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medulloblastoma in children
S258
Pediatric Neurosurgery
IP.6-7051
Adjuvant chemotherapy using ifosfamide, cisplatin and etoposide for PNET/medulloblastoma in children
Yutaka sawamura, Jun-Ichi Murata, Tsutomu Kato, Jun Ikeda,Mitsuhiro Tada, HiroshiAbe. Department of Neurosurgery. University of Hokkaido, Schoolof Medicine, Sapporo, Japan Introduction: The major problems currently associated with management of PNETlmedulioblastoma are high mortalityand late morbidity derivedfrom craniospinalradiotherapy. Thirty-fivechildren with either PNETor medulloblastoma wereassessed to define the role of adjuvantchemotherapy. Methods: Prior to 1991, 23 children were treated mainly with surgery and craniospinal radiotherapy. Since 199t , 12 newly-diagnosed children were treated witha cisplatin-based intensivechemotherapy (ifosfamide 900 mglsqm, cisplatln 20 mg/sqm, etoposide60 mg/sqm, Day 1-5), every4 weeks for 8 cycles. Children under2 years old were treatedwith8 cyclesof the chemotherapy alone, and then irradiated after the age of 2 years. Results: In 23 children treated prior to 1991, 2- and 5-year survival rates were 52% (12/23) and 39% (9/23),respectively. Sevenchildren are alive with a mean observation periodof 161 monthsafter surgery, however, six of them had serious mental retardation and endocrinological deficits. In 12 children treated withnewchemotherapy protocolafter 1991 , complete remissions wereachieved in 10 of 12 patients. Two-yearsurvival rate was 83% (10/12) and disease-free survivalrate was 67% (8/12)with a meanobservation period of 29 months after surgery. Discussion and Conclusions: Cisplatin-based chemotherapy appeared to be an effective adjuvant chemotherapy for management of the children with PNET/medulloblastoma.
IP-6-7061 fossa Brainstem auditory evoked potentials for posterior mass lesions in pediatric patients K. Sestakauskas, I. Uloziena, A.-J. Gruodis. Clinicof Neurosurgery, Kaunas MedicalAcademyHospital, Kaunas, Lithuania Brainstem auditory evoked potentials (BAEP) - a method of objectiveassessment of auditory pathway conduction is not a routinetool in the neurosurgical department. We tried to compare pathologic changesof BAEPwith clinicaland radiological findings in 14 pediatric patients (pts.) wi1h posterior fossa mass lesions. CT revealed brainstem gliomasin 8, cerebellar gliomas- in 5, cerebellopontine angle (CPA)traumatic haematoma - in 1 pt. Abnormalities of BAEP were found in most cases, presenting from prolongation of interpeak latencies to disappearances of BAEP waves. Brainstem gliomasresulted in impaired conductivity in brainstem segments manifesting by almostsymmetric disappearance of waves in 3 pts., prolongation of brainstem conduction time unilaterally in 1, bilaterally - in 1 pt., bilateral prolongation of brainstem and prolonged nerve conduction times unilaterally - in 2 pts., bilaterally - 1 pt. Cerebellar gliomas caused prolongation of brainstem conduction time unilaterally - in 2 patients, bilaterally - in 2 pts., showing worse conduction on the side of major growth of the tumour, according to CT. One pt. with cerebellar glioma had no BAEPabnormalities and only slight clinicalsignsof cerebellar dysfunction. CPA traumatichaematoma resulted in impairednerve and brainstem conduction on the ipsilateral side. Follow-up after 2 weeks of conservative treatment in the latter case revealed improved to almost normalconduction with only increased intersidedifference. We found that BAEP abnormalities corresponded well with CT data and reflected the clinical course of the disease. BAEP could be a routine tool for objectiveassessment of the functional state of the brainstem, especially in not alwayscooperative pediatricpatients.
Ip.6-7071
The dermoid of the calvarium. Case report
A. Dzubera, P. Hlincik, J. Klepoch. Dept. of Neurosurgery of Commenius University DerersHospital, Bratislava, SlovakRepublic The dermoid of the calvarium is a very rare diagnosis. Histologically it is a disgerminal benign cystic tumor, composed of all dermal cells, tissues and structures. Localisation of the dermoids is usually the middle sagittal line. There is a possibility of subdural growthof the cyst. The largestsampledescribedin the literature was to casesover a periodof 18 years. In our 10-yearmaterial there were 2 cases. We report one of them. 14-yearold girt admitted in October 1994. Elasticslow-growth tumorin the right frontoparietal region of calvarium since birth. Twice misdiagnosed calvarium. As an atheroma in an out-patient surgical office after radiodiagrams and CT examination successfulmanagement in our department. CT-scans after 1 year withoutany pathology.
