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Table of Contents   
LETTER TO THE EDITOR  
Year : 2015  |  Volume : 8  |  Issue : 4  |  Page : 138-140
Primary gliosarcoma: A case report


Department of Pathology, Swami Ramanand Teerth Rural Government Medical College, Ambajogai, Maharashtra, India

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Date of Web Publication7-Aug-2015
 

How to cite this article:
Swami SY, Shendarkar AD, D'Costa G. Primary gliosarcoma: A case report. Ann Trop Med Public Health 2015;8:138-40

How to cite this URL:
Swami SY, Shendarkar AD, D'Costa G. Primary gliosarcoma: A case report. Ann Trop Med Public Health [serial online] 2015 [cited 2020 Feb 18];8:138-40. Available from: http://www.atmph.org/text.asp?2015/8/4/138/162396
Dear Sir,

Gliosarcoma (GS) was described for the first time in 1895 by Stroebe. [1] GS is a very rare primary mixed tumor in the central nervous system (CNS), with a biphasic pattern consisting of glial and malignant mesenchymal elements. Its onset is between the fourth and sixth decade of life, and it has a male:female ratio of 1.8:1. [2] The 2007 World Health Organization (WHO) classification scheme places primary GS (PGS) as a grade IV neoplasm and a variant of glioblastoma multiforme (GBM). [3]

We have reported a case of PGS. Immunohistochemical (IHC) analysis was performed to confirm the diagnosis and assess histogenetic origin.

A 60-year-old female, normotensive, nondiabetic individual presented with weakness of the right upper and lower limbs, altered consciousness, and a history of headache and vomiting since the past 4 months. Computed tomography (CT) brain revealed a mildly enhancing heterogenous intra-axial space-occupying lesion in the left frontotemperoparietal lobe with perilesional edema and mass effect suggestive of an aggressive lesion, mainly glioma. Distant metastasis was not found. The patient was then referred to a neurosurgeon.

The surgical specimen was received in the form of multiple grey-white pieces aggregating 4 × 3 cm. Microscopy [Figure 1] revealed tumor tissue comprising two cell types. Loose cellular areas of glial cells with pleomorphic, vesicular nuclei and prominent nucleoli admixed with occasional mitosis, increased vascularity, and pericytic hyperplasia. The other cell type [Figure 2] was densely packed spindle cells with pleomorphic, hyperchromatic nuclei, scanty cytoplasm arranged in fascicles, and perithelial arrangement. The stroma was scantily admixed with inflammatory cells. There were areas of necrosis and hemorrhage.
Figure 1: Cellular areas of glial cells with pleomorphic, vesicular nuclei and prominent nucleoli admixed with occasional mitosis [hematoxylin and eosin (H&E); 40×]

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Figure 2: Spindle cells with pleomorphic hyperchromatic nuclei, scanty cytoplasm arranged in fascicles, and perithelial arrangement (H&E; 10×)

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IHC revealed [Figure 3] tu mor cells positive for glial fibrillary acidic protein (GFAP) and vimentin [Figure 4], and negative for desmin [Figure 5] and smooth muscle actin (SMA) [Figure 6]. GFAP positivity was because of the glial tissue and vimentin positivity was because of the mesenchymal component, thus confirming the biphasic nature of the tumor.
Figure 3: IHC: positive for GFAP (10×)

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Figure 4: IHC: positive for vimentin (40×)

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Figure 5: IHC: negative for desmin (20×)

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Figure 6: IHC: negative for SMA (20×)

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   Discussion Top


GSs are rare and have an incidence of 1.8-2.8% that of GBMs. [4] Similar to other glial-based tumors; PGS affects adults between the sixth and seventh decade of life with the M: F ratio 1.4-1.8:1. [4],[5]

The clinical similarities of PGS to GBM have led many authors to conclude that these tumors are clinically indistinguishable. However, there are a number of important and distinct features of PGS that suggest that it is a separate entity. The striking features of PGS that distinguish it from GBM include its location and its differential radiographic and gross appearance. GS is almost never found infratentorially, and the majority of the reports describe its temporal lobe predilection, [4],[5],[6] while a few report a higher incidence in the frontal lobe. [5],[7]

The clinical characteristics of our cases with respect to age, sex, site, and symptoms are comparable to other studies.

