No abstract

Introduction: Medulloblastoma/primitive neuroectodermal tumour is the most common type of malignant brain tumour in children. Long-term survival rates have improved over the years with a combination of surgical, radiotherapeutic and chemotherapeutic treatment modalities in the developed world. This paper aims to analyse the epidemiology and outcome of medulloblastoma in Singapore and compare our results with those reported in the literature. Materials and Methods: A 9-year retrospective study was done using data reported to the Singapore Children’s Cancer Registry from June 1997 to June 2005. Only 39 children up to the age of 15 years diagnosed histologically with medulloblastoma or primitive neuroectodermal tumour arising from the cerebellum were included in the study. Follow-up data were collected up to June 2006 and analysed using SPSS v 13.0 software. Results: Medulloblastoma/primitive neuroectodermal tumour was the most common type of brain tumour, accounting for 40.7% of all brain tumours diagnosed in children in Singapore. The 5-year event-free survival rate was 44.5%, while the 5year overall survival rate was 51.5%. Nearly half (41%) of our patients had spinal metastasis at presentation and this was associated with a worse event-free survival (6.3% vs 71.9%, P = 0). Children under 36 months of age had a significantly poorer overall survival (28.8% vs 52.2%, P = 0.041). Conclusions: The outcome of medulloblastoma in Singapore was inferior to reported figures in the literature. We need to close identified gaps in care, like standardising assessment and treatment protocols, in order to improve our results. Research into molecular and genetic characteristics may also throw light on whether the disease is inherently more aggressive in our population. Key words: Medulloblastoma, Outcome, Singapore

BACKGROUND: Radiation treatment of GCT brain, in particular germinomas which are highly radio-sensitive,over the years has evolved from the cranio-spinal irradiation(CSI) to more conformal treatment like whole ventricle radiation followed by tumor bed boost (WV + TB), without compromising the results. We report our experience of treatment outcome of patients treated consecutively over a period of more than ten years with both the methods. METHOD: Twelve consecutive patients registered and treated in radiotherapy department between 2000 and 2013 after surgery (biopsy/decompression) and chemotherapy, were analysed in December 2013. Total Number (Histo-pathology) 12 (Germinoma-9, Non-Germinoma-2, No histology-1); Median age (Years) 13 (Range 5-24 years); Gender-M/F (%) 10:2(83:17); Co-morbidities None; Pre-Surgery KPS .90 (92%); Surgery (n) Biopsy/Decompression+ VP shunt (11), No Surgery (1); Sub-site Pineal-7(58%), Supra-sellar-5(42%). TREATMENT: Ten patients received 3D-CRT, while two received IMRT. Three received CSI while nine received WV + TB. CSI dose ranged from 23.4 Gy -36 Gy in 13-20 fractions. For WV the intended dose was 36Gy/20 fractions followed by 9Gy/5 fractions to TB. Pre-radiation all received combination chemotherapy, cisplatin or carboplatin + etoposide + irinotecan. RESULTS: All the patients tolerated and completed treatment with no serious adverse effects. Follow-up was done with serial tumor markers, hormonal, visual, auditory assessment and MRI. Two patients,both non-germinomas treated with CSI progressed and died with-in one year of treatment. Ten patients (9-germinomas, 1-no histology) till date are doing fine,without any loco-regional or distant failures. Ten year overall survival is around 80%.Two patients have developed hormonal imbalance and are on treatment,while one of them developed progressive bilateral optic atrophy which was present prior to radiotherapy. CONCLUSION:Our results are comparable with the reported literature.The overall survival in Germinomas is 100%,Treatment related effects are minimal as a result of reduced dose and limited treatment volume,-contributing possibly to better outcome along with various other reasons. BACKGROUND: Intracranial germ cell tumor (ICGCT) represents 3-15% of pediatric brain tumors. According to the current WHO classification, ICGCTs are classified as germinomas and non-germinomatous germ cell tumors. Germinomas are exquisitely radiosensitive, and chemo-radiotherapy is equally effective. Non-germinomatous germ cell tumors, though more aggressive than germinomas, can be treated effectively with intensive chemo-radiotherapy. ICGCTs in infants and very young children are typically mature teratomas which have excellent outcome after gross total resection. ICGCTs excluding mature teratomas, in this young age group, are extremely rare. There is also no published series or reported incidence of ICGCTs which excludes mature teratomas in children younger than 3 years of age. METHODS AND MATERIALS: We describe a series of 15 ICGCTs excluding mature ter...