Thursday, 10 July 1997
Ip-B-7oal
An orbital Langerhans histiocytosis case in childhood
AhmetBekar, KayaAksoy, Seref Dogan, BeWI Sevinir, Ahmet Ozmen. Uludag University, Schoolof Medicine, Departments of Neurosurgery, Ophthalmology and Pediatric Oncology, Bursa, 16059, Turkey Introduction: Histiocytosis X is characterized by the localand diffuseinfiltration of monocytes which originate from the promonocytes of the bone marrow. It is classified as eosinophilic granuloma, Hand-Schueller-Chrisfian disease and Letterer-Siwe syndrome from the point of the central nervous system involvement. In prognostic evaluation. There are monocytic, polyocytic disseminated and unifocal skeletal forms. Histiocytosis X of the orbit is reported rarely in childhood. Clinical Presentation: A one-year old body presented with a mass at the temporal side of the right orbit. This mass was present for a month and CT of the orbit revealed a massand bonedestruction at the temporal side of the right orbit. Intervention: The mass was resected totally. The histological findings of positive S-100 protein in the immunocytochemical study suggested the possibility of Langerhans histiocytosis. The patient showed no systemic symptoms. He wastreated withVinblastine 0.001 mg/kg/perweek (intravenously 6 months) and prednisolon 30 mglm2/per day (per orally 4 weeks). Conclusion: During one year follow-up, the patient showedno recurrence. Inthe literature the prognosis is reported to be good in the localizedand unifocal formsof the Langerhans histiocytosis cases.
IP-6-70gl Sinus pericranii: A case of childhood KayaAksoy, AhmetBekar, KudretTOreyen, Suat E. Yelik. U1udag University, Schoolof Medicine, Departments of Neurosurgery, Bursa, 16059, Turkey Introduction: Sinuspericranii is a rarevascularanomalyinvolving an abnormal communication between the extra cranial and intracranial venous circulations. A sinus pericranii case of the left frontoparietal regionwhich appeared to be a posttraumatic case is presented. Clinical Presentation: A 13-yearold patientwas admitted to our clinic with a left frontoparietal extra cranial soft mass. 10 years prior to admission he had fallen down and had an injury of the left frontoparietal region. His clinical evaluation revealed contusion without fracture. The mass was more apparent when the patient was lying down. The direct injection of the contrast medium into the mass revealed that the ertracranial mass cavity was connected to the superiorsagittalsinus, throughthe emissaryveins. Intervention: The mass located in the subgaieal. epiperiosteal space was totally resected and the connection of the intracranial sinus was closed. The mass was diagnosed as sinus pericranii and considered to be secondary to trauma. Conclusion: During3 monthsfollow-up, he showed no recurrence.
IP-6-710 I Arteriovenous malformations of the brain in children W. Szymanski, L. Polis,E. Blaszczyk. PolishMother'sMemorialHospital, Department of Neurosurgery. Lodz, Poland Arteriovenous malformations (AVM) of the brain are not rare in childhood. Pathological arteries, arterioles, veins and venules constitute the nodus of AVM. The combination of an abnormal vessel wall lacking the muscularlayer. and an elevation of blood pressure may produce hemorrhage or changes in the surrounding brain tissue. In the department of neurosurgery 56 children with AVM were diagnosed in the last 5 years. The clinical symptoms were: intracranial hemorrhage in 33 (50.7%): seizures in 12 (21.%); headache in 6 (15.5%); neurological deficit in 4 (8.5%) and other symptoms in 3 (4.2%). CT scansweremadein all patients. Angiography wasperformed in 46 patients. The clinical condition was characterized by the Glasgow Coma Scale (GCS) and the Children Coma Scale (CCS). 47.2% of the children had a score of 13-15 (good condition) , and 20.8% were in poor condition with a GCS score of < 8. The AVMwas classified according to the Spetzler-Martin grading system. In 21 children an emergency operation had to be carried out. The results of surgery were characterized by the Glasgow Outcome Scale (GOS), and relatedto AVM size, clinicalcondition and the grade according to the Speller-Martin system.
I
, P-6-711 ~iag~ostics and surgical treatment of brain abscess In
children
.