Extracranial metastases from the cerebral glioma, including GBM, are very rare, while the propensity for a GS to metastasize is well established. Even in the early days of Feigin, several authors reported cases of metastatic foci that contained admixtures of both gliomatous and sarcomatous elements. [8],[9] The presence of these metastases was a large contribution to the premise that a PGS is a clinically separate entity from a GBM and truly biphasic in nature.


   Conclusion Top


PGS represents a group of tumors due to their rare presentation, poor prognosis, and the limited literature on them. Primary sarcomas of the CNS are extraordinarily rare. Metastatic sarcomas are more common, although rare. In either case, there is no double glial and sarcomatous expression, which defines GS. [10],[11]

The current reported literature does provide a number of distinguishing clinical and pathogenetic features of PGS to suggest that they are indeed separate entities from GBMs. These differences include the GS predilection for the temporal lobe, their potential to appear similar to a meningioma grossly on operation, their increased metastatic potential, and the infrequency of epidermal growth factor receptor (EGFR) mutation. [9]

 
   References Top

1.
Stroebe H. Uber entstehung und bau der hirngliome. Beitr Pathol Anat 1895;18:405-86.  Back to cited text no. 1
    
2.
Pardo J, Murcia M, García F, Alvarado A. Gliosarcoma: A rare primary CNS tumor. Presentation of two cases. Rep Pract Oncol Radiother 2010;15:98-102.  Back to cited text no. 2
    
3.
Louis DN, Ohgaki H, Wiestler OD, Cavenee WK, Burger PC, Jouvet A, et al. The 2007 WHO classification of tumors of the central nervous system. Acta Neuropathol 2007;114:97-109.  Back to cited text no. 3
    
4.
Lutterbach J, Guttenberger R, Pagenstecher A. Gliosarcoma: A clinical study. Radiother Oncol 2001;61:57-64.  Back to cited text no. 4
    
5.
Perry JR, Ang LC, Bilbao JM, Muller PJ. Clinicopathologic features of primary and post irradiation cerebral gliosarcoma. Cancer 1995;75:2910-8.  Back to cited text no. 5
    
6.
Galanis E, Buckner JC, Dinapoli RP, Scheithauer BW, Jenkins RB, Wang CH, et al. Clinical outcome of gliosarcoma compared with glioblastoma multiforme: North Central Cancer Treatment Group results. J Neurosurg 1998;89:425-30.  Back to cited text no. 6
    
7.
Meis JM, Martz KL, Nelson JS. Mixed glioblastoma multiforme and sarcoma: A clinicopathologic study of 26 radiation therapy oncology group cases. Cancer 1990;67:2342-9.  Back to cited text no. 7
    
8.
Feigin IH, Gross SW. Sarcoma arising in glioblastoma of the brain. Am J Pathol 1955;31:633-53.  Back to cited text no. 8
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9.
Han SJ, Yang I, Tihan T, Prados MD, Parsa AT. Primary gliosarcoma: Key clinical and pathologic distinctions from glioblastoma with implications as a unique oncologic entity. J Neurooncol 2010;96:313-20.  Back to cited text no. 9
    
10.
Gray F, De Gironami U, Poirier J. Manual of Basic Neuropathology. 4 th ed. Elsevier; 2004. p. 26.  Back to cited text no. 10
    
11.
Louis DN, Ohgaki H, Wiestler OD, Cavanee WK. WHO Classification of Tumors of the Central Nervous System. Lyon, France: IARC Press 2007. p. 147-9.  Back to cited text no. 11
    

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Correspondence Address:
Sunil Y Swami
Bhagwanbaba Chowk, Gitta Road, Shepwadi, Ambajogai - 431 517, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/1755-6783.162396

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