gliosarcoma ( gsm ) is a primary tumor of the brain composed of neoplastic glial cells in association with spindle celled sarcomatous elements . it was first described by stroebe but gained acceptance from detailed histological analyses of feigin and gross . the 2007 world health organization classification scheme places primary gsm as a grade 4 neoplasm and a variant of glioblastoma multiforme ( gbm ) . the relative frequency of pediatric gsm is 1.9% among glioblastomas and 0.5% among pediatric central nervous system tumors . ours is the second youngest case reported in literature and youngest with the lesion in the parieto - occipital region . a 5-year - old female child came to us with history of fever and loss of appetite since 2 weeks and right sided weakness since 4 days . she had history of generalized convulsion 3 months back and one more focal convulsion in right sided limbs 4 days back followed by same side limb weakness . there was history of headache that was holocranial and with morning exacerbation . on examination , magnetic resonance imaging ( mri ) showed a large heterogeneously enhancing space occupying lesion in the left parieto - occipital region causing significant mass effect and midline shift . the lesion was partly solid and partly cystic with rim enhancement and in close proximity to the occipital horn of the lateral ventricle [ figures 1 and 2 ] . the postcontrast axial image showing the large lesion in the left parieto - occipital region with peripheral contrast enhancement the postcontrast coronal image showing the lesion with mass effect and displacement of the lateral ventricle the patient was operated in right lateral position . a parieto - occipital craniotomy was performed , and the brain was found to be extremely tense . a small cortisectomy was done in the posterior parietal region and tumor identified [ figure 3 ] . the tumor was grayish in color , firm in consistency and had a good plane of cleavage from surrounding brain parenchyma . immunohistochemistry ( ihc ) was performed which was ihc - negative for glial fibrillary acidic protein and s-100 . the small cortisectomy through which piecemeal excision of the tumor was performed postoperatively patient improved in sensorium and right sided limb power . the patient underwent concurrent chemoradiation of 60 gy along with temozolomide 75 mg / m on all days of radiation . the patient had headache and lethargy on postoperative day 15 for which a repeat imaging was done which showed hydrocephalus . the patient was asymptomatic at 6 months followup . at 9 months following surgery , she got readmitted with drowsiness , decerebration , and respiratory tract infection . complete excision of the tumor with residual enhancement of the choroid plexus of the ventricle enlarged ventricles with ventriculoperitoneal shunt in situ the diagnosis of gsm should be made when a tumor contains an admixture of two distinctive neoplastic tissues . certain authors have suggested a presence of at least one atypical mesenchymal cell proliferation in one confluent medium power ( 10 objective with 10 eyepiece ) field for a diagnosis of gsm . earlier theory suggested that the sarcomatous component originates from neoplastic transformation of hyperplastic blood vessels found in high - grade glioma . the newer theory suggests monoclonal origin of both components of gsm with sarcomatous component originating via aberrant mesenchymal differentiation of the malignant glioma . this theory explains the absence of significant difference in clinical outcome between glioblastoma and gsm . even though several studies have showed no significant differences between gsm and gbm with regard to age , sex , size , clinical presentation , and median survival , they have been established to be two different pathologies . some studies showed a temporal lobe predilection for gsm whereas others found no such difference . the youngest case in literature of a 4-year - old child also showed tumor in the temporal lobe . there have been anecdotal reports of the neoplasm in varied locations such as posterior fossa . few studies have also found that gsm tends to be smaller at presentation as compared to gbm . in spite of these similarities , the two pathologies differ on intraoperative appearance ( gsm similar to meningioma due to its well - circumscribed nature ) , tendency for extraneural metastasis and infrequency of epidermal growth factor receptor mutations . gliosarcoma most commonly affects adults in the sixth or seventh decade of life and more commonly men ( male : female ratio of 1.41.8:1 ) . a total of 24 cases of pediatric gsms have been reported in the literature with a median age of 11 years with an increased incidence following exposure to rt . the presenting signs and symptoms are consistent with those of expanding space occupying lesion such as headache , hemiparesis , seizures , and cognitive decline . comparision of the two youngest cases of gliosarcoma reported in literature treatment modalities for gsm include tumor resection , postoperative rt and chemotherapy . the median survival among all gsm patients has been reported to be 9 months ; however , there are some variables that alter this . patients diagnosed prior to 50 years had a higher median survival period of 15 months as compared to 7 months for those diagnosed after age 50 . radical excision prolonged survival to 711 months as compared to 4 months with biopsy alone . although gliosarcomas occur most commonly in the sixth or seventh decade and have a temporal lobe predilection , they can occur in any age group or any region .
the relative frequency of pediatric gliosarcoma ( gsm ) is 1.9% among glioblastomas and 0.5% among pediatric central nervous system tumors . 
 a 5-year - old female child came to us with history of fever and loss of appetite since 2 weeks and right sided weakness since 4 days . 
 magnetic resonance imaging showed a large heterogeneously enhancing space occupying lesion in the left parieto - occipital region . 
 a parieto - occipital craniotomy with radical excision of tumor was performed . 
 the patient was given adjuvant therapy following surgery and survived until 9 months following surgery . 
 the etiopathogenesis , treatment modalities and prognosis of gsm is discussed .