C.M. Secara, V.L. Petrachi, A.S. Bajurea, N.G. Predenciuc, A.A. Plesco. Institute for Motherand Child (IMC), Kishinev, Moldova We have analyzed 34 children with brain abscesses operated in the pediatric surgerydepartment of the IMCin Moldova. A total of 42 interventions had been carried out. Etiology: the cause of the abscess was ear-nose-throat pathology requiring anthromastoidotomy in 9 cases; trauma with infection in 8 cases, in-
Pediatric Neurosurgery
IP.6-7051
Adjuvant chemotherapy using ifosfamide, cisplatin and etoposide for PNET/medulloblastoma in children
Yutaka sawamura, Jun-Ichi Murata, Tsutomu Kato, Jun Ikeda,Mitsuhiro Tada, HiroshiAbe. Department of Neurosurgery. University of Hokkaido, Schoolof Medicine, Sapporo, Japan Introduction: The major problems currently associated with management of PNETlmedulioblastoma are high mortalityand late morbidity derivedfrom craniospinalradiotherapy. Thirty-fivechildren with either PNETor medulloblastoma wereassessed to define the role of adjuvantchemotherapy. Methods: Prior to 1991, 23 children were treated mainly with surgery and craniospinal radiotherapy. Since 199t , 12 newly-diagnosed children were treated witha cisplatin-based intensivechemotherapy (ifosfamide 900 mglsqm, cisplatln 20 mg/sqm, etoposide60 mg/sqm, Day 1-5), every4 weeks for 8 cycles. Children under2 years old were treatedwith8 cyclesof the chemotherapy alone, and then irradiated after the age of 2 years. Results: In 23 children treated prior to 1991, 2- and 5-year survival rates were 52% (12/23) and 39% (9/23),respectively. Sevenchildren are alive with a mean observation periodof 161 monthsafter surgery, however, six of them had serious mental retardation and endocrinological deficits. In 12 children treated withnewchemotherapy protocolafter 1991 , complete remissions wereachieved in 10 of 12 patients. Two-yearsurvival rate was 83% (10/12) and disease-free survivalrate was 67% (8/12)with a meanobservation period of 29 months after surgery. Discussion and Conclusions: Cisplatin-based chemotherapy appeared to be an effective adjuvant chemotherapy for management of the children with PNET/medulloblastoma.
IP-6-7061 fossa Brainstem auditory evoked potentials for posterior mass lesions in pediatric patients K. Sestakauskas, I. Uloziena, A.-J. Gruodis. Clinicof Neurosurgery, Kaunas MedicalAcademyHospital, Kaunas, Lithuania Brainstem auditory evoked potentials (BAEP) - a method of objectiveassessment of auditory pathway conduction is not a routinetool in the neurosurgical department. We tried to compare pathologic changesof BAEPwith clinicaland radiological findings in 14 pediatric patients (pts.) wi1h posterior fossa mass lesions. CT revealed brainstem gliomasin 8, cerebellar gliomas- in 5, cerebellopontine angle (CPA)traumatic haematoma - in 1 pt. Abnormalities of BAEP were found in most cases, presenting from prolongation of interpeak latencies to disappearances of BAEP waves. Brainstem gliomasresulted in impaired conductivity in brainstem segments manifesting by almostsymmetric disappearance of waves in 3 pts., prolongation of brainstem conduction time unilaterally in 1, bilaterally - in 1 pt., bilateral prolongation of brainstem and prolonged nerve conduction times unilaterally - in 2 pts., bilaterally - 1 pt. Cerebellar gliomas caused prolongation of brainstem conduction time unilaterally - in 2 patients, bilaterally - in 2 pts., showing worse conduction on the side of major growth of the tumour, according to CT. One pt. with cerebellar glioma had no BAEPabnormalities and only slight clinicalsignsof cerebellar dysfunction. CPA traumatichaematoma resulted in impairednerve and brainstem conduction on the ipsilateral side. Follow-up after 2 weeks of conservative treatment in the latter case revealed improved to almost normalconduction with only increased intersidedifference. We found that BAEP abnormalities corresponded well with CT data and reflected the clinical course of the disease. BAEP could be a routine tool for objectiveassessment of the functional state of the brainstem, especially in not alwayscooperative pediatricpatients.
Ip.6-7071
The dermoid of the calvarium. Case report
A. Dzubera, P. Hlincik, J. Klepoch. Dept. of Neurosurgery of Commenius University DerersHospital, Bratislava, SlovakRepublic The dermoid of the calvarium is a very rare diagnosis. Histologically it is a disgerminal benign cystic tumor, composed of all dermal cells, tissues and structures. Localisation of the dermoids is usually the middle sagittal line. There is a possibility of subdural growthof the cyst. The largestsampledescribedin the literature was to casesover a periodof 18 years. In our 10-yearmaterial there were 2 cases. We report one of them. 14-yearold girt admitted in October 1994. Elasticslow-growth tumorin the right frontoparietal region of calvarium since birth. Twice misdiagnosed calvarium. As an atheroma in an out-patient surgical office after radiodiagrams and CT examination successfulmanagement in our department. CT-scans after 1 year withoutany pathology.
