|Year : 2016 | Volume
| Issue : 3 | Page : 148-150
Gliosarcoma: A rare variant of glioblastoma multiforme
Banyameen Mohamad Iqbal, Jehan Nizam Ansari, Iqra Mushtaq
Department of Pathology, Dr. D. Y. Patil Medical College, Hospital and Research Centre, Pune, Maharashtra, India
|Date of Web Publication||14-Dec-2016|
Banyameen Mohamad Iqbal
Department of Pathology, Dr. D. Y. Patil Medical College, Hospital and Research Centre, Pimpri, Pune - 411 018, Maharashtra
Source of Support: None, Conflict of Interest: None
The World Health Organization described gliosarcoma (GS) as a rare variant of glioblastoma multiforme. It is a biphasic neoplasm consisting of both gliomatous and sarcomatous elements. It was first described by Stroebe in 1895. Its peak incidence is in fourth to sixth decade of life with increased male to female ratio. We are presenting a case report of a 48-year-old male patient who presented to the hospital with signs and symptoms of headache, vomiting, and weakness in both upper and lower limbs. Computed tomography scan and magnetic resonance imaging revealed a mass lesion in the left posterior parietal lobe. The tumor was excised, and histopathology examination confirmed the diagnosis of GS.
Keywords: Biphasic brain tumor, brain tumor, malignant brain tumors
|How to cite this article:|
Iqbal BM, Ansari JN, Mushtaq I. Gliosarcoma: A rare variant of glioblastoma multiforme. Saudi J Health Sci 2016;5:148-50
| Introduction|| |
Gliosarcoma (GS) is a rare primary neoplasm of the central nervous system (CNS) classified by the World Health Organization as a variant of glioblastoma multiforme (GBM). It is a mixed neoplasm composed of both gliomatous and sarcomatous elements.  It was first described by Stroebe in 1895  and increasingly recognized by Feigin and Gross in 1955  by detailed histological analyses. GS corresponds to <2% of all glioblastomas with a peak incidence in fourth to sixth decades of life with a mean age being 53 years. Male:female ratio is 1.8:1, and the clinical presentation, natural history, and radiologic profile are similar to those of primary glioblastoma.  Initially, GS was considered a collision between two independent tumors in which the sarcomatous part was believed to come from the proliferation of the vascular component.  However, recent immunohistochemical and genetic studies failed to support this theory, suggesting a monoclonal origin for both histological components. 
| Case Report|| |
A 48-year-old male patient presented to the hospital with 1 month history of headache, which was severe at times and partly relieved on taking analgesics. He complained of nausea and a few episodes of vomiting few days back with increased severity of headache not responding to analgesics. He also had an episode of altered sensorium after vomiting which relieved of its own. The patient complained of weakness in the right upper and lower limbs for the last 5 days. On admission, his consciousness was clear, and Glasgow Coma Scale score was 15/15, reflexes were normal, and power was Grade 2 in the right upper limb, Grade 3 in the left upper limb, and Grade 4 in both lower limbs. He was advised a computed tomography scan of the brain which revealed a well-circumscribed heterogeneous isodense to hyperdense lesion measuring 4.5 cm in its maximal diameter in the left posterior parietal lobe with significant perilesional edema, mass effect, and midline shift. Magnetic resonance imaging (MRI) of the brain revealed a lesion in the posterior parietal lobe which appears to be a heterogeneous mass both in T1- and T2-weighted images, with strong peripheral enhancement and central hemorrhage and/or necrosis [Figure 1]a and b. His blood examination revealed a total leukocyte count of 19,000/cumm with 80% polymorphs and 15% lymphocytes. Other than his erythrocyte sedimentation rate which was 45 mm in 1 st h, all other investigations were within normal limits. The lesion was excised and sent for histopathological examination. Macroscopically, it was a relatively well-circumscribed and firm mass with areas of necrosis and hemorrhage. Microscopic features revealed biphasic tumor morphology with some round to oval tumor cells (astrocytes) showing pleomorphic, hyperchromatic nuclei [Figure 2]a while others were spindle shaped and arranged in fascicles [Figure 2]b. A high mitotic rate was observed. Large areas of tumor necrosis and neural elements were also seen along with endothelial cell proliferation [Figure 3]. Collections of foamy macrophages and inflammatory cells were seen focally. Reticulin stain shows deposition of collagen/reticulin fibers around the tumor cells [Figure 4]a. Glial fibrillary acidic protein (GFAP) stain shows cytoplasmic positivity in many tumor cells [Figure 4]b. Based on these findings a diagnosis of GS was made. The patient was doing comparatively better after surgery as his symptoms were relieved. He was scheduled for radiotherapy which, however, he refused. He was subsequently lost to follow-up after 6 months.
|Figure 1: (a and b) Magnetic resonance imaging of the brain revealing a lesion in the posterior parietal lobe which appears to be a heterogeneous mass both in T1- and T2-weighted images, with strong peripheral enhancement and central hemorrhage and/or necrosis|
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|Figure 2: Photomicrograph showing (a) round to oval tumor cells (gliomatous component) with pleomorphic, hyperchromatic nuclei and (b) spindle-shaped cells arranged in fascicles (sarcomatous component) (H and E, ×40)|
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|Figure 3: Photomicrograph showing large areas of tumor necrosis (white arrows) and neural elements (black arrows) (H and E, ×40)|
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|Figure 4: Photomicrograph showing (a) reticulin stain with deposition of collagen/reticulin fibers around the tumor cells (arrows) and (b) glial fibrillary acidic protein stain with cytoplasmic positivity in tumor cells (H and E, ×40)|
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| Discussion|| |
GS is an uncommon and highly malignant tumor of the CNS. It is a rare subtype of GBM. The WHO classification identified GS as a Grade 4 neoplasm. Its clinical presentation, prognosis, and therapy are similar to that of GBM.  Clinical manifestation of GS includes effects due to increased intracranial tension, such as headache and vomiting. Some patients may complain of aphasia, hemiparesis, and seizures depending on tumor location.  GS usually occurs in the cerebral hemisphere and involves the temporal, frontal, parietal, and occipital lobe. Rarely cases of GS have been reported in the posterior fossa or spinal cord while a few reports recorded a higher rate of incidence in the frontal lobe. Histogenesis of GS is not fully understood, however a number of possible mechanisms for GS histogenesis such as the transformation theory, which states that "part of a glial tumor is dedifferentiated to primitive cells and transformed into sarcomatous components, support the idea that radiation therapy might induce a dedifferentiation of the glial cell to the sarcomatous cells in cases with previous CNS tumors."  However, in our case, no prior radiation therapy was given. Histologically, GS is composed of two separate malignant cell populations (biphasic tissue pattern), one component being gliomatous (heterogeneous infiltrative areas with hemorrhage and necrosis and stains for GFAP compatible with GBM) and the other with malignant mesenchymal differentiation. Reis et al., assessing the genetic profile of 19 GS patients, have found identical PTEN mutation, p53 nuclear accumulation, p16 deletion, and CDK4 amplification in both tumor areas.  Recently, Actor et al. have reported that 57% of all chromosomal imbalances detected by comparative genomic hybridization of eight GS were shared by both components. These authors have also detected identical p53 mutations in both glial and sarcomatous areas in 13 of 38 patients. They also described epidermal growth factor receptor overexpression in 8% of 38 tumors.  GS progresses very rapidly and has a poor prognosis. The mean survival time of GS has been reported from 4 to 18 months only while, if untreated, the mean survival is only 4 months.  Treatment is same as that for GBM. