnostic and prognostic markers is required to individualize therapy. The following review summarizes our current knowledge about genetic abnormalities in paediatric patients with T-ALL. As molecular biology techniques provide insights into the biology of cancer, our study focuses on new potential therapeutic targets and predictive factors which may improve the outcome of young patients with T-ALL.

Objective: The aim of this study was to evaluate the association between large for gestational age (LGA) at birth and future risk of childhood neoplasm. Study design: a population-based cohort to compare the long-term risk (up to the age of 18 years) of childhood neoplasms (benign and malignant) in children that were born LGA vs. those that were appropriate for gestational age (AGA), between the years 1991&ndash, 2014. Childhood neoplasms diagnosis were defined according to international classification of disease 9 (ICD-9) codes recorded medical files. Kaplan&ndash, Meier survival curves were used in order to compare cumulative incidence of oncological morbidity over the study period. The Cox proportional hazards model was used to control for confounders. Results: 231,344 infants met the inclusion criteria, out of those 10,369 were diagnosed LGA at birth. Children that were LGA at birth had a higher incidence of leukemia (OR 2.25, 95%CI 1.08&ndash, 4.65, p = 0.025) as well as kidney tumors (OR = 4.7, 95%CI = 1.02&ndash, 21.9, p = 0.028). In addition, cumulative incidence over time of childhood malignancies, leukemia, and kidney tumors were significantly higher in LGA children (Log Rank = 0.010, 0.021, and 0.028, respectively). In a Cox regression model controlling for other perinatal confounders, LGA at birth remained independently associated with an increased risk for childhood malignancy (adjusted HR 1.51, 95%CI 1.02&ndash, 2.23, p = 0.039). Conclusion: LGA at birth is associated with increased long-term risk for childhood malignancy and specifically leukemia and kidney tumors. This possible link may help to improve current knowledge regarding potential exposures that are associated with childhood cancer development.

Chromiec Tomasz, Widłak Karolina, Hetman Marcin, NurzyńskaFlak Joanna. Head and neck rhabdomyosarcoma in a 4 year old girl – case report. Journal of Education, Health and Sport. 2018;8(9):814-819 eISNN 2391-8306. DOI http://dx.doi.org/10.5281/zenodo.1415407 http://ojs.ukw.edu.pl/index.php/johs/article/view/5956 https://pbn.nauka.gov.pl/sedno-webapp/works/877175 The journal has had 7 points in Ministry of Science and Higher Education parametric evaluation. Part b item 1223 (26/01/2017). 1223 Journal of Education, Health and Sport eissn 2391-8306 7 © The Authors 2018; This article is published with open access at Licensee Open Journal Systems of Kazimierz Wielki University in Bydgoszcz, Poland Open Access. This article is distributed under the terms of the Creative Commons Attribution Noncommercial License which permits any noncommercial use, distribution, and reproduction in any medium, provided the original author (s) and source are credited. This is an open access article licensed under the terms of the Creative Commons Attribution Non commercial license Share alike. (http://creativecommons.org/licenses/by-nc-sa/4.0/) which permits unrestricted, non commercial use, distribution and reproduction in any medium, provided the work is properly cited. The authors declare that there is no conflict of interests regarding the publication of this paper. Received: 02.08.2018. Revised: 18.08.2018. Accepted: 13.09.2018. Head and neck rhabdomyosarcoma in a 4 year old girl – case report Tomasz Chromiec, Karolina Widłak, Marcin Hetman, Joanna Nurzyńska-Flak Student Scientific Circle at the Department and Clinic of Pediatric Hematooncology, Medical University of Lublin ABSTRACT Introduction: Rhabdomyosarcoma is the most common soft tissue neoplasm in the pediatric population. Its diagnosis may prove to be a dauting task due to non-specific symptoms it tends to produce, which is explainable by the fact that it may arise in any part of the body. Both histology and the place of origin of the tumour significantly affect the prognosis. Case report: A four year old girl with a history of non-painful, heamopurulent ear discharge, which was initially diagnosed as chronic granulomatous otitis media presented at the Department of Pediatric Otolaryngology upon the recurrence of these symptoms. The biopsy results showed an embryonal rhabdomyosarcoma-like infiltration and the patient was referred to the Department of Pediatric Hematooncology at the Children's Hospital. The patient underwent a resection that had been preceded by a course of neoadjuvant chemotherapy. Following the surgery, a course of chemoradiotherapy was implemented. Currently the patient remains in remission. Conclusions: An early diagnosis is crucial in any neoplastic disease and rhabdomyosarcoma is no exception. Such diagnosis should be entertained in any instance of otolaryngologic symptoms that are refractory to standard treatment. Key words: rhabdomyosarcoma. childhood neoplasm, otorrhea

