Clinicopathological features and treatment outcomes of brain stem gliomas in Saudi population

Table 1 Clinicopathological characteristics of cohort

Mean age at diagnosis (yr)	12.62 (0.8-64) SD ± 13.42	Diffuse astrocytoma grade II	4/30 (13.3%)
Gender		Anaplastic astrocytoma	10/30 (33.3%)
Male	31 (63.3%)	Glioblastoma multiforme	4/30 (13.3%)
Female	18 (36.7%)	Astroblastoma	1/30 (3.3%)
According to age		Nonspecified glioma	5/30 (16.7%)
Children and adolescents	40 (81.6%)	No	19 (38.8%)
Adults	9 (18.4%)	Radiological diagnosis
Duration of symptoms (d)	83.4, SD ± 47.5	Focal	12 (24.5%)
Karnofsky Performance Status	80 (50-100)	Tectal	3 (6.1%)
Symptoms at time of presentation		Dorsally exophytic	6 (12.2%)
Headache	21 (42.8%)	Cervicomedullary	3 (6.1%)
Vomiting	11 (22.4%)	DIPG	22 (44.9%)
Diplopia/ squint	19 (38.8%)	Others	3 (6.1%)
Unsteady gait	17 (34.7%)	Surgery	30/49 (61.2%)
Difficulty in swallowing or choking	13 (26.5%)	Total/maximal resection	14/30 (36.7%)
Motor weakness/ paresis	10 (20.4%)	Subtotal resection	14/30 (36.7%)
Convulsions	4 (8.2%)	Biopsy only	2/30 (6.1%)
Dysphonia/ dysarthria	11 (22.4%)	VP shunt	19/30 (63.3%)
Altered consciousness	5 (10.2%)	ETV	4/30 (13.3%)
Isolated facial paresis	6 (12.2%)	EDV	5/30 (16.7%)
Hearing problems	3 (6.1%)	IONP	14/30 (36.7%)
Fever	2 (4.1%)	Radiation therapy	32/49 (65.3%)
Failure to thrive	1 (2.0%)	Postoperative	14/32 (43.7%)
Neurological signs at time of presentation		Radiotherapy alone	18/32 (56.3%)
Mental status change	8 (16.3%)	Total dose (Gy)	50.4-59.4
Cranial nerve palsies	32 (65.3%)	Fractions	30-33
Trigeminal	2 (6.3%)	Duration (wk)	6-6.5
Abducens	18 (56.3%)	Technique
Facial	12 (37.5%)	3DCRT	15 (46.9%)
Vestibulocochlear	2 (6.3%)	IMRT	17 (53.1%)
Glossopharyngeal	12 (37.5%)	Chemotherapy	23/49 (46.9%)
Vagus	8 (25.0%)	Concurrent	12/23 (52.2%)
Motor deficit	14 (28.6%)	TMZ	12/12 (100%)
Sensory deficit	6 (12.2%)	Neoadjuvant	7/23 (30.4%)
Bilateral Babinski sign	13 (26.5%)	Vincristine + carboplatin	4/7 (51.1%)
Cerebellar signs	25 (51.0%)	High dose chemotherapy with stem cell rescue	2/7 (28.7%)
Nystagmus	17 (34.7%)	Cyclophosphamide	1/7 (14.3%)
Bilateral papilledema	19 (38.8%)	Adjuvant/ salvage	11/23 (47.8%)
Pathological diagnosis		BCNU + procarbazine + vincristine	5/11(45.7%)
Yes	30 (61.2%)	Vincristine + carboplatin	4/11 (36.2%)
Pilocytic astrocytoma	6/30 (20.0%)	Irinotecan + bevacizumab	2/11 (18.1%)

DIPG: Diffuse intrinsic pontine glioma; VP shunt: Ventriculoperitoneal; ETV: Endoscopic third ventriculostomy; EDV: Endoscopic ventricular drain; IONP: Intra-operative neurophysiology; 3DCRT: Three dimensional conformal radiation therapy; IMRT: Intensity modulated radiation therapy; TMZ: Temozolomide; BCNU: 1,2-bis (2-chloroethyl) 1-nitrosourea.

