Annals of Tropical Medicine and Public Health

LETTER TO THE EDITOR
Year
: 2015  |  Volume : 8  |  Issue : 4  |  Page : 138--140

Primary gliosarcoma: A case report


Sunil Y Swami, Ashwini D Shendarkar, Grace D'Costa 
 Department of Pathology, Swami Ramanand Teerth Rural Government Medical College, Ambajogai, Maharashtra, India

Correspondence Address:
Sunil Y Swami
Bhagwanbaba Chowk, Gitta Road, Shepwadi, Ambajogai - 431 517, Maharashtra
India




How to cite this article:
Swami SY, Shendarkar AD, D'Costa G. Primary gliosarcoma: A case report.Ann Trop Med Public Health 2015;8:138-140


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 Apr 8 ];8:138-140
Available from: http://www.atmph.org/text.asp?2015/8/4/138/162396


Full Text

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}{Figure 2}

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}{Figure 4}{Figure 5}{Figure 6}

 Discussion



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



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

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10Gray F, De Gironami U, Poirier J. Manual of Basic Neuropathology. 4 th ed. Elsevier; 2004. p. 26.
11Louis DN, Ohgaki H, Wiestler OD, Cavanee WK. WHO Classification of Tumors of the Central Nervous System. Lyon, France: IARC Press 2007. p. 147-9.