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  Table of Contents    
CASE REPORT  
Year : 2012  |  Volume : 55  |  Issue : 4  |  Page : 521-524
Pediatric gliosarcoma with fibrosarcomatous differentiation: Report of a rare case


1 Department of Pathology, Institute of Neurology, Madras Medical College, Chennai, India
2 Department of Pathology, Institute of Pathology, Madras Medical College, Chennai, India
3 Department of Medical Oncology, Madras Medical College, Chennai, India

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Date of Web Publication4-Mar-2013
 

   Abstract 

Gliosarcoma is a rare variant of glioblastoma with a biphasic pattern showing glial and mesenchymal differentiation. It is seen in adults during their fifth to sixth decades of life and is extremely rare in children. We report a case of primary gliosarcoma with fibrosarcomatous differentiation in an 11-year-old boy presenting with headache and vomiting. Imaging showed a contrast-enhancing isodense space-occupying lesion with areas of calcification in the right temporoparietal cortex. A total excision was done and, on histopathologic examination, a differential diagnostic consideration of gliosarcoma and teratoma with malignant transformation was made. After immunohistochemical analysis, a final diagnosis of gliosarcoma with fibrosarcomatous differentiation was then made. Primary gliosarcoma is a very rare tumor in children with a poor prognosis.

Keywords: Gliosarcoma, pediatric gliosarcoma, temporoparietal mass

How to cite this article:
Ravisankar S, Chander R V, Devadoss PK. Pediatric gliosarcoma with fibrosarcomatous differentiation: Report of a rare case. Indian J Pathol Microbiol 2012;55:521-4

How to cite this URL:
Ravisankar S, Chander R V, Devadoss PK. Pediatric gliosarcoma with fibrosarcomatous differentiation: Report of a rare case. Indian J Pathol Microbiol [serial online] 2012 [cited 2020 Sep 19];55:521-4. Available from: http://www.ijpmonline.org/text.asp?2012/55/4/521/107797



   Introduction Top


Gliosarcoma is a variant of glioblastoma with a biphasic pattern showing glial and mesenchymal differentiation. The sarcomatous areas commonly resemble a fibrosarcoma, but may also show a variety of lines of mesenchymal differentiation including bone, cartilage, or muscle. [1],[2],[3] Gliosarcoma commonly affects adults in fifth to sixth decades of life and is extremely rare in children. We report a case of primary gliosarcoma with fibrosarcomatous differentiation in an 11-year-old boy.


   Case Report Top


An 11-year-old male child presented to the out-patient department with headache and vomiting on and off for past 2 months. Computed tomography scan showed a contrast-enhancing isodense space-occupying lesion with areas of calcification in right temporoparietal cortex with surrounding edema, which was suggestive of meningioma. On magnetic resonance imaging, the lesion was T1 hypointense and T2 hyperintense with irregular enhancement on contrast [Figure 1].
Figure 1: (a) Computed tomography showing a contrast-enhancing isodense space-occupying lesion with calcification in right temporoparietal cortex. (b) Magnetic resonance imaging showing hyperintense lesion on T2-weighted image

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Craniotomy was done and part of the lesion was sent for squash cytology. On squash cytology, the smear appeared cellular showing discohesive sheets and clusters of pleomorphic oval to polygonal cells with abundant eosinophilic cytoplasm showing cytoplasmic vacuolation and marked nuclear atypia in a background of necrosis and hemorrhage. A suggestion of atypical teratoid/rhabdoid tumor was given [Figure 2].
Figure 2: Squash cytology

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Total excision of the space-occupying lesion was done and sent for histopathologic examination. Grossly, the specimen was received as multiple irregular gray-white soft tissue fragments, ranging from 0.5 × 0.5 cm to 4 × 3 × 2 cm, with an irregular and nodular external surface. Cut surface appeared variegated with gray tan areas, glistening areas, cystic areas, and hemorrhagic areas. Few calcified areas were also seen [Figure 3].
Figure 3: Gross appearance