Highlights • The present study analyzed the diagnosis pattern of childhood malignant diseases using public health data and viral surveillance data in Korea. • The number of newly diagnosis showed the highest monthly average in January, and the lowest in September. • Seasonal trends were apparent in acute myeloid leukemia, Non-Hodgkin’s lymphoma, acute lymphoblastic leukemia and germ cell tumors. • In winter, significantly more patients were diagnosed with acute lymphoblastic leukemia, acute myeloid leukemia, neuroblastoma, and Hodgkin’s lymphoma than in other seasons. • There was a temporal correlation with the diagnostic trends of several diseases and the prevalence of recent human parainfluenza virus., Background Several studies have reported a seasonal trend in the diagnosis of childhood cancer suggesting seasonal factors such as infection. The present study aimed to analyze the diagnosis pattern of childhood malignant diseases using public health data, and to compare this pattern with seasonal viral infection trends. Method Using the open data source of the Health Insurance Review and Assessment Service, we extracted data regarding all patients under 21 years of age and who had any cancer, aplastic anemia or myelodysplastic syndrome between September 2009 and December 2013. The positive detection rates of 11 viruses was collected from the surveillance data of Korea Centers for Disease Control and Prevention, and seasonality analysis were conducted with both data. Results In total, 9085 patients were diagnosed with malignant disease during the study period; there were about 175 new cases per month on average. Monthly stacked time series by year showed an apparent seasonal variation with the highest monthly average in January as 236, and the lowest in September as 120. In winter, significantly more patients were diagnosed with acute lymphoblastic leukemia, acute myeloid leukemia, neuroblastoma, and Hodgkin’s lymphoma than in other seasons. There was a temporal correlation with the diagnostic trends of several diseases and the prevalence of recent human parainfluenza virus. Conclusion This study tentatively suggests that the diagnosis of childhood malignancy follows a seasonal trend in Korea, and has a possible correlation with viral prevalence in several diseases. Further long-term analysis of epidemiological data is needed to explore possible causality.

Esparru kontzeptuala eta justifikazioa: Gaur egun, urtero 1000 haur dira gutxi gora behera minbiziz diagnostikatzen direnak Espainian. Nahiz eta helduen minbiziak baino intzidentzia baxuagoa izan, honek izugarrizko inpaktua du bai paziente bai familiarengan, gurasoek bizitzako egoerarik traumatikoena bezala definituz. Hala ere, egoera honen aurrean ikusi da erresilientzia garatzea posible dela, erresilientzia ezbeharrak modu konstruktibo batean gainditu eta hazkuntza prozesu pertsonal bat bezala definituz. Honi gehituz, gurasoek hainbat aurre egite estrategia (coping) erabiltzen dituzte egoera honi aurre egiteko. Hori dela eta, haurren gurasoak aldaketa traumatikoen aurrean beren barne oreka bio-psiko-sozialari eusteko gai izateaz gain, hazkuntza prozesu bat garatzean erresiliente kontsideratuko dira, eta horretarako, aurre egite estrategia eraginkorrak zeintzuk diren identifikatzea beharrezkoa da. Helburua: Paziente onkologiko pediatrikoen gurasoen erresilientzia prozesuan lagungarriak diren aurre egite estrategiak identifikatzea. Metodologia: 2008 eta 2019 urte bitartean argitaratutako bibliografia erabili da literaturaren berrikusketa burutzeko, bilaketa gaztelaniaz eta ingelesez publikatutako artikuluetara mugatuz. Datu base hauek kontsultatu dira: Pubmed, Medline, Cinhal, Psycinfo, Cochrane Library eta Cuiden. Horrez gain, eskuzko bilaketak ere burutu dira, horretarako Psycho-Oncology, Cancer Nursing, European Journal of Oncology Nursing, Clinical Journal of Oncology Nursing, European Journal of Cancer Care, Journal of Advanced Nursing eta Psicooncología aldizkarietan eta RNAO eta NICE praktika klinikorako gidak kontsultatuz. Guztira 23 artikulu aukeratu dira proposatutako helburuari erantzuteko. Emaitzak eta eztabaida: Minbizi pediatrikoari aurre egitean, guraso gehienak erresilientzia garatzeko gai direla ikusi da. Erresilientzia garatzeko aurre egite estrategiak beharrezkoak dira, ondorengoak lagungarriak izanik: arazoaren ebazpena, sostengu soziala bilatzea (familia, osasun talde eta egoera berdinean dauden gurasoengan), berregituratze kognitiboa eta emozioen adierazpen erregulatua, informazio bilaketa eta otoitz egitea. Bestalde, isolamendu soziala, saihestea edo abolizio emozionala eta distrakzioa coping positibo edo negatiboaren barne dauden zalantzan dago, hauek erresilientziaren garapenean lagungarriak ez izateagatik. Ondorioak, inplikazioak eta mugapenak: Gurasoengan erresilientziaren garapena eman ohi dela ikusi arren, lagungarriak diren aurre egite estrategiak identifikatzeko ikerketa gehiagoren beharra dagoela ikusi da. Bestalde, erizainaren inplikazioaren lerrotik, honek ezinbesteko garrantzia du gurasoen erresilientzia prozesuaren garapenean, gaur egun formakuntza gehiagoren beharra identifikatuz. Organizazio mailatik, gurasoen aurre egite eraginkorrean eta erresilientzia prozesu horretan laguntzeko interesgarria litzake erizainaren papera ospitalizazioan ez ezik lehen mailako arretan eta talde terapietan integratzeko aukera izatea.

Neuroblastoma (NB) is a heterogeneous and particularly malignant childhood neoplasm in its higher stages, prone to form metastasis in selected organs and for which there is still no efficient treatment available beyond surgery. Evidence indicates that chemokines and their receptors present involvement as mediators of neuroinflammation and have a neurophysiological role. In the present study, we aimed to verify if CCR5 (rs333) and CXCL12 (rs1801157) allelic variants were associated with NB. For CCR5 (rs333) D32 carriers (OR: 5.96, IC: 2.21-16.06) and for CXCL12 genotype 3'A/3'A (OR:26.18, IC:6.15-111.4) there were statistically significant differences as well to allelic frequency (OR:4.20, IC: 2.19-8.03). Although no correlation was verified regarding prognostic parameters for both CCR5 and CXCL12 polymorphic variants, these polymorphisms may be associated with NB susceptibility which deserve attention for future investigations.

