Table 2.

Clinical Presentation and Outcome of the Study Subjects.

Surgery at diagnosis	Extent of resection	Age at diagnosis in years	Tumor location	Histology	BRAF alteration	Neurologic deficits at presentation	Hydrocephalus/ VP shunt	Adjuvant therapy	Progression/ PFS	Salvage therapy	Disease outcome	Follow-up, (in years)	Sequelae	Clinical outcome (compared with status at diagnosis)
Yes, n = 22	GTR = 4	6	Cervicomedullary, DE	Ganglioglioma	Inadequate	- R hemiparesis - Ataxia	No/no	None	No	–	NED	11	Right hemiparesis	No change
		16	Pontomedullary, DE	PA	NA	- Ataxia	Yes/yes	None	No	–	NED	3	-Tracheostomy - Multiple cranial nerve palsy - Ataxia - G tube dependent	Worse
		5	Pontomedullary, DE	PA	Negative	- Dysphagia - FTT	Yes/no	None	No	–	NED	9	None	Better
		1.8	Medullary, DE	PA	Negative	- Abnormal tongue movements -FTT	Yes/no	None	No	–	NED	12	Hemiatrophy and fasciculation of tongue	Better
	NTR = 6	5	Pontine, focal	PA	NA	- Right sensorineural hearing loss	No/no	None	Yes/3 mo	CT VCR/carboplatin	NED	2.5	- U/L deafness - Right facial nerve paresis	Worse
		1.6	Cervicomedullary, DE	PA	Inadequate	- Dysphagia -FTT - Quadriparesis	Yes/no	None	Yes/4 mo	Resection + CT VCR/carboplatin	NED	18	Central sleep apnea	Better
		0.8	Cervicomedullary, DE	PA	Inadequate	- Torticollis - R sixth nerve palsy -RUE monoparesis	No/no	None	No	–	SD	10	None	Better
		6	Pontomedullary, DE	PA	Inadequate	- L sixth nerve palsy - L hearing loss - L seventh nerve palsy - L hemiparesis	Yes/no	None	No	–	SD	12	- L seventh nerve palsy - L sensorineural hearing loss - L hemiparesis	No change
		5.5	Pontomedullary, DE	Ganglioglioma	KIAA1549- BRAF	- Dysphagia	No/no	None	No	–	SD	6	None	Better
		2	Midbrain tegmentum	PA	KIAA 1549- BRAF	- L hemiparesis	No/no	None	Yes/4 mo	CT VCR/carboplatin	NED	1.6	None	Better
	STR = 7	2	Midbrain tegmentum, LE	PA	NA	- Dysphagia - Diplopia - Ataxia - FTT	Yes/yes	None	Yes/15 mo	CT TPCV	SD	10.5	- Dysphagia - Left hemiparesis	Worse
	STR = 7	16	Cervicomedullary, DE	PA	Inadequate	- None	No/no	None	No	–	SD	2	None	No change
		11	Pontine, focal DE	PA	Negative	- None	Yes/no	None	Yes/16 y	Resection +RT	SD	17	None	No change
		4.25	Cervicomedullary, DE	PA	NA	- Apnea - Ataxia - Dysphagia - Right hemiparesis	No/no	None	Yes/6 mo	CT VCR/carboplatin	SD	14.5	- Sleep apnea - Bulbar dysfunction - Right hemiparesis - Tracheostomy - G tube dependent	Worse
		16	Pontomedullary, DE	PA	Inadequate	None	Yes/no	RT	No	–	SD	15	Mild ataxia	Worse
		5	Pontomedullary, LE	PA	KIAA 1549- BRAF	- None	Yes/no	CT TPCV	No	–	NED	2	None	No change
		8	Cervicomedullary, DE	Mixed OA	NA	- Obstructive sleep apnea - Stridor	No/no	RT	No	–	SD	8	Obstructive sleep apnea	No change
	PR = 2	14	Pontine, focal	Grade II Astro	Inadequate	- R seventh nerve palsy - B/L abductor palsy	No/no	CT TMZ	No	–	SD	7	- R seventh nerve palsy - B/L abductor palsy	No change
		0.3	Pontomedullary, DE	Mixed OA	NA	- Dysphagia - FTT	Yes/yes	CT VCR/carboplatin	Yes/3 y	RT	SD	13	- Central sleep apnea - T racheostomy	Worse
	Biopsy = 3	11	Midbrain tegmentum, DE	Grade II Astro	NA	- Dysphagia - B/L ptosis - Ataxia - Dysarthria	Yes/no	RT+CT TPCV	No	–	SD	6	None	Better
		13	Midbrain tegmentum	PA	KIAA 1549- BRAF	- Ataxia - Right hemiparesis	Yes/yes	RT	No	–	SD	13	Right hemiparesis	Better
		8	Midbrain	Grade II Astro	NA	-Left hemiparesis	No/no	RT	No	–	SD	4	None	Better
None, n = 3		11	Medullary, DE	NA		- None	No/no	None	No	–	SD	2	None	No change
		14	Midbrain tegmentum	PA	Inadequate	-Left hemiparesis - Right third nerve paresis	Yes/yes	RT+ 1 cycle CT VCR/carboplatin	Yes/3 mo after RT, during CT	Resection after failure of RT and CT	NED	5.5	- Left hemiparesis - Right third nerve paresis	No change
		7	Medullary, DE	NA		- Hyponasal speech	Yes/yes	RT	No	–	SD	15	Hyponasal speech	No change

Abbreviations: CCNU, lomustine; CT, chemotherapy; DE, dorsally exophytic; FTT, failure to thrive; GTR, gross total resection; LE, laterally exophytic; NA, not available; NED, no evidence of disease; NTR, near total resection; OA, oligoastrocytoma; PA, pilocytic astrocytoma; PFS, progression-free survival; PR, partial resection; RT, radiotherapy; SD, stable disease; STR, subtotal resection; TMZ, temozolomide; TPCV, thioguanine, procarbazine, lomustine, vincristine