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Hematoxylin and eosin stained sections revealed a highly cellular neoplasm composed predominantly of spindle-shaped cells with pleomorphic oval to elongated hyperchromatic nuclei. The cells were arranged in interdigitating fascicles and "herring bone" pattern in few foci with frequent mitoses. There were also foci showing deep-staining round cells arranged in small clusters and focal glandular pattern with areas of necrosis and hemorrhage. Few foci showing chondroid differentiation were also noted [Figure 4].
Figure 4: (a) Photomicrograph showing fascicles of spindle-shaped cells alternating with glial component (H and E, ×100). (b) Fibrosarcomatous cells with marked nuclear pleomorphism and frequent mitosis (H and E, ×400). (c) Few foci showed deep-staining round cells in small clusters and focal glandular formation (H and E, ×100). (d) Foci showing chondroid differentiation (H and E, ×100)

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A differential diagnosis of (1) gliosarcoma with leiomyosarcomatous differentiation and (2) teratoma with malignant transformation was made.

Immunostaining for Vimentin showed cytoplasmic positivity in 70% of the cells. Smooth muscle actin showed focal weak positivity in 30% of the cells. Immunostaining for S100, epithelial membrane antigen, myogenin, and glial fibrillary acidic protein was found to be negative [Figure 5].
Figure 5: (a) Vimentin positivity; (b) smooth muscle antigen focal weak positivity; (c) glial fibrillary acidic protein negativity

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A final diagnosis of gliosarcoma with fibrosarcomatous differentiation was then made.

The patient then underwent concurrent chemoradiation of 60 Gy along with temozolamide 75 mg/m 2 on all days of radiation and is presently on maintenance with temozolamide 150 mg/m 2 from day 1 to day 5 every 28 days for 6 cycles. He is currently on the second cycle of maintenance with a slight symptomatic improvement.


   Discussion Top


Gliosarcomas, which comprise 2-3% of glioblastomas, are rare biphasic tumors of the central nervous system, composed of alternating areas of glioblastomatous component admixed with sarcomatous component. They commonly affect adults in the fifth to sixth decades of life and are extremely uncommon in children, with a male:female ratio of 1.4:1 to 1.8:1. [4] The relative frequency of pediatric gliosarcoma is 1.9% among glioblastomas and 0.5% among pediatric central nervous system tumors. A total of 23 cases of pediatric gliosarcomas have been reported in the literature, with a median age of 11 years and male:female ratio of 1.2:1. [5] A few reports suggested a relatively higher incidence in infants and in patients with a previous history of radiotherapy.

They preferentially involve the temporal lobe followed by the frontal lobe and have a poor prognosis. The presenting signs and symptoms include a rapidly expanding intracranial tumor leading to aphasia, headache, hemiparesis, seizures, and cognitive decline, depending on the location. The clinical behavior of gliosarcomas is not significantly different from that of glioblastoma and both have overall poor survival rates. Gliosarcomas are associated with an increased likelihood of dissemination and extracranial metastases. [6]

On computed tomography scans, the lesions can appear with large necrotic areas and showing heterogeneous contrast enhancement, similar to glioblastoma multiforme or as a hyperdense lesion with well-defined margins and showing homogenous enhancement, similar to that of meningioma. [7]

Squash preparation shows a high-grade neoplasm with glial and mesenchymal elements. The glial component consists of pleomorphic round to oval cells and numerous gemistocytes in a fibrillary stroma. The mesenchymal components include fibrosarcoma-like, rhabdoid, osteoclastic giant cell, undifferentiated, along with heterologous components such as chondroid or osteoid tissue. A rich arborizing capillary network may be evident along with increased mitoses and areas of necrosis. [8] Atypical teratoid/rhabdoid tumor in squash smears shows largely discohesive primitive tumor cells with high affinity for blood vessels, along with rhabdoid cells, epithelioid cells, lipidized cells, and multi-nucleated cells. [9]

Macroscopically, the gliosarcoma shows a firm, well-circumscribed, and lobulated appearance similar to meningioma or sarcoma, and with a variegated, gritty, firm cut surface with areas of necrosis.