Testicular tumors are relatively uncommon in children. The tumors of germ cell origin are still frequently encountered. The pure embryonal rhabdomyosarcomas of testis, not associated with sarcomatous component of the germ cell tumor, is a very rare entity. It is a highly aggressive tumor of childhood and young adolescents. In testes, it arises commonly from paratesticular tissue; primary intratesticular being extremely rare. To the best of authors knowledge, only fifteen cases of primary pure intratesticular tumor have been reported in the literature so far. We report a case of eight-year-old child who presented with a progressive painless right testicular enlargement over two months. Ultrasonography showed a heterogenous intratesticular mass. High orchidectomy was performed. Histopathological examination of the specimen assisted with immunohistochemistry revealed embryonal rhabdomyosarcoma.

Objective The aim of this study was to evaluate the association between maternal smoking during pregnancy and future risk of childhood neoplasm risk. Study design A population based cohort analysis comparing the risk for long-term childhood neoplasms in children born (1991–2014) to mothers that smoked during pregnancy vs. those that did not. Childhood neoplasms were pre-defined based on ICD-9 codes, as recorded in the hospital medical files. Children with congenital malformations and multiple gestations were excluded from the analysis. Kaplan-Meier survival curves were constructed to compare cumulative oncological morbidity over time. Cox proportional hazards model was used to control for confounders. Results 241,273 infants met the inclusion criteria; out of those 2841 were born to mothers that smoked during pregnancy. Offspring to smoking mothers had higher incidence of benign (OR 1.6, 95%CI 1.02–2.58; p value = .038) but not malignant tumors. Total cumulative neoplasm incidence was significantly higher in smoking women (Log Rank = 0.001) but no significant difference in the incidence of malignant tumors was noted (Log Rank = 0.834). In a Cox regression model controlling for maternal confounders; a history of maternal smoking during pregnancy remained independently associated only with increased risk for benign tumors (adjusted HR 2.5, 95%CI 1.57–3.83, p = .001). Conclusion Maternal smoking during pregnancy is associated with increased long-term risk for benign but not malignant tumors. This is important when counseling mothers regarding potential future risks and recommended lifestyle modifications. Despite this large population study with long follow-up, childhood malignancies are rare, and clarifying the possible association may require further studies.

Malignant rhabdoid tumor (MRT) is a highly aggressive childhood neoplasm and mainly presents in kidney and brain. We report the case of a patient with extrarenal extracranial MRT in the craniovertebral junction (CVJ). A 3-year-old boy presented with tetraparesis that had rapidly developed for 2 weeks. The tumor was located in the ventral side of the C1 and C2 extradural space and had invaded the lower clivus. The symptom recovered after subtotal resection of the tumor through a lateral approach with removal of bilateral lateral masses at C1 and instrumental occipital-C2 posterior fusion. Definite histological diagnosis of the extradural tumor was difficult; however, a metastatic lesion in the rib showed a proliferation of INI1/SMARCB1-negative spindle and rhabdoid cells, indicating the tumor was MRT. Stereotactic CyberKnife radiotherapy with a marginal dose of 26 Gy contributed to control of local regrowth of the tumor before histopathological confirmation and induction of chemotherapy with ifosfamide, cisplatin, and etoposide. The patient survived for 29 months without local recurrence of the tumor and with independent activity, however then died of multiple metastases. Treatment strategies for MRT in the CVJ should include an optimal surgical approach for reduction of tumor volume and stabilization of the spine, followed by high dose chemotherapy. Stereotactic radiotherapy may be useful for local control.

Genetic factors are important for developing primary and subsequent malignancies in children. This study investigated the role of genetic factors involved in DNA-repair. Designed as a feasibility study, it addressed the possibility of obtaining samples for genetic analyses from former patients through the German Childhood Cancer Registry. Testing feasibility was as important as the biological question itself. We analyzed the expression of DNA-repair genes in untreated primary fibroblasts of 20 individuals with a second neoplasm compared to 20 matched single neoplasm cases using customized cDNA microarrays (1344 gene sequences, about 800 genes). Matching was by first neoplasm, age, and year of first diagnosis. Forty-six percent of the 52 contacted second neoplasm cases and 18% of the 132 single neoplasm patients participated in the study. The DNA-repair gene results show small differences in the basal gene expression of FTH1 and CDKN1A. To our knowledge, this is the first study using gene expression arrays in untreated primary fibroblasts regarding second neoplasms after a childhood neoplasm. We were able to recruit childhood cancer patients for genetic analyses long after diagnosis. The biological importance of the differences in the DNA-repair gene expression has to be elucidated yet.

