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DSS 2012 Case 5

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 (>95 th percentile) No neurological deficits.

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DSS 2012 Case 5

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  1. DSS 2012 Case 5 Mohamed El Hag Duncan Stearns Michael Coffey Mark Cohen

  2. 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

  3. MRI: T2 & T1 with contrast

  4. Dural-based,well-demarcated tumor

  5. Biphasic morphology

  6. Question for discussion: Prognosis? (diagnosis already provided by radiologist)

  7. Disclosure Unfortunately, the authors have nothing to disclose, including the answer to their question.

  8. GFAP SYN NF

  9. 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 Desmoplastic infantile astrocytoma and ganglioglioma (DIA/G)

  10. D(S)IA/Gs with anaplastic features *multifocal at presentation, including hypothalamus

  11. DIA/Gs with anaplastic transformation

  12. 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

  13. 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.

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