• History

    An 18 year old male is sent to the emergency room by his ophthalmologist who was working up his progressive visual loss.  The loss has been over the past 3 months and his workup included an MRI of the brain.  The patient also endorses headaches for the same time period.  
    The visual loss is described as a blurriness in the left eye only.  

    A&Ox3, NAD
    Some loss of acuity grossly in the left visual field
    Remainder of cranial nerves II-XII are intact
    Full strength throughout


    Fig 1

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    Fig 5

    1. What is the most common tumor in this location?
    2. Biopsy was found to be hemangiopericytoma. Which of the following would most likely be present on pathology?
    3. What is the standard treatment of choice for these lesions?
    4. Which of the following describes you?
    5. I practice in one of the following locations.

    Case Explanation 

    Although the optimal surgical approach has not been established contemporary management of pineal region tumors includes: endoscopic biopsy, cerebrospinal fluid (CSF) sampling and possible CSF diversion.  In this patient an endoscopic 3rd ventriculostomy (ETV) and tumor biopsy were attempted, but could not be performed due to inadequate tuber cinereum space, and tumor vascularity (demonstrated on preoperative angiography). 
    Question 1: The most common tumors in this area are germinomas followed by astrocytomas, pineocytoma and other germ-cell tumors.  Following negative CSF tumor marker analysis and cytology, open biopsy (posterior interhemispheric approach) revealed a hemangiopericytoma (HPC).  
    Question 2: Microscopically HPCs’ demonstrate a staghorn vascular pattern.  They are also positive for vimentin (negative EMA and S-100).  In our case, this was confirmed by methylation array analysis.  Necrosis and neovascularization would be suggestive of a high grade tumor, typically a glioblastoma.  Verocay bodies are seen in schwannomas.  Fried egg pattern is typically seen in an oligodendroglioma.  
    Question 3: The standard treatment for these tumors is gross total resection followed with adjuvant radiotherapy if needed. The patient subsequently underwent staged endovascular embolization followed by gross total resection. Both the biopsy and resection were performed through a posterior interhemispheric approach.  Final resection required a transtentorial modification to remove posterior fossa and tentorial tumor.  


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    • Veeravagu A, Jiang B, Patil CG, et al. CyberKnife stereotactic radiosurgery for recurrent, metastatic, and residual hemangiopericytomas. J Hematol Oncol 2011;4:26.
    • Ahn ES, Goumnerova L.  Endoscopic biopsy of brain tumors in children: diagnostic success and utility in guiding treatment strategies.  J Neurosurg Pediatrics. 2010 Mar; 5 (3): 255-62
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    • Pineal region tumors. In: Greenberg M, Hrsg. Handbook of Neurosurgery. 8th Edition. Thieme; 2016.
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    • Regis J, Bouillot P, Rouby-Volot F, et al. Pineal Region Tumors and the Role of Stereotactic Biopsy: Review of the Mortality, Morbidity, and Diagnostic Rates in 370 Cases. Neurosurgery. 1996;39:907-914.
    • Rutkowski MJ, Sughrue ME, Kane AJ, et al. Predictors of mortality following treatment of intracranial hemangiopericytoma. J Neurosurg 2010;113(2):333–339
    • Chamberlain MC, Glantz MJ. Sequential salvage chemotherapy for recurrent intracranial hemangiopericytoma. Neurosurgery 2008;63(4):720–726, author reply 726–727

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