Neuroblastoma is the most common extracranial solid tumor to occur during infancy and early childhood. However, primary renal neuroblastoma is rare, and only scattered case reports exist in the English medical literature. We report 8 cases that accumulated at our institution over the past 15 years and summarize their clinicopathologic features. The composite picture of a patient with renal neuroblastoma is that of a boy of 17 months of age, who presented with a large renal mass, about 9 cm in size, accompanied by hypertension. The mass was typically hemorrhagic, either encapsulated or unencapsuated, and infiltrating. A renal neuroblastoma can be undifferentiated, poorly differentiated, or differentiating; it falls into either the favorable or the unfavorable histology category, and presentation at higher stages is the rule. The N-myc is usually unamplified, and the bone marrow is usually not involved at presentation. Unless the tumor is undifferentiated or very poorly differentiated, patients with renal neuroblastoma fare well, although not without new and improved modalities of treatment. Primary renal neuroblastoma is perhaps more common than people realize; a higher level of awareness and early recognition are important for its prognosis and management, as they are very different from Wilms tumor.

Background Childhood cancer remains the leading cause of disease-related mortality for children. Whereas, improvement in care has dramatically increased survival, the risk factors remain to be fully understood. The increasing incidence of childhood cancer in Florida may be associated with possible cancer clusters. We aimed, in this study, to identify and confirm possible childhood cancer clusters and their subtypes in the state of Florida. Methods We conducted purely spatial and space–time analyzes to assess any evidence of childhood malignancy clusters in the state of Florida using SaTScan™. Data from the Florida Association of Pediatric Tumor Programs (FAPTP) for the period 2000–2007 were used in this analysis. Results In the purely spatial analysis, the relative risks (RR) of overall childhood cancer persisted after controlling for confounding factors in south Florida (SF) (RR = 1.36, P = 0.001) and northeastern Florida (NEF) (RR = 1.30, P = 0.01). Likewise, in the space–time analysis, there was a statistically significant increase in cancer rates in SF (RR = 1.52, P = 0.001) between 2006 and 2007. The purely spatial analysis of the cancer subtypes indicated a statistically significant increase in the rate of leukemia and brain/CNS cancers in both SF and NEF, P

Purpose It is known that the prognosis of childhood cancer is relatively good, however actual representative nationwide data on childhood cancer, particularly of survival rate, are rare. In this study we attempted to establish the overall survival rate of major childhood cancer. Materials and methods The primary source of data of childhood cancer under 15 years of age were the registry files of the Central Cancer Registry Report (Ministry of Health & Welfare) from 1993 to 1997. The above data was compared to death case data files of the same period obtained from the Korea National Statistical Office using the personal identification code. We calculated the 1, 3, and 5 year survival rates using the life table of SPSS and Kaplan-Meier method and compared the survival rate of disease according to prognostic factors. Results A total of 6,720 cases of pediatric cancer from the Central Cancer Registry files were computerized and sorted by personal identification (ID) code to extract duplicated cases as well as cases with incomplete data. The final number of cases entered in this study was 4,983. 1) The number of confirmed death cases was 1,448 (29.1%). 2) The disease distribution showed that the most common pediatric cancer was leukemia (1,468/4,983, 29%), followed by brain tumors (503/4,983, 10%), lymphoma (315/4,983, 6%), Wilms tumor (165/4,983, 3%), etc. in order by number of patients. 3) The 5 year survival rate of disease was as follows: overall 62%, acute lymphocytic leukemia 61%, acute non-lymphocytic leukemia 32%, malignant lymphoma 72%, neuroblastoma 47%, medulloblastoma 51%, Astrocytoma 66%, Wilms tumor 83%, etc. Conclusion We analyzed and report the 5 year survival rate of overall childhood cancer and of each of the twelve major childhood cancers from in Korea 1993 to 1997 to provide basic data on childhood cancer statistics.

The melanotic neuroectodermal tumor of infancy (MNTI) is a rare childhood neoplasm with an alarming but classical clinical presentation. We present the case of a 2-month-old male infant treated with surgery for an aggressive MNTI on the alveolar process of the maxilla. Radiographic examination showed a diffuse osteolytic radiolucent lesion in the right maxilla, and displacement and dysmorphic changes in the developing primary tooth buds. The patient remained well without evidence of recurrence for 6 months after surgery. We discussed clinicopathological features, management alternatives, and outcome.

Learning Objectives After completing this course, the reader will be able to: Appreciate the heterogeneity of neuroblastoma and identify clinical and biological prognostic factors. Explain the determinants and the clinical significance of the neuroblastoma risk stratification system. Select appropriate treatment regimens for neuroblastoma patients that are tailored according to risk stratification. Access and take the CME test online and receive one hour of AMA PRA category 1 credit at CME.TheOncologist.com Neuroblastoma, a childhood neoplasm arising from neural crest cells, is characterized by a diversity of clinical behavior ranging from spontaneous remission to rapid tumor progression and death. To a large extent, outcome can be predicted by the stage of disease and the age at diagnosis. However, the molecular events responsible for the variability in response to treatment and the rate of tumor growth remain largely unknown. Over the past decade, transformation-linked genetic changes have been identified in neuroblastoma tumors that have contributed to the understanding of tumor predisposition, metastasis, treatment responsiveness, and prognosis. The Children's Oncology Group recently developed a Neuroblastoma Risk Stratification System that is currently in use for treatment stratification purposes, based on clinical and biologic factors that are strongly predictive of outcome. This review discusses the current risk-based treatment approaches for children with neuroblastoma and recent advances in biologic therapy.

