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BACKGROUND: Central nervous system (CNS) germinoma relapses are rare. However, the latest Children’s Oncology Group (COG), ACNS1123 stratum 2, described three relapses along the biopsy tract out of eight total relapses; two along the biopsy and endoscopic third ventriculostomy (ETV) tract and one along the biopsy tract and external ventricular drain. Given these findings, we sought to describe other cases of GCT recurrence along surgical tracts. METHODS: We performed a literature review from 1980-2021 to identify cases of GCT recurrence along biopsy and ventriculostomy tracts. RESULTS: Three patients with pineal lesions were identified (13, 14, and 34-years old). All underwent ETV, cerebrospinal fluid (CSF) sampling, and tumor biopsy. Two patients had uncomplicated postoperative courses. One developed persistent hydrocephalus and underwent repeat ventriculostomy. Two were diagnosed with pure germinoma and treated with focal radiotherapy of 45 Gy alone or chemotherapy and radiation (50.4 Gy to the tumor volume and 36 Gy to adjacent high-risk area). The third was treated with methotrexate and corticosteroids. All three had recurrence on surveillance MRI (1-3 years after diagnosis). One had recurrence at the ventriculostomy bur hole site, and two had recurrence along the ventriculostomy tract. On biopsy, all three lesions were germinomas. One received pre-radiation chemotherapy and palliative radiation (36 Gy to surgical bed and 30.6 Gy to surrounding high-risk area). Another received chemotherapy and craniospinal radiation of 15 Gy. The third had a complicated post-biopsy course. He ultimately underwent chemotherapy and radiation (24 Gy). All were still alive at time of report. Two were disease free at 12 and 18 months, while one was still receiving chemotherapy. CONCLUSION: Recurrence along the biopsy or ventriculostomy tract is rarely reported in the literature. Future evaluation to determine the incidence could inform if biopsy and ventriculostomy tracts should be included in the radiation therapy field.

Background/aim Nivolumab is an immune checkpoint inhibitor with high antitumor activity in selected neoplasms. The aim of the study was to evaluate the efficacy and safety of nivolumab in pediatric patients with various types of highly malignant advanced tumors. Patients and methods Ten patients with a median age of 15.1 years were included in the study. The indications for treatment were: malignant skin melanoma (n=5), brain tumor (n=2), malignant melanoma of the brain (n=1), Hodgkin lymphoma (n=1) and soft tissue sarcoma (n=1). Results Complete disease remission was observed in 4 patients. Overall survival at 24 months from diagnosis for the entire group was 0.36. Two patients receiving combination therapy of nivolumab and ipilimumab did not achieve a remission. Adverse events of immunotherapy were observed in 4/10 patients. Conclusion Nivolumab is a promising option in pediatric advanced malignancies. Treatment with immunotherapy was relatively well tolerated, and emerging side-effects were manageable.

Medulloblastoma, the most common malignant pediatric brain tumor, is a heterogeneous disease, with the existence of at least four molecular types: Wingless (WNT), Sonic Hedgehog (SHH), Group 3 and Group 4 tumors. The latter two groups, which can be identified by an application of multi-gene expression or methylation profiling, show sometimes ambiguous categorization and are still classified for diagnostic reason as non-SHH/non-WNT medulloblastomas in updated WHO 2016 classification. In order to better characterize non-SHH/non-WNT tumors, we applied the method based on the Nanostring nCounter Technology, using the 26 genes codeset in 68 uniformly treated medulloblastoma patients. This allowed for identification of tumors, which shared common Group 3 and Group 4 gene signatures. We recognized three transcriptional groups within non-WNT/non-SHH tumors: Group 3, Group 4 and the Intermediate 3/4 Group. Group 3, in line with previously published results, showed poor prognosis with survival rate

