Indian Journal of Pathology and Microbiology

CASE REPORT
Year
: 2012  |  Volume : 55  |  Issue : 4  |  Page : 521--524

Pediatric gliosarcoma with fibrosarcomatous differentiation: Report of a rare case


Shantha Ravisankar1, R Vimal Chander2, Prem Kumar Devadoss3,  
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

Correspondence Address:
Shantha Ravisankar
Department of Pathology, Institute of Neurology, Madras Medical College, Chennai - 600 003
India

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.



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;55:521-524


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 Oct 22 ];55:521-524
Available from: https://www.ijpmonline.org/text.asp?2012/55/4/521/107797


Full Text

 Introduction



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



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}

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}

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}

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 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 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



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



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.

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