Low Grade Gliomas treated in the University of Cape Towns Combined - - PowerPoint PPT Presentation
Department of Paediatrics & Child Health Annual Research Day October 2014 Low Grade Gliomas treated in the University of Cape Towns Combined Paediatric Neuro-Oncology Service Alan Davidson, Anthony Figaji, Komala Pillay, Tracy Kilborn,
Alan Davidson, Anthony Figaji, Komala Pillay, Tracy Kilborn, Llewellyn Padayachy, Marc Hendricks, Ann van Eyssen, Jeannette Parkes Haematology/Oncology, Neurosurgery, Pathology, Radiology and Radiotherapy @ RCCH and GSH
Research Day October 2014 Real time decision-making is achieved via a weekly neuroimaging
meeting and regular folder rounds, and long term follow up and surveillance is provided by a combined clinic which attended by paediatric endocrinologists, an educational psychologist and an
Despite involvement by paediatric oncology, chemotherapy only
came into routine use for incompletely resected or inoperable LGGs at the turn of the century.
This study was a retrospective analysis of the epidemiology,
management and outcomes of children with low grade gliomas in
Of the 1516 children diagnosed with malignancy at the Red Cross
Children’s Hospital between 2001 and 2013, 246 had brain tumours representing only 16.2%.
Research Day October 2014
PRIMARY APPROACH
Where possible tumours were resected completely at diagnosis. Irresectable tumours were subject to:
[1] watchful waiting [2] chemotherapy (<5-8yrs) or [3] radiotherapy
Patients with Nf1 were surveilled with regular ophthalmologic
review, and the preference was to defer imaging in asymptomatic
chemotherapy was given. AT RELAPSE OR PROGRESSION
Surgery if possible. Irresectable tumours were assigned to radiotherapy, or second or
third line chemotherapy was initiated.
Research Day October 2014
60 children were diagnosed with LGGs between 2001 and 2013. There 29 boys and 31 girls. Age ranged from 0.41 to 13.75 years The Median age was 5.38 years. Research Day October 2014
Supratentorial: 36 (60%) – 25 (42%) from midline structures Infratentorial: 24 (40%) – 18 (30%) in the cerebellum Metastatic disease was seen in 4 patients: 1 cerebral lesion, 2
hypothalamic tumours and 1 brainstem LGG.
Research Day October 2014
The majority were WHO Grade I (46 patients or 77%) . Patients with WHO grade II LGGs tended to be older than those with
grade I LGGs (median age 8.59 vs 4.42).
Most of those with metastases had WHO grade II tumours.
SACCSG September 2014
TUBEROUS SCLEROSIS
Sub-ependymal Giant Cell Astrocytomas – 2
NEUROFIBROMATOSIS Type 1:
Juvenile Pilocytic Astrocytomas – 2
(1) tectum (2) cerebrum
Optic Gliomas – 4
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SURGERY
SURGERY – 13 debulked / 19 resected (17 cerebellar tumours) BIOPSY ONLY – 22 patients NO SURGERY - 6 patients (all optic gliomas)
OTHER MODALITIES
RADIOTHERAPY – 5 patients CHEMOTHERAPY – 9 patients EXPECTANT MANAGEMENT – 14 (including 5/6 Nf1 patients) Research Day October 2014
NEUROCUTANEOUS SYNDROME PATIENTS
Tuberous Sclerosis: One SEGA was totally resected; the other was
Neurofibromatosis Type 1: The patient with a tectal lesion had
debulking surgery at diagnosis, and then required surgery at
alive and progression free. RADIOTHERAPY
Fifteen patients came to radiotherapy (25%). 1st line therapy 5; 2nd line therapy 4; 3rd line therapy 6. Research Day October 2014
Thirteen patients in all were treated with chemotherapy. Age Range: 0.41 – 8.25 years. Median Age: 2.67 years. Indications: Irresectable tumours 12; Tectal recurrence 1 Sites: Hypothalamus 8 / Optic Tract 3 / Tectum 1 / Cerebrum 1 Histology: Juvenile Pilocytic Astrocytoma 10 Pilomyxoid Astrocytoma 3
Regimens:
1st line - Vincristine/Carboplatin(VC) 1 ; Temozolomide 1;
Vincristine/Etoposide/Carboplatin(VEC) 1
2nd line – VC 3; Vinblastine(Vb) 3; VbC 1 3rd line – Bevacizumab/Irinotecan(BI) 2; Vb 1 Research Day October 2014
Alive and Disease Free 22 Stable Disease off Treatment 26 Stable Disease on Treatment 5 Recurrent Disease – awaiting reoperation 1 Died of disease 3 (2 from shunt complications) Lost presumed dead 2 Lost 1
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OS was better for WHO
grade I tumours (92.3%) than for WHO grade II tumours (74.2%) with a p value of 0.05.
There was no difference in PFS, nor any difference by Site. Five of 18 cerebellar tumours recurred but all were salvaged with
reoperation, and two went on to receive radiotherapy with an OS for the whole group of 100%.
Only 2/15 patients (13%) progressed after radiotherapy.
Low Grade Glioma 2001-2013 5-year Overall Survival by Histology Log Rank p value = 0.05 Complete Censored 20 40 60 80 100 120 140 160 180 Time - months 0.4 0.5 0.6 0.7 0.8 0.9 1.0 Cumulative Proportion Surviving Grade 1 [n=46] Grade 2 [n=14]
Research Day October 2014
Of the thirteen patients treated with chemotherapy:
Estimated OS at 5 years was 100%, but PFS was only 33%. All patients were exposed to carboplatin; 5/13 or 39% developed
sustained hypomagnesaemia.
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Metastases occur more often in WHO II grade tumours. Surgery was successful in controlling Cerebellar tumours. Radiotherapy was only required for 25%, but produced excellent results. Chemotherapy provides excellent control in young patients with
irresectable tumours.
Overall Survival was better for WHO grade I tumours. Multidisciplinary team management is a viable and effective strategy for
the management of low grade gliomas in low and middle income settings.
Research Day October 2014
Steve Delport, Ariane Spitaels and Michelle Carrihill Jackie Bean Christine du Toit Michelle Meiring and Di Burger
Research Day October 2014