• History and Examination 
     
    History
    22 yo female, presented with history of chronic headache and pregressive loss of vision for 3 months
    History of recent seizures within 1 week before hospital admission.
    Systemic review, past medical, past surgical, and drug history are unremarkable
     
    Exam
     Significant decreased vision [1/6], otherwise no neurological deficit.
     

    Figure 1

    Figure 2

    Figure 3

    1. Where does the lesion originate from?

    2. What is the most likely diagnosis?

    3 .What is the best surgical approach?

    4. What is the further post operative management?

    5. Which of the following describes you?

    6. I practice in one of the following locations.

    7. Comments
     
    • I think the tumor is intraventricular, in the right frontal cavity, causing obstruction, and intracranal hipertension
    • Please I want feedback from self assessment
    • May be chorroid plexus papiloma
    • Quite educative.  I highly commend the CNS for these monthly Case of the month quiz.
    • 1-Positivity to neuronal markers such as:

      synaptophysin, neuronal specific enolase should be examined.
      2-Adjuvant therapy mainly radiotherapy (chemotherapy might be added). 
    • I have recently treated a similar case (40y/o Male) which was reported as a gemicytositic astrocytoma. The tumor had rim like peripheral calcification on CT scan. SEGA is my first choice here. 
    • Subependymoma as second choice          
    • Good case
    • I think it is chorroid plexus papiloma 
    • A TAG LINE: Neuro-oncological emergency could be used! 
    • It is great to share and discuss cases, and in the same time, increase your knowledge. Thank you very much!
    • Post-operative treatment depends on pathology. With working diagnosis of central neurocytoma no other treatment needed.
    • Submitted by:
    • 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

       

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