DSS 2012 Case 5 Mohamed El Hag Duncan

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 44 cm (>95 th percentile) • No neurological deficits

MRI: T 2 & T 1 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 ganglioma (DIA/G) • • Taratuto AL, et al. Superficial cerebral astrocytoma attached to dura. Report of six cases in infants. Cancer. 1984 Vanden. Berg SR, et al. Desmoplastic supratentorial neuroepithelial tumors of infancy with divergent differentiation potential ("desmoplastic infantile gangliomas"). 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 Vanden. Berg (87) Case #6 3 m MFs, necrosis A, NR 2. 7 yrs Duffner (94) Case #3 4 m MFs A, NR 5 yrs Al-Sarraj (96) 8 m MFs, Palisading necrosis A, NR 3 yrs Kuchelmeister (96) 9 m MFs, MVP, necrosis A, NR >2 yrs De. Munnynck (02)* 2 y MFs, MVP, necrosis DOD 1 yr Hoving (08) 7 m MFs, necrosis DWD 9 mos *multifocal at presentation, including hypothalamus

DIA/Gs with anaplastic transformation Follow-up (postoutcome GBM) Case Age @ Dx Age @ GBM Loh (2011) 2 y 5 y A, NR 11 yrs Phi (2011) 7 m 10 y 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 gangliomas”). 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 gangliomas: 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 ganglioma. Clin Neuropathol 15: 280. De Munnynck K, Van Gool S, Van Calenbergh F, Demaerel P, Uyttebroeck A, Buyse G, et al (2002) Desmoplastic infantile ganglioma: a potentially malignant tumor? Am J Surg Pathol 26: 1515– 1522. Hoving, EW, Kros, JM, Groninger E, & Den Dunnen WFA (2008) Desmoplastic infantile ganglioma 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 ganglioma. 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.