ALK gene rearrangements were identified in a variety of cancers, including neuroblastoma, where the presence of ALK expression is associated with adverse prognosis. ALK mutations have recently been found in the pediatric brain tumor medulloblastoma, and microarray data indicate that ALK is highly expressed in a subset of these tumors. Therefore, we investigated whether ALK expression correlates with transcriptional profiles and clinical features of medulloblastoma. Tumors from 116 medulloblastoma patients were studied at diagnosis for the detection of ALK expression at the RNA level by an application of NanoString technology and at the protein level by immunohistochemistry using antibody ALK clone D5F3. The results indicate that ALK expression, at both the RNA and the protein levels, is strongly associated with the WNT-activated type of tumors and therefore may serve as a useful marker for the detection of this type of medulloblastoma. Importantly, ALK protein expression alone is also an indicator of good prognosis for medulloblastoma patients.

Introduction Diffuse intrinsic pontine glioma (DIPG) is a rare clinically, neuro-radiologically, and molecularly defined malignancy of the brainstem with a median overall survival of approximately 11 months. Our aim is to evaluate the current tendency for its treatment in Europe in order to develop (inter)national consensus guidelines. Methods Healthcare professionals specialized in DIPG were asked to fill in an online survey with questions regarding usual treatment strategies at diagnosis and at disease progression in their countries and/or their centers, respectively. Results Seventy-four healthcare professionals responded to the survey, of which 87.8% were pediatric oncologists. Only 13.5% of the respondents biopsy all of their patients, 41.9% biopsy their patients infrequently. More than half of the respondents (54.1%) treated their patients with radiotherapy only at diagnosis, whereas 44.6% preferred radiotherapy combined with chemotherapy. When the disease progresses, treatment strategies became even more diverse, and the tendency for no treatment increased from 1.4% at diagnosis to 77.0% after second progression. 36.5% of the healthcare professionals treat children younger than 3 years differently than older children at diagnosis. This percentage decreased, when the disease progresses. Most of the participants (51.4%) included less than 25% of their patients in clinical trials. Conclusion This survey demonstrates a large heterogeneity of treatment regimens, especially at disease progression. We emphasize the need for international consensus guidelines for the treatment of DIPG, possible by more collaborative clinical trials.

INTRODUCTION: Pineoblastoma is a rare, highly aggressive supratentorial tumour, more frequently diagnosed in young children. The prognosis remains poor. AIM: To present own experience with children with pineoblastoma. PATIENTS AND METHODS: 14 pts: 8 girls and 6 boys treated between 1998 and 2017 were analyzed. 5 pts (36%) were under 3 years of age. 7 pts underwent complete tumour resection, 2-partial resection and 5- biopsy. At the onset of treatment six patients had disseminated disease. 9 pts were treated according to protocol for MB HR group (with radiotherapy), 5 pts according to own baby protocol. RESULTS: 9 pts (64%) achieved good response to treatment (CCR-4 pts, CR-4 or PR-1). Four patients experienced tumour progression. 7 out of 14 patients are alive 3 ms to 17years from diagnosis (median 3years 4ms). Seven patients died (10ms to 8years from diagnosis). 1 pt died of long term post- radiotherapy sequelae. 5 yr EFS was 23 %, OS- 57%. 10 yr EFS and OS were 23 and 30,1% consecutively. 10 out of 14 pts relapsed 7ms to 4 years 3ms from diagnosis. 9 pts received second line treatment- 4 are alive. Children younger than 3 years of age treated without radiotherapy had worse outcome with 5 yrs OS estimated at 0% vs 74,1% in older children treated with chemotherapy and radiotherapy (p=0,014). CONCLUSION: Pineoblastoma is highly malignant CNS neoplasm of childhood with dismal prognosis. In our series children below 3 years of age treated without radiotherapy had poorer responses and worse outcome.