The histologic features include fascicles of sarcomatous component, usually resembling a fibrosarcoma or malignant fibrous histiocytoma, interspersed with areas of typical glioblastomatous component, thus creating a biphasic arrangement. Gliosarcomas with adenoid formations resembling a metastatic carcinoma, chondroid or osseous metaplasia, leiomyosarcomatous or rhabdomyosarcomatous elements have also been described. [1],[2],[3] Some cases may show a distinctive epithelial histology showing squamoid or glandular appearances which are immunonegative for glial fibrillary acidic protein (GFAP), thus creating not only diagnostic dilemmas, but also clinical management difficulties as to whether these areas represent metastasis or a primary manifestation of a high-grade glial neoplasm. [10]

Early reports have suggested that the sarcomatous components originated from the neoplastic transformation of the hyperplastic blood vessels which are commonly found in high-grade gliomas. [11] Recent reports suggest a monoclonal origin of both components of gliosarcoma, with the sarcomatous component arising from aberrant mesenchymal differentiation of the malignant glioma. [12]

Most extracranial metastases are reported in the lung and liver; intramedullary metastasis to the cervical spine has also been reported. [6]


   Conclusion Top


Primary gliosarcoma is a clinically challenging and very rare tumor in children and adults, with a poor prognosis in untreated cases. In our experience, this was the first case of primary gliosarcoma with fibrosarcomatous differentiation occurring in a child.

 
   References Top

1.Banerjee AK, Sharma BS, Kak VK, Ghatak NR. Gliosarcoma with cartilage formation. Cancer 1989;63:518-23.  Back to cited text no. 1
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2.Haddad SF, Moore SA, Schelper RL, Goeken JA. Smooth muscle can comprise the sarcomatous component of gliosarcomas. J Neuropathol Exp Neurol 1992;51:493-8.  Back to cited text no. 2
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3.Barnard RO, Bradford R, Scott T, Thomas DG. Gliomyosarcoma. Report of a case of rhabdomyosarcoma arising in a malignant glioma. Acta Neuropathol 1986;69:23-7.  Back to cited text no. 3
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4.Lutterbach J, Guttenberger R, Pagenstecher A. Gliosarcoma: A clinical study. Radiother Oncol 2001;61:57-64.  Back to cited text no. 4
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5.Karremann M, Rausche U, Fleischhack G, Nathrath M, Pietsch T, Kramm CM, et al. Clinical and epidemiological characteristics of pediatric gliosarcomas. J Neurooncol 2010;97:257-65.  Back to cited text no. 5
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6.Beaumont TL, Kupsky WJ, Barger GR, Sloan AE. Gliosarcoma with multiple extracranial metastases: Case report and review of the literature. J Neurooncol 2007;83:39-46.  Back to cited text no. 6
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7.Zhang BY, Chen H, Geng DY, Yin B, Li YX, Zhong P, et al. Computed tomography and magnetic resonance features of gliosarcoma: A study of 54 cases. J Comput Assist Tomogr 2011;35:667-73.  Back to cited text no. 7
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8.Parwani AV, Berman D, Burger PC, Ali SZ. Gliosarcoma: Cytopathologic characteristics on fine-needle aspiration (FNA) and intraoperative touch imprint. Diagn Cytopathol 2004;30:77-81.  Back to cited text no. 8
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9.Parwani AV, Stelow EB, Pambuccian SE, Burger PC, Ali SZ. Atypical teratoid/rhabdoid tumor of the brain: Cytopathologic characteristics and differential diagnosis. Cancer 2005;105:65-70.  Back to cited text no. 9
    
10.Ozolek JA, Finkelstein SD, Couce ME. Gliosarcoma with epithelial differentiation: Immunohistochemical and molecular characterization. A case report and review of the literature. Mod Pathol 2004;17:739-45.  Back to cited text no. 10
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11.Feigin I, Allen LB, Lipkin L, Gross SW. The endothelial hyperplasia of the cerebral blood vessels with brain tumors, and its sarcomatous transformation. Cancer 1958;11:264-77.  Back to cited text no. 11
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12.Biernat W, Aguzzi A, Sure U, Grant JW, Kleihues P, Hegi ME. Identical mutations of the p53 tumor suppressor gene in the gliomatous and the sarcomatous components of gliosarcomas suggest a common origin from glial cells. J Neuropathol Exp Neurol 1995;54:651-6.  Back to cited text no. 12
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Correspondence Address:
Shantha Ravisankar
Department of Pathology, Institute of Neurology, Madras Medical College, Chennai - 600 003
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.107797

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    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]

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