Dr. Walid Elgaddafi, Benghazi, Libya
Case Explanation:
PMA is currently understood to be a pediatric brain tumor with a distinctive monomorphous pilomyxoid histological pattern ,a more aggressive behavior than classical PA and is typically located in the hypothalamic/chiasmatic region. On imaging, PMA is typically well circumscribed without surrounding edema, consisting of solid with some cystic components and enhances homogeneously with contrast. Although PMA is a WHO grade II low-grade astrocytoma, it appears to follow a more benign clinical course in the three adult patients reported thus far, in addition to our case.
Intra-operatively, the tumor was totally intra-ventricular, and gross total resection was done, through right transcortical approach (middle frontal gyrus). With a histopathology report confirming the diagnosis of pilomyxoid astrocytoma.
References:
Jones, D. T.; Kocialkowski, S; Liu, L; Pearson, D. M.; Bäcklund, L. M.; Ichimura, K; Collins, V. P. (2008). "Tandem duplication producing a novel oncogenic BRAF fusion gene defines the majority of pilocytic astrocytomas". Cancer Research 68 (21): 8673–7. doi:10.1158/0008-5472.CAN-08-2097. PMC
Komotar RJ, Mocco J, Zacharia BE, Wilson DA, Kim PY, Canoll PD, et al. Astrocytoma with Pilomyxoid features presenting in an adult. Neuropathology 2006;26:89-93.
Wippold FJ, Perry A, Lennerz J (May 2006). "Neuropathology for the neuroradiologist: Rosenthal fibers". AJNR Am J Neuroradiol 27 (5): 958–61. PMID 16687524.
Fuller C, Narendra S. Pilocytic astrocytoma and pilomyxoid astrocytoma. In: Adesina AM, Tihan T, Fuller CE, Poussaint TY, editors. Atlas of Pediatric Brain Tumors. New York: Springer; p. 5-8.
Abosch A, McDermott WM, Wilson CB. Lateral ventricular tumors. In: Kaye AH and Black PMcL , eds. Operative Neurosurgery. London-New York –Sydney:
Churchill Livingstone 2000, pp. 799-814
Apuzzo ML, Chikoyani OK, Gott PS, Teng EL, Zee CS, Giannotta SL, Weiss MH. Transcallosal, interfornicial approaches for lesions affecting the third ventricle: surgical considerations and consequences.
Neurosurgery 10:547-554, 1982
Social Media:
Continue the discussion on LinkedIn with our members-only
Case of the Month Discussion Group. Create a dialogue and see how your recommendations compare with your colleagues across the globe.