DSS 2012 Case 5
Mohamed El Hag
Duncan Stearns
Michael Coffey
Mark Cohen
Clinical History
• 3 month old girl with a bump on the side of
her head
• Previously healthy, normal prenatal
ultrasounds
• Head circumference 44cm (>95th
percentile)
• No neurological deficits
MRI: T2 & T1 with contrast
Dural-based,
well-demarcated tumor
Biphasic morphology
Question for discussion:
Prognosis?
(diagnosis already provided by
radiologist)
Disclosure
Unfortunately, the authors have
nothing to disclose, including the
answer to their question.
GFAP
SYN
NF
Desmoplastic infantile astrocytoma and
ganglioglioma (DIA/G)
•
•
Taratuto AL, et al. Superficial
cerebral astrocytoma attached
to dura. Report of six cases in
infants. Cancer. 1984
VandenBerg SR, et al.
Desmoplastic supratentorial
neuroepithelial tumors of
infancy with divergent
differentiation potential
("desmoplastic infantile
gangliogliomas"). Report on 11
cases of a distinctive
embryonal tumor with favorable
prognosis. J Neurosurg. 1987
• Presentation in
infancy
• Macrocrania
• Voluminous size
• Cystic component
• Divergent
differentiation
• Desmoplastic stroma
• Good prognosis
D(S)IA/Gs with anaplastic features
Case
Age
Features
Outcome
Follow-up
VandenBerg (87)
Case #6
3m
MFs, necrosis
A, NR
2.7 yrs
Duffner (94)
Case #3
4m
MFs
A, NR
5 yrs
Al-Sarraj (96)
8m
MFs, Palisading
necrosis
A, NR
3 yrs
Kuchelmeister (96)
9m
MFs, MVP,
necrosis
A, NR
>2 yrs
DeMunnynck (02)*
2y
MFs, MVP,
necrosis
DOD
1 yr
Hoving (08)
7m
MFs, necrosis
DWD
9 mos
*multifocal at presentation, including hypothalamus
DIA/Gs with anaplastic transformation
Case
Age @
Dx
Age @
GBM
outcome
Follow-up
(postGBM)
Loh
(2011)
2y
5y
A, NR
11 yrs
Phi
(2011)
7m
10y
AWD
14 mos
Clinical follow-up (6 months)
• s/p complete resection (required three
craniotomies)
• Post-op ischemic changes with left sided
weakness
• Chemotherapy w/o radiation therapy
• No evidence of residual or recurrent tumor
Taratuto AL, Monges J, Lylyk P, et al (1984) Superficial cerebral astrocytoma attached to dura: report
of six cases in infants. Cancer 54:2505–12.
Vandenberg SR, May EE, Rubinstein LJ, Herman MM, Perentes E, Vinores SA, Collins P, Park TS
(1987) Desmo- plastic supratentorial neuroepithelial tumors of infancy with divergent
differentiation potential (“desmoplastic infantile gangliogliomas”). Report of 11 cases of a
distinctive embryonal tumor with favorable prognosis. J Neurosurg 66:58–71.
Duffner PK, Burger PC, Cohen ME, Sanford RA, Krischer JP, Elterman R, Aronin PA, Pullen J,
Horowitz ME, Parent A, Martin P, Kun L (1994) Desmoplastic infantile gangliogliomas: an
approach to therapy. Neurosurgery 34:583–589.
Al-Sarraj ST, Bridges LR (1996) Desmoplastic cerebral glioblastoma of infancy. British Journal of
Neuropathology 10(2):215-219.
Kuchelmeister K, Steinhauser A, Korf B, et al (1996) Anaplastic desmoplastic infantile ganglioglioma.
Clin Neuropathol 15:280.
De Munnynck K, Van Gool S, Van Calenbergh F, Demaerel P, Uyttebroeck A, Buyse G, et al (2002)
Desmoplastic infantile ganglioglioma: a potentially malignant tumor? Am J Surg Pathol 26:
1515–1522.
Hoving, EW, Kros, JM, Groninger E, & Den Dunnen WFA (2008) Desmoplastic infantile ganglioglioma
with a malignant course. J neurosurg Pediatrics 1:95-98.
Loh J-K, Lieu A-S, Chai C-Y, Howng S-L (2011) Malignant transformation of desmoplastic infantile
ganglioglioma. Pediatric Neurology 45:135-137.
Phi JH, Koh EJ, Kim S-K, Park S-H, Cho B-K, & Wang K-C (2011) Desmoplastic infantile astrocytoma:
recurrence with malignant transformation into glioblastoma: a case report. Childs Nerv Syst
27:2177-2181.