P23MANAGEMENT AND OUTCOME OF ADULT MEDULLOBLASTOMAS: ARE WE FOLLOWING RECOMMENDED GUIDELINES?

INTRODUCTION: Medulloblastomas are very rare tumours in adults compared to children. Clinical pathways for medulloblastoma are well established for children. The purpose of this study was to analyse clinical outcome of adult medulloblastomas. METHOD: Single centre retrospective case review from 1995...

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Veröffentlicht in:Neuro-oncology (Charlottesville, Va.) Va.), 2014-10, Vol.16 (Suppl 6), p.vi5-vi5
Hauptverfasser: Srikandarajah, N., Zakaria, R., Barborie, A., Husband, D.J., Jenkinson, M.D.
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container_end_page vi5
container_issue Suppl 6
container_start_page vi5
container_title Neuro-oncology (Charlottesville, Va.)
container_volume 16
creator Srikandarajah, N.
Zakaria, R.
Barborie, A.
Husband, D.J.
Jenkinson, M.D.
description INTRODUCTION: Medulloblastomas are very rare tumours in adults compared to children. Clinical pathways for medulloblastoma are well established for children. The purpose of this study was to analyse clinical outcome of adult medulloblastomas. METHOD: Single centre retrospective case review from 1995 - 2012. Clinical, radiological and neuropathological data were gathered and analysed. RESULTS: 13 patients (9 male, 4 female) were identified; median age 24 years (range: 17-64). Annual incidence in our region is 0.2 cases per million per year. Headache was the most common presenting symptom, mean duration before diagnosis was 8 weeks. Tumours were located in lateral cerebellum (n = 10) and vermis (n = 3). All patients had localized disease at presentation and underwent surgical resection and tissue biobanking followed by cranio-spinal irradiation. Three patients also received adjuvant chemotherapy. MRI spine and CSF analysis were only completed after surgery in all cases. Median follow-up was 32 months (range: 3-126). Mean overall survival (OS) was 92 months (95%CI 62-122 months) with 5 year OS of 55%. Progression free survival (PFS) was 77% with median follow-up of 32 months falling to 56% at 5 years. Three of 13 cases (23%) recurred at a mean of 38 months. All patients who received adjuvant chemotherapy remained recurrence free. CONCLUSION: Diagnosis of adult medulloblastoma is often only made after surgery. Staging MRI should occur pre-operatively to minimize artifact. In our series, surgery and craniospinal radiotherapy was the primary treatment and the role of chemotherapy could be further beneficial. There may be a role for regional management of these very rare tumours.
doi_str_mv 10.1093/neuonc/nou249.21
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Clinical pathways for medulloblastoma are well established for children. The purpose of this study was to analyse clinical outcome of adult medulloblastomas. METHOD: Single centre retrospective case review from 1995 - 2012. Clinical, radiological and neuropathological data were gathered and analysed. RESULTS: 13 patients (9 male, 4 female) were identified; median age 24 years (range: 17-64). Annual incidence in our region is 0.2 cases per million per year. Headache was the most common presenting symptom, mean duration before diagnosis was 8 weeks. Tumours were located in lateral cerebellum (n = 10) and vermis (n = 3). All patients had localized disease at presentation and underwent surgical resection and tissue biobanking followed by cranio-spinal irradiation. Three patients also received adjuvant chemotherapy. MRI spine and CSF analysis were only completed after surgery in all cases. Median follow-up was 32 months (range: 3-126). Mean overall survival (OS) was 92 months (95%CI 62-122 months) with 5 year OS of 55%. Progression free survival (PFS) was 77% with median follow-up of 32 months falling to 56% at 5 years. Three of 13 cases (23%) recurred at a mean of 38 months. All patients who received adjuvant chemotherapy remained recurrence free. CONCLUSION: Diagnosis of adult medulloblastoma is often only made after surgery. Staging MRI should occur pre-operatively to minimize artifact. In our series, surgery and craniospinal radiotherapy was the primary treatment and the role of chemotherapy could be further beneficial. 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Clinical pathways for medulloblastoma are well established for children. The purpose of this study was to analyse clinical outcome of adult medulloblastomas. METHOD: Single centre retrospective case review from 1995 - 2012. Clinical, radiological and neuropathological data were gathered and analysed. RESULTS: 13 patients (9 male, 4 female) were identified; median age 24 years (range: 17-64). Annual incidence in our region is 0.2 cases per million per year. Headache was the most common presenting symptom, mean duration before diagnosis was 8 weeks. Tumours were located in lateral cerebellum (n = 10) and vermis (n = 3). All patients had localized disease at presentation and underwent surgical resection and tissue biobanking followed by cranio-spinal irradiation. Three patients also received adjuvant chemotherapy. MRI spine and CSF analysis were only completed after surgery in all cases. Median follow-up was 32 months (range: 3-126). Mean overall survival (OS) was 92 months (95%CI 62-122 months) with 5 year OS of 55%. Progression free survival (PFS) was 77% with median follow-up of 32 months falling to 56% at 5 years. Three of 13 cases (23%) recurred at a mean of 38 months. All patients who received adjuvant chemotherapy remained recurrence free. CONCLUSION: Diagnosis of adult medulloblastoma is often only made after surgery. Staging MRI should occur pre-operatively to minimize artifact. In our series, surgery and craniospinal radiotherapy was the primary treatment and the role of chemotherapy could be further beneficial. 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Clinical pathways for medulloblastoma are well established for children. The purpose of this study was to analyse clinical outcome of adult medulloblastomas. METHOD: Single centre retrospective case review from 1995 - 2012. Clinical, radiological and neuropathological data were gathered and analysed. RESULTS: 13 patients (9 male, 4 female) were identified; median age 24 years (range: 17-64). Annual incidence in our region is 0.2 cases per million per year. Headache was the most common presenting symptom, mean duration before diagnosis was 8 weeks. Tumours were located in lateral cerebellum (n = 10) and vermis (n = 3). All patients had localized disease at presentation and underwent surgical resection and tissue biobanking followed by cranio-spinal irradiation. Three patients also received adjuvant chemotherapy. MRI spine and CSF analysis were only completed after surgery in all cases. Median follow-up was 32 months (range: 3-126). Mean overall survival (OS) was 92 months (95%CI 62-122 months) with 5 year OS of 55%. Progression free survival (PFS) was 77% with median follow-up of 32 months falling to 56% at 5 years. Three of 13 cases (23%) recurred at a mean of 38 months. All patients who received adjuvant chemotherapy remained recurrence free. CONCLUSION: Diagnosis of adult medulloblastoma is often only made after surgery. Staging MRI should occur pre-operatively to minimize artifact. In our series, surgery and craniospinal radiotherapy was the primary treatment and the role of chemotherapy could be further beneficial. There may be a role for regional management of these very rare tumours.</abstract><pub>Oxford University Press</pub><doi>10.1093/neuonc/nou249.21</doi></addata></record>
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title P23MANAGEMENT AND OUTCOME OF ADULT MEDULLOBLASTOMAS: ARE WE FOLLOWING RECOMMENDED GUIDELINES?
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