OBJECTIVES: Previous analyses indicated that expression of ALK at the RNA level is correlated with the WNT-activated type of medulloblastoma. Therefore, we investigated if ALK expression at the protein level may serve as an useful marker for identification of this type of medulloblastoma. METHODS: Tumors from 134 patients were studied at diagnosis by immunohistochemistry using antibody ALK clone D5F3 #3633 (Cell Signaling). Consensus scoring was applied as the following: 0 (negative) for 0-10%, 1+ for >10-50%, 2+ for >50-100% of positive tumor cells. Transcriptional subtypes of medulloblastoma tumors were established in all cases. Thirty infratentorial ependymal tumors were also investigated to determine if ALK protein expression may serve as a marker for differential diagnosis. RESULTS: All 19 WNT tumors were positive for ALK expression and in all but one positive reaction was present in >50% of tumor cells (score 2+). Only four ALK positive tumors were detected among the remaining 85 tumors: one tumor with a score 2+ in Group 4 and three tumors with a score 1+ (one in Group 3 and two in SHH group). Therefore, expression of ALK is strongly associated with the WNT type of disease (p

The aim of the study was to asses QoL in Medulloblastoma (MB) survivors according to risk groups. MATERIAL AND METHOD: Between 2005-2015 132 patients (40 girls, 92 boys aged 2 9/12 – 17 6/12) were treated of MB. 44 patients were standard risk and 88 high risk. Patients in SR group received radiotherapy to CNS 25Gy whereas HR - 36Gy. Age at analysis 6 1/12 to 29. Analysis included 5 year EFS/OS and assessment of: weight/height in percentiles according to risk groups and age at diagnosis, hearing loss, hearing aids, hair loss and occurrence of posterior fossa syndrome(PFS). Psychosocial assessment, including IQ, education, work, physical and social activities, interpersonal relationships was performed and compared in risk groups. RESULTS: 5 years EFS was 83% in SR and 76,6% HR (p=0,112), OS 89,7% and 82,3%. There was no significant difference in hearing loss between risk groups, but there was greater use of hearing aids in the latter (p=0,003), hair loss was markedly worse in HR (p=0,022). SR patients and those older than 10 years at diagnosis were higher as compared to other survivors. PFS occurred more often in HR group (p=0,005). IQ, educational achievements, physical and social activity and interpersonal relationships were significantly better in SR group (p

Astroblastoma is a rare CNS tumor of glial origin distinguished from other gliomas in the recent International Classification of Tumors of the Central Nervous System proposed by the World Health Organization. We present our experience with 3 astroblastoma patients. Between 2003–2017 among 978 patients with tumors of glial origin treated at our Institution 3 patients (0,3 %) were diagnosed with astroblastoma. There were 2 girls and 1 boy aged 2.5, 3, 5.5 years. Two patients had supratentorial tumors, one tumor was located infratentorially. All patients underwent subtotal tumor resection received pre-irradiation chemotherapy, local irradiation (54Gy) and maintenance chemotherapy. All patients are alive 1, 5 yrs 8 m, 13 yrs2m from diagnosis. Our results as of other authors confirm favorable prognosis and rarity of astroblastoma in pediatric population.

Background: Papillary pineocytoma is an extremely rare tumor usually with a poor outcome. Case report: We report a case of a 10-year-old-girl with pineal gland tumor and obstructuve hydrocephalus diangnosed using MRI. The child was successful treated by insertion of a ventriculoperitoneal shunt and consecutive tumor resection by supracerebellar-infratentorial approach. Histopathological examination showed a papillary structure of the pineocytoma. As such, tumors are considered to be aggressive the child was subjected to radio- and chemotherapy. Conclusion: At six year follow-up after surgery, the patient is symptom-free and the MRI shows no tumor recurrence.