The melanotic neuroectodermal tumor of infancy (MNTI) is a rare childhood neoplasm with a classical clinical presentation. It appears as a rapidly enlarging pigmented mass in the upper jaw and can be easily mistaken for a malignant neoplasm. Although possessing an aggressive growth rate and radiographic appearance, the MNTI almost always behaves in a benign fashion and can be treated with local excision. However, few cases of malignant transformation and recurrences have been reported in literature and that makes follow-up very important. This paper describes two cases of MNTI with classical histological presentation.

Lipoblastoma is a benign lesion of immature fat cells that is found almost exclusively in pediatric population. This tumor is a rare tumor that occurs in infancy and early childhood, accounting for less than 1% of all childhood neoplasm. It is more common in male than in female and often presents as an asymptomatic, rapidly enlarging, soft lobular mass on the extremity. Although benign, it gives great difficulty in its management, due to its extensions into different facial planes, especially in lipoblastomatosis. Thus, complete surgical excision is the treatment of choice.

Considerando que os tumores cerebrais correspondem à segunda neoplasia mais freqüente na infância, sendo apenas superados pelas leucemias, realizamos um estudo clínico prospectivo em setenta pacientes com tumor cerebral, com idade variando de um a quinze anos, por um período de quatro anos (1993-1997), através de acompanhamento ambulatorial. Avaliamos quarenta e um pacientes do sexo masculino e vinte e nove do sexo feminino. Observamos uma freqüência discretamente maior de tumores infratentoriais (38 casos) em relação aos de localização supratentorial (32 casos). O tumor mais freqüentimente observado foi o meduloblastoma (21 casos), seguido pelo astrocitoma (15 casos) e pelo germinoma (11 casos). No acompanhamento ambulatorial, observamos que 75,5% dos pacientes evoluíram com sequelas neurológicas. Em 34,3% houve recidiva tumoral e 21,4% dos pacientes evoluíram para óbito. Taken as proved that brain tumors are the second most frequent childhood neoplasm - only outnumbered by leukemias - we have undertaken a clinical perspective study with seventy brain tumor patients ranging from one to fifteen years of age, throughout a four-year period (1993-1997), based on ambulatory-oriented follow-up. Forty-one male and twenty-nine female patients were analyzed, in that a slightly higher number of infratentorial tumors was observed (thirty-eight cases), compared to those supratentorially located (thirty-two cases). The most repeatedly observed during the study was the medulloblastoma (twenty-one patients), followed by the astrocytoma (fifteen patients) and the germinoma (eleven patients). It should be pointed out that during the ambulatory follow-up 75,5% of patients developed neurological sequels. A tumor recurrence was noticed in 34,3% of them, while 21,4% eventually died. Universidade Federal de São Paulo (UNIFESP) Escola Paulista de Medicina University of Taubaté Universidade Federal de São Paulo (UNIFESP) Escola Paulista de Medicina Department of Neurology and Neurosurgery Universidade Federal de São Paulo (UNIFESP) Escola Paulista de Medicina Pediatric Neuro-oncology Division UNIFESP, EPM, Department of Neurology and Neurosurgery UNIFESP, EPM, Pediatric Neuro-oncology Division SciELO

This paper describes the database of children with cancer in the EUROCARE study and the methodology used to analyse and report survival. This is the first systematic evaluation of survival after childhood cancer on a large scale in Europe: approximately 45 000 cases were included, diagnosed between 1978 and 1992 (34 814 cases diagnosed in 1978–1989 and an additional set of 9495 cases diagnosed in 1990–1992) and followed-up until 1995. Data were provided from 34 population-based registries (four specialised for childhood cancer registrations and one specialised registry for childhood leukaemia) in 17 countries of Europe (where there was national coverage in 10 countries). Quality of the data was fairly good, given the general differences among the countries and their health systems, thereby allowing for comparisons between them. Among cases diagnosed in 1978–1989, overall 2.0% were lost to follow-up, 91.8% were microscopically diagnosed and 93.4% of alive cases had at least 5 years of observation. Survival proportions (observed survival) were calculated for each of the countries involved, by age group (0, 1–4, 5–9, 10–14 years), gender, different time periods and selected diagnostic groups. Age-standardised cumulative survival rates and European averages (weighted and pooled) were also computed. Cox regression models were used to evaluate geographical and temporal differences. The EUROCARE database represents a unique source of information on survival of childhood cancer patients in Europe, intercountry differences and time trends in survival.

Cancer is an infrequent disease in childhood. However, it is responsible for 13.06% of child deaths between 1 and 14 years in Cuba. The aim of our work was to describe the incidence of childhood cancer in the period 1986 to 1990 using data reported to the National Cancer Registry (NCR) of Cuba. All cancer cases between 0 and 14 years of age reported to the NCR in the period 1986 to 1990, were included. The cases were classified histologically, by age and gender. Average age-specific and age-standardized rates were calculated; 1428 children were registered, an average of 286 each year, with a mean annual rate of 117.8 per million. The world-population standardized rate was 120.7 and the male-to-female ratio was 0.98. Leukaemias, lymphomas and malignant tumours of the central nervous system were the most common childhood neoplasm groups. The majority of leukaemias were acute lymphoblastic leukaemias (ALL), and the incidence peak extended until 6 years of age. The first peak of incidence of Hodgkin's disease is found in older age-groups. Burkitt's lymphoma showed a male-to-female ratio of 0.44. Most of the hepatic tumours were carcinomas, and only one fourth were hepatoblastomas. In bone tumours, similar rates were observed for osteosarcomas and Ewing's sarcoma. Int. J. Cancer 72:551–555, 1997. © 1997 Wiley-Liss, Inc.

