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LETTER TO EDITOR
Year : 2014  |  Volume : 62  |  Issue : 4  |  Page : 460-462

Hemangiopericytoma of pineal region: Case report and review


1 Department of Neurosurgery, National Institute of Mental Health and Neurosciences, Bangalore, Karnataka, India
2 Department of Neuropathology, National Institute of Mental Health and Neurosciences, Bangalore, Karnataka, India

Date of Web Publication19-Sep-2014

Correspondence Address:
Paritosh Pandey
Department of Neurosurgery, National Institute of Mental Health and Neurosciences, Bangalore, Karnataka
India
Paritosh Pandey
Department of Neurosurgery, National Institute of Mental Health and Neurosciences, Bangalore, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.141277

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How to cite this article:
Maiti TK, Nagarjun M N, Arimappamagan A, Mahadevan A, Pandey P, Maiti TK, Nagarjun M N, Arimappamagan A, Mahadevan A, Pandey P. Hemangiopericytoma of pineal region: Case report and review. Neurol India 2014;62:460-2

How to cite this URL:
Maiti TK, Nagarjun M N, Arimappamagan A, Mahadevan A, Pandey P, Maiti TK, Nagarjun M N, Arimappamagan A, Mahadevan A, Pandey P. Hemangiopericytoma of pineal region: Case report and review. Neurol India [serial online] 2014 [cited 2020 Oct 23];62:460-2. Available from: https://www.neurologyindia.com/text.asp?2014/62/4/460/141277


Sir,

Intracranial hemangiopericytomas (HPC) are malignant meningothelial tumors, developing from malignant transformation of pericytes. Majority of them are located in the base of the skull, in the middle or posterior fossa. The HPC in the posterior third ventricle region is very uncommon and thus this report.

A 24-year-old male presented with headache and vomiting of one-month duration. Neurologic examination was normal except for bilateral papilledema. Magnetic resonance imaging (MRI) brain showed a well-defined lesion in posterior third ventricular region, iso to hypointense on T1, hyper on T2, enhancing intensely on contrast, causing obstructive hydrocephalus [Figure 1]a-d. A ventriculoperitoneal shunt was done for hydrocephalus. Cerebrospinal fluid analysis was negative for tumor markers. Subsequently he underwent right-sided Poppen's approach and excision of lesion. The tumor was found to be attached to the tentorium and was highly vascular. Majority of the vascularity was coming from the falx and the tentorium. Following the detachment of the tentorial attachment, the tumor was excised completely. Patient had no deficits in the postoperative period, and was discharged on postoperative day-4. Histopathology revealed characteristic features of HPC with large dilated staghorn vascular channels and foci of stromal hyalinization and cystic changes intersecting the tumor cells that have elongated vesicular nuclei arranged in sheets [Figure 1]e. Mitotic activity exceeded 5/10 hpf but no necrosis was noted. Tumor cells exhibited strong immunoreactivity for CD34 [Figure 1]f A diagnosis of Grade II HPC was made. In view of high mitosis, and the diagnosis of hemangiopericytoma, he received radiotherapy of 50 Gy in 25 fractions. At 1-year follow up, he was asymptomatic and had no deficits. The MRI of the brain did not show any evidence of residual or recurrent tumor [Figure 2]a-c.
Figure 1: (a-d) MRI brain of a 24 year old man show a well defined lesion in the posterior third ventricular region, isointense on T1, hyper on T2, with uniform contrast enhancement. E, F: Histopathology revealed cellular tumor with characteristic stag horn vessels intersecting the tumor (e). Tumor cells are strongly labeled by CD34 (f). [E:H and ExObj,20, F: CD34Obj.40]

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Figure 2 (a-c): MRI Brain with contrast, Axial, Sagittal and coronal sequences revealing complete excision of tumor without any recurrence

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HPCs represent less than 1% of all intracranial tumors, and approximately 2-4% of all meningeal tumors. Despite sharing a common arachnoid location and near indistinguishable radiological features with benign meningioma, HPC is distinguished by a more aggressive biology that facilitates local recurrence and distant metastasis. [1] As recurrence is almost invariable, sometimes initial treatment within few months of, radical excision followed by adjuvant therapy, mainly radiotherapy, remains the mainstay of treatment. [2] They almost always present as solitary, supratentorial, dural-based lesions, often arising from the falx, tentorium, dural sinuses and skull base. However, pineal region location is rare with very few reported cases in literature.

Only six cases of pineal region hemangiopericytomas have been reported so far in the literature, to the best of our knowledge. [3],[4],[5],[6],[7],[8] The location coupled with tumor vascularity often make gross total resection challenging. The outcomes of previous reports reflect the same. [3],[5],[6] However, a recent report by Jian et al. suggests that these patients can be managed successfully. [7] In our case, the resection was uneventful, and patient is recurrent- free 1-year post-treatment. We preferred operating via occipital transtentorial approach because of large supratentorial involvement, and potentially larger surgical corridor through the same.

In conclusion, hemangiopericytomas rarely arise in the posterior third ventricular region. Despite, the depth of the tumor and high vascularity, they can be managed successfully with microsurgical techniques.

 
  References Top

1.Louis DN, Ohgaki H, Wiestler OD, Cavenee WK, Burger PC, Jouvet A, et al. The 2007 who classification of tumours of the central nervous system. Acta Neuropathol 2007;114:97-109.  Back to cited text no. 1
    
2.Chacko G, Chacko AG, Rajshekhar V, Muliyil JP. Intracranial hemangiopericytomas: Correlation of topoisomerase iialpha expression with biologic behavior. Surg Neurol 2006;65:11-7.  Back to cited text no. 2
    
3.Olson JR, Abell MR. Haemangiopericytoma of the pineal body. J Neurol Neurosurg Psychiatry 1969;32:445-9.  Back to cited text no. 3
    
4.Stone JL, Cybulski GR, Rhee HL, Bailey OT. Excision of a large pineal region hemangiopericytoma (angioblastic meningioma, hemangiopericytoma type). Surg Neurol 1983;19:181-9.  Back to cited text no. 4
    
5.Lesoin F, Bouchez B, Krivosic I, Delandsheer JM, Jomin M. Hemangiopericytic meningioma of the pineal region. Case report. Eur Neurol 1984;23:274-7.  Back to cited text no. 5
    
6.Sell JJ, Hart BL, Rael JR. Hemangiopericytoma: A rare pineal mass. Neuroradiology 1996;38:782-4.  Back to cited text no. 6
    
7.Jian BJ, Han SJ, Yang I, Waldron JS, Tihan T, Parsa AT. Surgical resection and adjuvant radiotherapy for a large pineal hemangiopericytoma. J Clin Neurosci 2010;17:1209-11.  Back to cited text no. 7
    
8.Hasturk AE, Basmaci M, Bozdogan N, Canbay S. Hemangiopericytoma of the pineal region. Neurosciences (Riyadh) 2011;16:159-61.  Back to cited text no. 8
    


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