LG-49. LOW GRADE GLIOMA (LGG) OF THE SPINAL CORD. SPECIFIC LOCATION NEEDS SPECIFIC TREATMENT Marta Perek-Polnik, Iwona Filipek, Monika Drogosiewicz, Ewa Swieszkowska, Wieslawa Grajkowska, Elzbieta Jurkiewicz, Marcin Roszkowski, and Bozenna Dembowska-Baginska; The Children’s Memorial Health Institute, Warsaw, Poland INTRODUCTION: Spinal cord LGG constitute approximately 3,5% of all LGG locations. No specific therapeutic guidelines are available for this specific LGG site. AIM: Analysis of childhood spinal cord LGG. METHODOLOGY: 28 patients treated between2004-2015wereanalyzed for: sex, age, symptoms, pathology, treatment and outcome. RESULTS: There were 20 boys and 8 girls, aged 10 months-17 years, median 4.5 years. Paresis,scoliosis and pain were the most common symptoms. Pathology revealed Pilocytic astrocytoma in 21, Pilomyxoid astrocytoma in 4, LGG NOS in 3 patients. In 5 patients total or subtotal resections was performed, in 17 partial resection, in 6 biopsy were performed. In 13 pts neurological improvement was observed, in 7 – deterioration, the rest remained stable. 13 patients had no further treatment. 15 received chemotherapy: 7 following surgical treatment, 8 at tumor progression. 12 patients started with LGG protocol, 3 with VLB. In 10 patients stabilization, in 5 minimal tumor regression were observed. All patients are alive with median follow-up 3 years 8 months. 5 yr and 10 yr EFS are 52,1%, 27,7, respectively. 10 patients are walking without paresis, 9 with paresis, 2 have scoliosis, 6 pts use wheelchair, 1 is paralized. CONCLUSIONS: Spinal cord LGG have a good prognosis. Surgery improves neurological outcome, but radical resections are not mandated to achieve that. Some patients with tumor residual may not require further treatment, but at tumor progression chemotherapy is the treatment of choice. Quality of life should be the priority in choosing surgical strategy. Neuro-Oncology 18:iii78–iii96, 2016. doi:10.1093/neuonc/now075.49 #The Author(s) 2016. Published by Oxford University Press on behalf of the Society for Neuro-Oncology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

Ependymomas are relatively rare neoplasms of the central nervous system that typically develop along cerebral ventricles and central canal of spinal cord. Occasionally, the tumours of ependymal origin arise supratentorially in brain parenchyma as ectopic cortical mass without any connection to the ventricular system. Ependymomas are heterogeneous group of tumours including cellular, papillary, clear cell and tanacytic histology. The papillary ependymoma is an unusual variant of ependymoma characterized by distinct morphology resembling other papillary tumours and corresponding to WHO grade II malignancy. We present an unique case of ependymoma with distinctive papillary morphology at ectopic superficial cortical localization. The tumour occurred in eleven-years-old girl as a large, well-circumscribed mass in the left parietal lobe without continuity with the ventricular system. The patient presented with severe headache, vomiting and sudden-onset right hemiparesis. Histopathologically, the tumour revealed distinct papillary pattern with numerous pseudorosettes. Immunohistochemically, the neoplastic cells of both papillary structures and pseudorosettes were positive for glial fibrillary acidic protein and vimentin, whereas they were only slightly immunoreactive for epithelial membrane antigen and negative for cytokeratins. Ultrastructural findings revealed the presence of cilia usually located in the neoplastic cell bodies and intermediate glial-like filaments. The final diagnosis of papillary ependymoma at ectopic superficial localization was based on both, immunophenotypic profile and ultrastructural features that confirmed ependymal nature of neoplastic cells.

To describe the incidence and type of central venous catheters (CVC) complications in children treated for solid tumours.Between 1997-2005, 500 paediatric patients were treated for cancer. The CVC complications were analyzed according to the CVC type, blood product transfusion (BT) and parenteral nutrition (TPN). Chi-square test was used for statistics.For 566 surgically inserted CVCs: 147 (25.8%) were ports, 413 (73,6%) tunnelled catheters: Broviacs--227 (39.9%), Groshongs--186 (32.7%) and other--6 (1%). total number of CVC days was 288 944, (median: 422, range: 2-2583). 297 complications (rate of 1.02/1000 CVC days) were observed: 81 catheter infections (0.28), 77 mechanical complications (0.266), 59 no aspiration events (0.204), 52 thrombotic occlusions (0.179) and 28 tunnel infections (0.096). At the end of the study period 121 (28%) CVCs were prematurely removed due to: infection (52), mechanical cause (49), thrombotic occlusion (14), no aspiration (6). Mechanical complications in catheters comparing to ports were more frequent (p= 0.007). There were more infections in Broviacs than Groshongs catheters (p=0.022) and in children receiving BT and TPN (p=0.046 and 0.003).CVC's related complications were relatively rare. Most common were infections and concerned catheters and these complications were most frequent in patients receiving BP and TPN. Risk of mechanical complications was higher in catheters than ports.