Ewing sarcoma, including classical Ewing sarcoma of the bone and primitive neuroectodermal tumors arising in bone or extraosseous primary sites, is a highly aggressive childhood neoplasm. We present two cases of Ewing sarcoma arising from the vagina in young girls. Previously reported cases in literature focused on their pathologic rather than radiographic features. We describe the spectrum of multimodality imaging appearances of Ewing sarcoma at this unusual primary site. Awareness of vaginal Ewing tumors may facilitate prompt diagnosis and lead to a different surgical approach than the more commonly encountered vaginal rhabdomyosarcoma.

Objective: Progress in the diagnosis and treatment of childhood neoplasm in the past few decades is one of the most gratifying achievements in the field of oncology. This study was aimed to ascertain the burden (incidence and prevalence) and histopathologic features of neurogenic tumors occurring in the pediatric population. Materials and Methods: The study evaluated a total of 492 cases of pediatric tumors over a period of 8 years from 2007 to 2015, including patients of 0–12 years of age group; attending the out-patients and in-patients Department of Pediatrics and Pediatric Surgery at Jawaharlal Nehru Medical College, AMU, Aligarh, with the complaints of tumor or tumor associated sign and symptoms. Clinical profile, hematological and histopathological examination along with immunohistochemical analysis were implicated to attain a conclusive diagnosis. Result: Out of 492 pediatric tumor cases, 255 (52%) cases were benign and 237 (48%) cases were malignant. Neurogenic tumor (brain tumors) comprised 49 (10%) of the total case, being most common malignant solid neoplasm and second most common (next to leukemia) overall malignancy, constituting 49/237 (21%) cases. Astrocytoma 22/49 (45%) cases were the most common type brain tumor followed by medulloblastoma 15/49 (31%), ependymoma 9/49 (18%), and craniopharyngioma 3/49 (6%). Conclusion: Effective management of pediatric neurogenic tumor is a multipronged approach involving effort of good Pediatric neurosurgeon, Pathologist and a host of Oncology specialists with insight into childhood neoplasms.

Survival rates for childhood cancers were analyzed with a total of 2,209 cases who were registered in a population-based cancer registry in Osaka, Japan in 1975-1984. These cases were reclassified according to Birch's classification and the survival rate of each diagnostic group was calculated by Kaplan-Meier methods. Death certificate-only cases, which amounted to 3.9% of all incidence, were excluded from the calculation. The five-year cumulative survival rate for both sexes was 46% for all cancer children. Among 12 major diagnostic groups, the most favorable survival was seen in retinoblastoma (87.5%), followed by renal tumors, epithelial neoplasms, and gonadal and germ-cell tumors. The outcome was unfavorable in leukemias, sympathetic nervous system tumors, hepatic tumors and malignant bone tumors. Comparing the survival in 1975-1979 with that in 1980-1984, the rate for all childhood cancer rose from 41% to 51%. Improvement in survival was also observed in 4 groups; acute lymphocytic leukemia, acute non-lymphocytic leukemia, non-Hodgkin's lymphoma and osteosarcoma. One attributable factor for the rise of survival was proved to be improvement of medical treatment by Cox's hazard model analysis. Comparison of survival rates in Osaka with those in England and the U.S. revealed that the prognosis for acute lymphocytic leukemia and acute non-lymphocytic leukemia was less favorable in Osaka than in England and the U.S.

Background. Dermatofibrosarcoma protuberans (DFSP) is an intermediate grade soft tissue neoplasm originating from the dermal layer of the skin. It usually occurs in adults; however, it can rarely be seen in infancy and childhood. Diagnosis of DFSP in children is quite difficult-given-rarity of this lesion, its variegated appearance, and its presentation sometimes at unusual sites.Case. We present the case of five-year-old boy who came with painless lesion on a forehead. Fine needle aspiration cytology (FNAC) suggested possibility of mesenchymal neoplasm. Patient was advised excision biopsy. Final diagnosis of DFSP was made based on histopathological findings. The patient was then advised reexcision surgery with wide margins. The patient was lost to followup and later turned up after two months with recurrence of a similar swelling at the same site.Conclusion. DFSP in children is rare and difficult to diagnose. Treatment of childhood DFSP is often delayed leading to incomplete excision. Hence, there is need to recognize and appropriately manage this uncommon childhood neoplasm.

Syskon till barn med cancer ägnas lite tid inom hälso- och sjukvården. De är ofta emotionellt försummade av hälso- och sjukvårdspersonal samt närstående. Syftet med studien var att belysa behovet av stöd hos syskon till barn med cancer. Metoden var en litteraturstudie innehållande totalt 16 vetenskapliga artiklar. Artiklarna var både av kvalitativ och kvantitativ ansats. Det framkom i re- sultatet att syskon till barn med cancer upplevde känslor av oro, ångest, avundsjuka, ilska, irritation, skuld, ensamhet, nedstämdhet och symptom på de- pression. Hos de friska syskonen som fick stöd minskade de negativa känslorna markant. Det är av yttersta vikt att sjuksköterskan uppmärksammar behovet av stöd hos syskonen och att stödet individ- anpassas. Sjuksköterskan behöver kunskap i hur de tar reda på vilket behov av stöd de friska syskonen har. Dessutom behövs kunskap om konkreta stödåt- gärder som sjuksköterskan kan använda. Siblings of children with cancer is given little timein the health care system. They are often emotionallyneglected by health care professionals and relatives.The aim of the study was to highlight the need forsupport to siblings of children with cancer.The method was a literature review containing a totalof 16 scientific articles. The articles were ofboth qualitative and quantitative method. It was found in the results that siblings of children with cancer experienced feelings of worry, anxiety,jealousy, anger, irritability, guilt, loneliness andsymptoms of depression. The negative feelingsdecreased significantly when the healthy siblingsreceived support. It is important that the nurse recog-nizes the need for support among siblings and thatthe support is individualized. Nurses need knowledge of how to find out which need of support the healthy siblings have. Furthermore, knowledge is needed about specific support actions that nurses can use.

