Table 1 Clinical and radiographic features of neuroepithelial tumors with PLAG-family genetic alterations

#1

9 mo

F

EWSR1-PLAGL1 fusion

8.9 cm left frontal mass

NTR, GTR, GTR

carboplatin/etoposide

Residual/recurrent disease by imaging at 3 months with additional resection at 6 months, additional recurrence/resection at 1.2 years for local rapid growth, alive with no evidence of disease by imaging at 1.5 years

1.5 yrs

#2

11 mo

M

EWSR1-PLAGL1 fusion

6 cm right frontal mass

GTR

ACNS0334,

then modified MEMMAT,

followed by tazometostat

Recurrence/resection at 7 months with intracranial multifocal disease,

died of disease progression

at 11 months

11 months

#3

2 yrs

F

EWSR1-PLAGL1 fusion

9.1 cm right occipital mass

NTR, GTR,

GTR

SJYC07 chemotherapy,

continued observation

Recurrence/resection at 1.6 years,

additional recurrence/resection

at 9 years, stable nodularity at resection cavity by imaging at 10.3 years

10.3 yrs

#4

12 yrs

F

EWSR1-PLAGL1 fusion

6.6 cm right occipital mass

NTR, GTR

focal proton beam 54 CGE, chemotherapy per ACNS0831 maintenance

Additional resection at 2 months for minimal residual neoplasm, alive with no evidence of disease by imaging at 2 years

2 yrs

#5

14 yrs

M

EWSR1-PLAGL1 fusion

8 cm right frontal mass, multicystic

Not available

Not available

Not available

Not available

#6

11 yrs

F

EWSR1-PLAGL1 fusion

6.6 cm left frontal mass

GTR

Focal radiation 54 Gy

Alive with no evidence of disease at 13 months, treatment related imaging changes

13 months

#7

2 yrs

F

PLAGL2 amplification

5.3 cm right cerebellar mass

GTR

Chemotherapy,

autologous bone marrow transplant

Alive with no evidence of disease

by imaging at 4.8 years

4.8 yrs

#8

4 yrs

M

PLAGL1 amplification

7.2 cm left temporal mass

STR, STR, NTR

CSI (23.4CGE) followed by focal boost (54CGE),

SJMB12 N1 chemotherapy

Minimal residual disease at 2 months,

no definitive disease by imaging

at 7 months

7 months