Neurofibromatosis type I (NF1) is a relatively frequent autosomal dominant disorder with different manifestations from mild cosmetic problems to severe disease requiring multidisciplinary treatment. THE AIM of our study was lo analyze types of disorders in paediatric population of NF1 patients treated in the Children's Memorial Health Institute and to present the schedule of care adopted in our institution.Medical records of 130 children, 70 girls and 60 boys aged from 1 year to 20 years 3 ms treated in the Children's Memorial Health Institute were analyzed. Type of mutation (familial or sporadic), age at diagnosis, type and frequency of disorders in the whole group of patients and in the familial and sporadic types were assessed.The familial type of NF1 was found in 58 patients, among whom there were, 23 patients from 10 families. It was sporadic in 40 pts and in 32 the type of mutation is unknown. The age at diagnosis ranged from 3 months to 17 years, median 6 years. The most frequent disorders were: T2-weighted hyperintensities of the white matter in 77 pts (59%), schooling problems in 60 pts (46%), skeletal deformations in 49 pts (37%), dysmorphia in 39 pts (30%), hyperactivity in 34 pts (26%), epilepsy in 29 pts (22%). There was a correlation between white matter hyperintensities and the presence of optic nerves gliomas (71%), schooling problems (58%), hyperactivity (77%) and dysarthria (85%). In 65 pts (53%) optic nerve gliomas were found, 45 patients required treatment. Plexiform neurofibroma requiring treatment was found in 25 pts (19%,), 6 had intraspinal penetration and in 4 spine compression was observed. Two patients were diagnosed with ganglioneuroma. Malignant tumours were found in 7 pts (5%), with a prevalence of soft tissues sarcomas. Skeletal disorders occurred more frequently in tNFl sporadic type than in familial cases (p=0.05). Based on our experience a management protocol was formulated. At the time of diagnosis this includes examination by the oncologist, neurologist, ophthalmologist, geneticist and additional investigations: abdominal ultrasound, chest X-rays, MR of the brain; found disorders indicate further consultations and studies. The check-up should be repeated annually and include all previous studies that revealed any abnormalities.1. Diagnosis of NF1 is delayed until complications of the disease occur. 2. In the studied group skeletal disorders were more frequent in the sporadic than in the familial type. 3. The paediatric population with NF1 varies from the adult NF1 patients in type of disorders, diagnostic and treatment requirements. Therefore there is a need for different care standards in the two groups. 4. The knowledge about NF1 and the standards of care should be transferred to general practitioners in order to diagnose these patients early and to refer them to specialized centres.

