OUTCOME OF INTRACRANIAL GERM CELL TUMOURS: 20-YEAR EXPERIENCE OF A SINGLE TERTIARY CENTER FROM A MIDDLE-INCOME COUNTRY

Neuro-oncology(2018)

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摘要
To evaluate the survival outcomes in patients diagnosed with intracranial germ cell tumours (iGCT). Retrospective review of all patients with iGCT treated in a single center from July 1996 to Jun 2017. Three treatment regimens were used: MSKCC, SIOP CNS GCT 96 and SIOP CNS GCT II. Thirty patients were identified with a median follow-up of 4.04 years (range 0.17–19.10). The mean age was 10.23 years. Male: female ratio was 5:1. Visual impairment (50%), endocrinopathy (30%) and precocious puberty (20%) were the commonest presenting symptoms. Duration of symptoms prior to presentation ranged from 1 to 108 weeks. Pure germinomas (n=15) and non-germinomatous germ cell tumours (NGGCT) (n=15) were equally distributed. Tumour location was pineal in 15 (50.0%), suprasellar in 8 (26.7%), hypothalamic in 2 (6.7%) and other sites (basal ganglia, thalamus, frontal lobe) in 5 (16.7%) patients. Eight patients (26.7%) had metastatic disease at diagnosis. Four cases underwent surgical debulking, 14 were biopsied and 7 had complete resection. Fifteen patients received chemo-radiotherapy, 7 had chemotherapy only and 8 underwent radiotherapy only. Elevated levels of serum alpha-fetoprotein and beta-human chorionic gonadotropin normalised after 2 courses of chemotherapy in all NGGCT cases. The 5-year event-free survival (EFS) and overall survival (OS) were 61.3 ± 14.1% and 78.3 ± 11.1% for pure germinoma, respectively. For NGGCT, the 5-year EFS and OS were 36.0 ± 13.3% and 41.3 ± 13.7%. Eleven recurrences occurred within a median duration of 11 months following diagnosis and 8 of them died within 10 months. Compared to pure germinoma, NGGCTs have poor prognosis.
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intracranial germ cell,middle-income
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