Table 1.
Case Comparison.
| Our Patient | Armao | Gardiman 1 | Gardiman 2 | Gardiman 3 | Gardiman 4 | Schniederjan 1 | Schniederjan 2 | Schniederjan 3 | Schniederjan 4 | Schniederjan 5 | Schniederjan 6 | Schniederjan 7 | Schniederjan 8 | Schniederjan 9 | |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Age/sex | 14 yo/F | 8 yo/M | 4 yo/M | 3 yo/F | 3 yo/F | 13 yo/M | 2.5 yo/M | 3 yo/M | 1.5 yo/M | 4 yo/F | 2 yo/F | 5 yo/F | 7 yo/F | 4 yo/F | 6 yo/M |
| Hydrocephalus | + | + | + | + | + | + | + | + | + | + | + | − | − | + | + |
| Bilateral CN 6 palsy | + | − | − | + | − | − | +a | ||||||||
| CSF with elevated protein and no malignant cells | + | + | |||||||||||||
| IP/IM tumor | − | − | − | − | − | − | − | +b | − | +c | − | +d | − | − | − |
| Meningeal nodules | +e | + | − | +e | +e | +f | − | − | |||||||
| Diffuse LM enhancement | + | + | + | + | + | + | + | + | + | + | + | + | + | + | + |
| Intraventricular involvement | +g | − | − | +h | − | + | |||||||||
| Thalamic calcifications | − | − | − | − | − | + | |||||||||
| Sella turcica enlargement | − | − | + | − | − | +i | |||||||||
| Coating of optic chiasm | + | + | |||||||||||||
| IP cystsj | + | + | + | + | + | + | + | + | + | + | + | + | |||
| Neuronal and glial markers on same cell | + | +k | + | + | + | + | − | − | − | + | + | − | − | + | + |
| OLIG-2 | + | ||||||||||||||
| p53 | +l | ||||||||||||||
| MIB-1/KI-67 elevated | − | − | − | <3%m | − | − | + | − | − | − | − | + | − | ||
| High mitotic index | + | − | − | + | +/−m | ||||||||||
| 1p/16q deletions | − | − | +/−, +/+n | − | −/− | +/− | +/+ | +/− | −/− | ?/?o | +/?p | +/− | +/+ | ||
| Diagnosis given | DLGNT | DLOq | DLGNT | DLGNT | DLGNT | DLGNT/DLOr | DLNET | DLNET | DLNET | DLNET | DLNET | DLNET | DLNET | DLNET | DLNET |
Abbreviations: LM, leptomeningeal; IP, intraparenchymal; DLGNT, diffuse leptomeningeal glioneuronal tumor; DLO, diffuse leptomeningeal oligodendrogliomatosis; DLNET, diffuse leptomeningeal neuroepithelial tumor; GFAP, glial fibrillary acid protein; CSF, cerebral spinal fluid; FLAIR, fluid-attenuated inversion recovery; yo, years old; F, female; M, male.
aReported as “diplopia.”
bIntramedullary lesion at T8.
cIntramedullary cystic and solid mass at C6 to C7.
dCerebellar lesion.
eCervical meningeal nodules.
fSuprasellar meningeal nodules.
g1 cm mass in left cavernous sinus.
hLeft frontal intraventricular lesion with perivascular pseudorosettes versus pseudopapillary structure.
iInternal auditory canals.
jOur patient’s cysts were nonenhancing; Armao patient’s cyst enhanced like CSF; all 4 Gardiman patients’ cysts were hyperintense on T2 and isohypointense on T1 and FLAIR.
kGFAP negative, Leu-7 positive; Leu-7 is associated with myelin and is present in a majority of oligodendrogliomas.
lFocally positive.
mBiopsy at initial presentation high mitotic index; biopsy at second presentation low mitotic index and <3% MIB-1.
nFirst biopsy—<1% MIB-1 and 1p deletion and second biopsy—50% MIB-1 and 1p/16q codeletion.
oTissue was not available for fluorescence in situ hybridization testing.
pPoor tissue adherence on chromosome 19 fluorescence in situ hybridization testing.
qDLO usually has a primary intra-axial mass—this case did not.
rGardiman diagnosed DLGNT and Rossi diagnosed DLO.