Thursday, 10 July 1997
Ip-B-7oal
An orbital Langerhans histiocytosis case in childhood
AhmetBekar, KayaAksoy, Seref Dogan, BeWI Sevinir, Ahmet Ozmen. Uludag University, Schoolof Medicine, Departments of Neurosurgery, Ophthalmology and Pediatric Oncology, Bursa, 16059, Turkey Introduction: Histiocytosis X is characterized by the localand diffuseinfiltration of monocytes which originate from the promonocytes of the bone marrow. It is classified as eosinophilic granuloma, Hand-Schueller-Chrisfian disease and Letterer-Siwe syndrome from the point of the central nervous system involvement. In prognostic evaluation. There are monocytic, polyocytic disseminated and unifocal skeletal forms. Histiocytosis X of the orbit is reported rarely in childhood. Clinical Presentation: A one-year old body presented with a mass at the temporal side of the right orbit. This mass was present for a month and CT of the orbit revealed a massand bonedestruction at the temporal side of the right orbit. Intervention: The mass was resected totally. The histological findings of positive S-100 protein in the immunocytochemical study suggested the possibility of Langerhans histiocytosis. The patient showed no systemic symptoms. He wastreated withVinblastine 0.001 mg/kg/perweek (intravenously 6 months) and prednisolon 30 mglm2/per day (per orally 4 weeks). Conclusion: During one year follow-up, the patient showedno recurrence. Inthe literature the prognosis is reported to be good in the localizedand unifocal formsof the Langerhans histiocytosis cases.
IP-6-70gl Sinus pericranii: A case of childhood KayaAksoy, AhmetBekar, KudretTOreyen, Suat E. Yelik. U1udag University, Schoolof Medicine, Departments of Neurosurgery, Bursa, 16059, Turkey Introduction: Sinuspericranii is a rarevascularanomalyinvolving an abnormal communication between the extra cranial and intracranial venous circulations. A sinus pericranii case of the left frontoparietal regionwhich appeared to be a posttraumatic case is presented. Clinical Presentation: A 13-yearold patientwas admitted to our clinic with a left frontoparietal extra cranial soft mass. 10 years prior to admission he had fallen down and had an injury of the left frontoparietal region. His clinical evaluation revealed contusion without fracture. The mass was more apparent when the patient was lying down. The direct injection of the contrast medium into the mass revealed that the ertracranial mass cavity was connected to the superiorsagittalsinus, throughthe emissaryveins. Intervention: The mass located in the subgaieal. epiperiosteal space was totally resected and the connection of the intracranial sinus was closed. The mass was diagnosed as sinus pericranii and considered to be secondary to trauma. Conclusion: During3 monthsfollow-up, he showed no recurrence.
IP-6-710 I Arteriovenous malformations of the brain in children W. Szymanski, L. Polis,E. Blaszczyk. PolishMother'sMemorialHospital, Department of Neurosurgery. Lodz, Poland Arteriovenous malformations (AVM) of the brain are not rare in childhood. Pathological arteries, arterioles, veins and venules constitute the nodus of AVM. The combination of an abnormal vessel wall lacking the muscularlayer. and an elevation of blood pressure may produce hemorrhage or changes in the surrounding brain tissue. In the department of neurosurgery 56 children with AVM were diagnosed in the last 5 years. The clinical symptoms were: intracranial hemorrhage in 33 (50.7%): seizures in 12 (21.%); headache in 6 (15.5%); neurological deficit in 4 (8.5%) and other symptoms in 3 (4.2%). CT scansweremadein all patients. Angiography wasperformed in 46 patients. The clinical condition was characterized by the Glasgow Coma Scale (GCS) and the Children Coma Scale (CCS). 47.2% of the children had a score of 13-15 (good condition) , and 20.8% were in poor condition with a GCS score of < 8. The AVMwas classified according to the Spetzler-Martin grading system. In 21 children an emergency operation had to be carried out. The results of surgery were characterized by the Glasgow Outcome Scale (GOS), and relatedto AVM size, clinicalcondition and the grade according to the Speller-Martin system.
I
, P-6-711 ~iag~ostics and surgical treatment of brain abscess In
children
.
C.M. Secara, V.L. Petrachi, A.S. Bajurea, N.G. Predenciuc, A.A. Plesco. Institute for Motherand Child (IMC), Kishinev, Moldova We have analyzed 34 children with brain abscesses operated in the pediatric surgerydepartment of the IMCin Moldova. A total of 42 interventions had been carried out. Etiology: the cause of the abscess was ear-nose-throat pathology requiring anthromastoidotomy in 9 cases; trauma with infection in 8 cases, in-