In 1971–1988, 4,021 malignant tumors occurring among children under 15 years of age were registered in the Osaka Cancer Registry, a population‐based registry which covers Osaka Prefecture, Japan. These patients were reclassified into 12 diagnostic groups by Birch's scheme using information on clinical diagnosis, histology and primary site. The annual age‐standardized incidence rate for childhood cancer per million children was 130.3 for males and 104.9 for females in 1971–88. Comparing the incidence rates for both sexes in 1981–88 with those in 1971–80 in Osaka, we observed a significant decrease of acute non‐lymphocytic leukemia (ANLL) and a significant increase of all cancers, acute lymphocytic leukemia, non‐Hodgkin lymphoma, sympathetic nervous system tumors, soft‐tissue sarcomas, and gonadal and germ‐cell tumors. Age‐standardized incidence rates in around 1971–80 of the above‐mentioned diagnostic groups were compared among 4 population‐based registries; Osaka, Miyagi (Japan), SEER (U.S.), and the National Registry of Childhood Tumors (England and Wales). Rates for ANLL and gonadal and germ‐cell tumors were higher and those for other diagnostic groups were lower in Osaka, especially for Hodgkin's disease. Thus, in 1980–88 in Osaka, rates for Hodgkin's disease remained low and rates for gonadal and germ‐cell tumors increased, though rates for other cancers appeared to resemble the levels in caucasian populations. The incidence of childhood cancer in Japan was estimated according to the diagnostic groups in Birch's scheme.

The majority of childhood cancer cases survive to adulthood. We describe the experience of marriage and reproduction as indicators of quality of life, in a population-based cohort of adult long-term survivors after early cancer reported to the Childhood Cancer Registry of Piedmont. The study included 1237 survivors with a malignant neoplasm diagnosed during 1967-2000 when aged 0-14 years, who attained age 18 years. Vital and marital status and number of offspring were assessed through the Vital Statistics Offices. Marriage and fertility deficits were estimated by comparison with the Piedmont population. Among the individuals included in this study, 919 (74.3%) never married and never lived as married. The marriage deficit was 32% [observed/expected 0.68; 95% confidence interval (CI): 0.55-0.83] in men and 18% (observed/expected 0.82; 95% CI: 0.68-0.98) in women. A total of 179 children were born to 120 women, with a fertility deficit of 41% (observed/expected 0.59; 95% CI: 0.51-0.69). In conclusion, the observed decrements in marriage in men and women and fertility in women suggest that efforts should be made to improve the recovery from physical and psychological traumas related to diagnosis and treatment of cancer.

We report a boy with acute lymphoblastic leukaemia (ALL) treated with chemotherapy and prophylactic cranial irradiation to a dose of 24 Gy. Six years after diagnosis he developed a glioma and died. Prior to 1979, four cases of second malignant neoplasm (SMN) of the brain had been reported in children treated for ALL. These SMNs occurred within 2 years of the original diagnosis (median 1.3 years) and at least two of four patients had not received prior radiotherapy. Since 1979, 28 cases of SMN of the brain have been reported including nine of 468 (1.9%) long-term survivors in one study. All occurred more than 3.7 years from diagnosis (median 6.5 years; range 4-13 years) and all received cranial irradiation (median 24 Gy; range 20-48 Gy). These data indicate a change in the pattern of SMNs which is most likely due to the introduction of cranial irradiation. As well, the frequency of SMNs in children treated for ALL appears to have increased, although it is still no greater than the risk of SMNs developing following the treatment of any other primary childhood neoplasm.

This study explores the relationship between histologic variants of bone sarcomas and previous therapy in patients in whom an unrelated malignant neoplasm had been diagnosed during childhood. Sarcomas of bone were the most common second malignant neoplasm (SMN) reported to the Late Effects Study Group, a 13institution consortium consisting of pediatric oncology centers from western Europe, Canada, and the United States. The authors attempted to relate the histologic subtypes of the 91 bone tumors to clinical factors such as previous therapy and genetic predisposition because morphologic variants have been shown to have biologic significance in other tumors and may have etiologic import. The literature concerning the subtypes of bone tumors, clinical and experimental, is also reviewed. The authors also investigated the effect of several factors on the time interval from the first diagnosis to the SMN (ie., the bone sarcoma). Anthracyclines significantly shortened the interval by about 3 years. The primary diagnosis also significantly affected the interval, with leukemia/lymphomas having the shortest interval and retinoblastoma the longest. The authors could not demonstrate any significant relationship between morphologic characteristics of the osteosarcoma and predisposing conditions. However, lesions diagnosed as chondrosarcoma and malignant fibrous histiocytoma occurred almost exclusively in patients who had received radiation therapy to the site in which the SMN developed. Cancer 67:193-201,1991, HEN MORE THAN ONE cancer occurs in a person, W the opportunity arises to investigate etiologic factors for carcinogenesis. Usually environmental agents are implicated, some of them being associated with therapy for the first cancer. The possibility that genetic factors may play a role should also be considered. Genetics is especially important for one childhood neoplasm, retinoblastoma, in which the association with osteogenic sarcoma in those with the genetic form is well known.’ Osteogenic sarcoma, however, is most often seen after ra

