DSS 2012 Case 5

Mohamed El HagDuncan StearnsMichael 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) 8mMFs, Palisading

necrosisA, NR 3 yrs

Kuchelmeister (96) 9mMFs, MVP, necrosis

A, NR >2 yrs

DeMunnynck (02)* 2yMFs, MVP, necrosis

DOD 1 yr

Hoving (08) 7m MFs, necrosis DWD 9 mos

*multifocal at presentation, including hypothalamus

DIA/Gs with anaplastic transformation

CaseAge @

DxAge @ GBM

outcome

Follow-up

(post-GBM)

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.