Despite progress in neuro-oncology, treatment results of children and adolescents with brain stem tumours remain poor. There are also controversies concerning the role of chemotherapy in the treatment of these tumours. THE AIM of our study was to analyze treatment results of patients with brain stem tumours treated with radiotherapy alone and with the addition of chemotherapy and to assess which chemotherapy protocol is the most efficient.Between 1981-2004, 126 patients were treated in our department. The patients were divided into 2 groups according to treatment methods applied. The first group consisted of 49 patients treated with radiotherapy alone. In the second group 77 patients were irradiated and received chemotherapy. Overall survival (OS) at 1 and 5 years was analyzed in both groups. The efficacy of treatment of patients from the second group was assessed by evaluating reaction to radiotherapy, to different chemotherapy protocols and duration of tumour regression. Additionally OS was assessed separately for patients with low grade gliomas treated in the second group.Seven out of 49 patients from the first group are alive with a follow up from 8 years 9 months to 24 years, median 6 years 7 months. In the second group, 25 out of 77 patients are alive with the follow up from 7 months to 8 years 2 months, median--l year 7 months. OS at l and 5 years for patients treated with radiotherapy alone is 48 and 14% and for those receiving additionally irradiation 59 and 18% respectively (statistically insignificant). Best tumour response was achieved in 42% of patients with radiotherapy but in 75% of cases regression lasted from 2 weeks to l year 3 months, median 4 months. Addition of chemotherapy consisting of cisplatinum and temozolamide resulted in tumour regression in 27% of patients lasting from 4 weeks to 4 years 5 months, median 4 months. OS for patients with LGG was 34.6% at 5 years.1. Radiotherapy in brain stem tumours allows to achieve tumour regression but short duration of remission and poor final outcome forces to search for other, effective treatment. 2. Patients with brain stem tumours may benefit from chemotherapy in terms of prolonging survival. 3. Chemotherapy consisting of temozolamide and cisplatinum seems to be promising and might add to improvement of treatment results, but further clinical studies are needed. 4. Patients with low grade gliomas of the brain stem constitute a separate group with a better prognosis.

Adolescents aged 15-19 years are variably included in analyses of childhood cancer. They should be considered separately because tumours that occur in adolescents differ from those in younger children.To describe the distribution of tumour types and treatment results in this group of patients treated in the Department of Pediatric Oncology at The Children's Memorial Health Institute.Retrospective analysis of medical records of patients aged 15-19 years treated in our institution was performed. It included demographic data, tumour types and treatment results.Between 1998-2004, 207 pts, 110 boys and 97 girls aged 15-19 yrs (median--16.5 yrs) were treated. Distribution of tumours was as follows: CNS tumours--74 pts (35.7%), HD -18 pts (8.7%), NHL--13 pts (6.3%), bone tumours--31 pts (15%), STS--23 pts (11.1%), gonadal tumours--12 pts (5.8%), carcinomas--16 pts (7.7%), hepatomas--5 pts (2.4%), neuroblastoma--3pts (1.5%) and other 12 pts (5.8%). Out of 207 pts 130 are alive (62.8%). Seventy seven (37.2%) pts died--64 (83.1%) from disease, 9 (11.6%) from chemotherapy complications, 4 due to other reasons. 111 pts completed treatment and are disease free for 11 months to 7 yrs (median 3 yrs 11 mos) from diagnosis. Nineteen patients are still treated. Treatment results are as follows: CNS tumours--58.1%, HD - 88.8%, NHL--69.2%, bone tumours--51.6%, STS--65.2%, gonadal tumours--83.3%, carcinomas-- 56.25%.Spectrum of malignancies that occur in adolescents 15-19 years of age differs from younger children. Unlike younger patients epithelial carcinomas of adults are observed in this age group. Outcome of treatment is inferior to younger patients. Adolescents should be offered optimal treatment. This specific group should be studied in many aspects.

THE AIM of our study was to evaluate results of conservative treatment of patients with unilateral retinoblastoma.Twenty one patients, 11 boys and 10 girls aged 2 months to 4, 5 years (median age 1 year) were studied. Local disease advancement according to Reese-Elsworth was defined in all patients. Neoadjuvant chemotherapy consisting of Vincristine, Etoposide and Carboplatin was administered. After every 2 courses tumour response was evaluated. Sixteen patients were treated with chemotherapy alone. Local treatment including brachytherapy, thermochemotherapy and cryotherapy was implemented and the choice of the method depended on the tumour's localization, size and response to chemotherapy. Statistical analysis using demographic data and survival curves were performed.On completion of treatment all patients achieved tumour regression. Eleven patients are progression free with a follow-up from 10 months to 6 years 4 months (median--2 yrs 5m). In 10 patients relapse was observed. A total of seven enucleations were performed in the examined group. In histopathological examination viable tumour cells were present in all removed eye balls. Distant metastases were not observed in any of these patients. All patients are alive with a follow-up from 10 months to 9 yrs 6 months (median--4 yrs 7 months) from diagnosis. Disease free survival and ocular survival is 44% and 54% respectively.Neoadjuvant chemotherapy in unilateral retinoblastoma allows to avoid enucleation in some patients.