The aim of this study was to compare the characteristics of respondents and nonrespondents in a survey of childhood cancer survivors recorded in the Childhood Cancer Registry of Piedmont and their current primary care general practitioners. Eligible subjects were identified from the Childhood Cancer Registry of Piedmont and the referring general practitioners were traced through the National Health Service. A postal questionnaire was sent both to childhood cancer survivors and to their general practitioners. Prevalence odds ratios were estimated for demographic and clinical characteristics in survivors and for demographic characteristics in general practitioners. A total of 1005 childhood cancer survivors and 857 general practitioners (132 of them had two or more cancer survivors in care) were included in the study. Completed questionnaires were obtained from 691 survivors (69%) and 615 general practitioners (72%). For survivors, the only associations with nonresponse were for age 35-44 years [prevalence odds ratio: 0.53 (95% confidence interval: 0.33-0.85)], being married [prevalence odds ratio: 1.45 (95% confidence interval: 0.96-2.18)] and diagnosis after 1977 [prevalence odds ratio: 0.66 (95% confidence interval: 0.42-1.03)]. For general practitioners, the only associations were for male sex [prevalence odds ratio: 1.62 (95% confidence interval: 1.13-2.32)] and place of work outside of the city of Turin [prevalence odds ratio: 1.93 (95% confidence interval: 1.07-3.47)]; furthermore associations were relatively weak. An association was also found between nonresponse in survivors and nonresponse in their general practitioners [prevalence odds ratio: 3.40 (95% confidence interval: 2.54-4.56)]. In conclusion, apart from age, marital status and period of diagnosis, there were little differences between respondent and nonrespondents, for the considered clinical and demographical characteristics. Participation of survivors and their general practitioners correlated, suggesting that involvement of the general practitioners in the study may be a method to increase participation of survivors of childhood cancers.

Rhabdoid tumor (RT) is an uncommon childhood neoplasm that typically arises within the kidney. It is characterized by an aggressive clinical course. Since its description in 1978, several cases of primary extrarenal RT, including a CNS localization, have been reported. The first case in the CNS was reported in 1985 and was defined as “rhabdoid tumor” initially, and was classified as grade IV in the most recent classification of the World Health Organization under the term of “atypical teratoid/rhabdoid tumor”. Nearly 200 cases of atypical teratoid/rhabdoid tumor of the CNS have been reported to date, most of them occurring in childhood. We report a case of primary RT of the brain located in the right frontal lobe with the clinical, radiographic and pathological features presenting at an unusual age. This tumor, which was composed purely of rhabdoid cells with no additional primitive neuro-ectodermal, epithelial and mesenchymal components, was in a 27-year-old male patient. In conclusion, RT should be considered also in the differential diagnosis of intracerebral neoplasms of adult patients.

Adrenal cortical carcinoma (ACC) is a rare childhood neoplasm that seldom manifests brain metastases; hence few papers in the literature focus on neurological manifestations associated with ACC. Although ACC is known to be a signature tumor type in several inherited cancer predisposition syndromes, particularly Li Fraumeni, ACC has not been previously associated with neurofibromatosis, type 1 (NF-1), an inherited disorder with frequent CNS lesions that might prompt concern for metastatic disease by neuroimaging studies. We present two pediatric patients with ACC and unusual CNS findings. The first child developed metastasis to the brain 4 years after resection of his adrenal primary and 2 and 1 years, respectively, after metastases to the liver and lungs. Soon after our experience with this patient, a girl with known NF-1 presented with virilization; adrenalectomy disclosed an ACC and systemic metastases were found within months. Disseminated disease prompted concern that her complex intracranial lesions identified by neuroimaging studies might represent brain metastases, but this proved to be NF1-related hamartomatous lesions. We review the literature on ACCs in pediatric patients regarding brain metastases and previous associations with NF-1.

Benign mullerian papilloma of the female reproductive tract is a rare childhood tumor that can easily be mistaken by those unfamiliar with the entity for botryoid rhabdomyosarcoma. Ultrastructural findings have been mentioned only in two individual case reports, and these both were issued many years ago. The aim of this update is to familiarize the reader with the clinical, light, and electron microscopic features associated with this distinctive entity, and thereby hopefully preclude the risk of making a serious diagnostic error. Two cases are illustrated, one very typical in its presentation and the other less so.

Time changes in mortality rates can be used to suggest future trends in the prognosis of childhood cancer. Estimates provided from recent studies led to divergent previsions of future trends. In this study we used data from the population-based Childhood Cancer Registry of Piedmont (CCRP) in order to measure the decrease in mortality. The study included 1,060 deaths in the period 1971–1998. Analyses were carried out using Poisson regression models and considered separately the total tumours, acute lymphoblastic leukaemia (ALL) and tumours of the central nervous system (CNS). We observed a progressive decline in the mortality rates: for all tumours the estimated annual percentage change was −3.6% (P