Table 2 Magnetic resonance imaging characteristics in our cohort of brain stem glioma n (%)

Subgroups	Enhancement enhancing	Non-enhancing	T2W image hyper	Intensity mixed	Character
					Cystic	Solid	Mixed
Focal (12)	17 (100)	-	10 (83.3)	2 (16.7)	3 (25)	6 (50)	3 (25)
Focal tectal (3)	2 (66.7)	1 (33.3)	1 (33.3)	2 (66.7)	3 (100)
Dorsally exophytic (6)	4 (66.7)	2 (33.3)	3 (50.0)	3 (50.0)		4 (667)	2 (33.3)
Cervicomedullary (3)	2 (66.7)	1 (33.3)	-	3 (100)	3 (100)
DIPG (22)	10 (45.5)	12 (54.5)	10(45.4%)	12 (54.5)		19 (86.4)	3 (13.6)

DIPG: Diffuse intrinsic pontine glioma.

Table 3 Surgical resection in our cohort of brain stem glioma n (%)

Subgroups	Resection		VP shunt	ETV	EVD	IONP
	Complete	Incomplete/biopsy
Focal (12)	6 (50.0)	6 (50.0)	3 (25.0)	1 (8.3)	2 (16.6)	5 (41.7)
Focal tectal (3)	2 (66.7)	1 (33.3)	2 (66.7)	1 (33.3)	1 (33.3)	2 (66.7)
Dorsally exophytic (6)	5 (83.3)	1 (16.7)	3 (50.0)	-	2 (33.3)	5 (83.3)
Cervicomedullary (3)	1(33.3)	2 (66.7)	-	-	-	2 (33.3)
DIPG (22)	-	6 (27.3)	11 (50)	2 (9.0)	-	1 (4.5)

VP shunt: Ventriculoperitoneal; ETV: Endoscopic third ventriculostomy; EDV: Endoscopic ventricular drain; IONP: Intra-operative neurophysiology.

Table 4 Radiation therapy in our cohort of brain stem glioma n (%)

Subgroups	Indication		Technique		Total dose (Gy)
	Postoperative	Radical	3DCRT	IMRT
Focal (12)	6 (50)	-	3 (50)	3 (50)	50.4-54
Focal tectal (3)	1 (33.3)	-	-	1 (100)	54
Dorsally exophytic (6)	1 (16.7)	-	1 (100)	-	54
Cervicomedullary (3)	2 (66.7)	-	2 (100)	-	50.4-54
DIPG (22)	6 (27.3)	16(72.7)	9 (40.9)	13 (59.1)	54-59.4

3DCRT: Three dimensional conformal radiation therapy; IMRT: Intensity modulated radiation therapy; Gy: Gray.

Table 5 Chemotherapy in our cohort of brain stem glioma n (%)

Subgroups	Neoadjuvant	Concurrent	Adjuvant/salvage
Focal (12)		-	3 (25.0)
Focal tectal (3)	-	-	-
Dorsally exophytic (6)	-	-	-
Cervicomedullary (3)	1 (33.3)	-	2 (66.6)
DIPG (22)	6 (27.3)	12 (54.5)	6 (27.3)

DIPG: Diffuse intrinsic pontine glioma.

Table 6 Multivariate analysis of various prognostic factors in brainstem glioma

Variables	RR (95%CI)	P value
Age at diagnosis (> 18 yr)	3.0 (1.8-6.0)	0.01
KPS < 80	3.3 (1.7–5.3)	0.02
Duration of symptoms (< 60 d)	6.7 (4.3-9.4)	0.002
Histopathology (high grade)	6.1 (3.5-10.2)	0.002
MRI characteristics (presence of necrosis)	3.0 (1.9-5.9)	0.01
Incomplete resection for favorable tumors	6.6 (3.9-12.2)	0.002
No concurrent chemotherapy with RT	3.1 (2.2- 8.2)	0.01