Proton MR spectroscopy (MRS) is a noninvasive chemical analysis of metabolites in brain tissue. Metabolite ratios are useful because age dependant normal values are known and differ significantly from the values obtained in various diseases of brain tissues.The aim of our study was to evaluate whether MRS correlates with magnetic resonance imaging (MRI), course of disease, pathology of resected tumours after preoperative chemotherapy and can be of prognostic value in patients with medulloblastoma (MB).Eight patients with histologically proven MB were studied. All patients underwent MRS and MRI at diagnosis and after neoadjuvant chemotherapy consisting of VCR,VP,CTX,CBCA. Preoperative chemotherapy was followed by surgery, radiotherapy and maintenance chemotherapy. Assessment whether MRI correlates with MRS after preoperative chemotherapy was performed. Additionally MRS was correlated with the course of disease and pathology results.Out of 8 patients 5 had partial remission of their tumours on MRI. Two of them had favorable reaction on MRS defined as elevation of NAA and Cr decrease in Cho,Lac/Lip. Both are alive and disease free. In 2 patients no changes, compared with MRS at diagnosis were found. Both died of the disease. One had an indistinct positive change and died. Stabilization on MRI was observed in 1 patient, who also had a good reaction on MRS. He is alive, disease free. Two patients had disease progression on MRI. Both had poor reaction on MRS, one died, one is alive with disease. It was noticed that good response on MRS correlated with chemotherapy induced tumour changes observed in pathology specimens from resected tumours. Small number of patients does not allow to draw conclusions but our results signal that MRS might be of prognostic value in brain tumours.

THE AIM of the study was to assess the survival of patients with medulloblastoma (MB)/PNET treated according to own protocol and to compare it with the earlier treatment protocol group.Analysis of 77 patients was undertaken. For the purpose of this study we divided our patients into 2 groups. First group consisted of 34 patients (21 high risk, 12 standard risk, 1 unknown) treated with surgery and radiotherapy and surgery followed by chemo- and radiotherapy. Among the second group including 43 patients (23 HRMB, 6 SRMB, 11 supratentorial PNET's, 3 unknown) treated according to own protocol since 1997. Surgery was performed in all patients. 4 courses of chemotherapy were administered (VCR, VP, CBDCA, CTX, IF, CDDP) and craniospinal irradiation was implemented. Maintenance chemotherapy consisting of 8 courses of VCR, CDDP, CCNU, was given. Event free survival (EFS) in two groups and in high-risk patients of both groups were compared.In the earlier treated group 4 yrs EFS was 50% versus 78% in recently treated patients. In high-risk patients 4 yrs EFS was 40% (with no plateau on the curve) in the historical group and 76% in currently treated.Introduction of our protocol resulted in marked improvement of treatment results, especially since most of our patients were in the high-risk group.

THE AIM of this study was to estimate the value of HMRS in the diagnosis of brain lesions observed in children treated with chemo and radiotherapy and to assess the possibility to differentiate these lesions from neoplasm or recurrent disease.We examined 6 children, aged from 7 to 15 yrs, 3 with brain tumours, 1 with esthesioneuroblastoma treated with chemo and radiotherapy and 2 patients with chemotherapy only, for other neoplasms (leukemia, Wilms tumor). Indications to perform imaging studies were routine for brain tumour patients (no clinical symptoms) and suspicion of disease recurrence in others. MR with 1.5 T scanner and additionally single voxel HMRS in PRESS sequence were performed.In all cases MRI showed extensive hyperintensive changes in brain tissue with significant mass effect. In 3 cases HMRS was within normal limits, in other 3 cases moderately elevated peak of choline and peak of lactate and lipids were found. In follow up examinations all lesions regressed and all patients are in good clinical condition.It could be difficult to differentiate white matter injury from malignancies on MRI. HMRS in case of doubtful findings on MRI in children treated with chemo and radiotherapy, especially in those without symptoms of their disease, is a helpful method.

To evaluate the risk factors for recurrence of MB/PNET we analyzed the medical records of 157 patients treated at the Children's Memorial Health Institute between February 1981 and February 1997. The following factors were evaluated: age at diagnosis, gender, tumor size, tumor cells in the CSF, postoperative status, extent of resection and methods of treatment. We evaluated chemotherapy (CHT) doses, interval between courses, interval between surgery (S) and first course of CHT, interval between S and radiotherapy (RTX), and breaks during RTX. We divided patients into six groups: S alone, S+CHT, S+RTX, S+CHT+ RTX, S+RTX+CHT, S+CHT+RTX+ CHT. Age at diagnosis, gender, tumor size, extent of resection, postoperative status, intervals between courses of CHT, between S and the first course of CHT, and between S and RTX, and breaks during RTX had no statistical influence on relapse occurrence. Tumor cells in CSF were routinely checked for from January 1992 onward. In this group of 75 patients, 40 had tumor cells positive at surgery (28 relapsed), while in the group of 35 patients with negative tumor cells 14 relapsed (P=0.004). Out of 26 patients treated with S+RTX alone, 13 relapsed. Among 14 patients treated with S+RTX and prolonged CHT 6 relapsed. Out of 14 patients treated with S+CHT 13 relapsed; among 49 who received S+CHT+RTX 35 relapsed; and out of 51 patients treated with S+CHT+RTX+CHT 30 relapsed. In the multivariate analysis of treatment methods chemotherapy implemented after radiotherapy had a positive, though not statistically significant, influence on outcome (P=0.06). Among those receiving CHT the mean percentage of the ideal dose administered had a statistically significant influence on relapse: in the group of relapsed patients the mean dose was 76.1%, while in the group in continuous remission it was 83.7% (P=0.0013). On the basis of our data, we conclude that the presence of tumor cells in the CSF had a significant influence on the occurrence of relapse. Administration of appropriate doses of chemotherapy is extremely important for the occurrence of relapse and the final outcome of treatment. Prolonged adjuvant chemotherapy after radiotherapy seems to lower the risk of recurrence.

Summary Background Atypical teratoid rhabdoid tumours (ATRT) are very rare children's cancers. Approximately 200 cases of ATRT located in the central nervous system have been described in the literature up till now. Aim The aim of this report was to analyze the results of treatment of 8 children with these very rare neoplasms of the central nervous system, who were treated according to the Polish Paediatric Brain Tumour Group protocol. Material and Methods Eight children aged from 4 months to 22 years, 5 girls, 3 boys with ATRT of the central nervous system are presented. All children have been operated on and received multidrug chemotherapy; 5 children received radiotherapy as well. In all craniospinal irradiation was applied, in doses of 35 Gy to the whole axis and 55 Gy to tumour boost. Results Five patients died and 3 children are still alive. The progression-free survival of all 8 patients was 3 to 73 months. The overall survival was 5 to 73 months. All living children received radiotherapy. Two of them had total surgical resection and one partial. Conclusions We conclude that radiotherapy prolonged survival in ATRT and should be incorporated in all treatment protocols for patients with this diagnosis.

Recent studies revealed the biological heterogeneity of medulloblastoma, with the existence of at least four groups which are associated with several clinical and morphological features. We investigated for further correlations between molecular types, location of tumours, their contrast enhancement pattern and survival of patients. Altogether 76 tumours were analyzed and molecular subtypes were identified by immunohistochemistry using representative antibodies, detection of chromosome 6 monosomy and CTNNB1 mutation. The site of the tumour was assessed on diagnosis using Magnetic Resonance images and intra-operative surgical reports. In addition, the gadolinium enhancement pattern was also investigated in pre-treatment tumours. Cerebellar hemispheric location was associated with SHH tumours (p 75 % of tumour volume) predicted worse OS and EFS than for those with none/weak or heterogeneous enhancement (>10–75 % of tumour volume), (both p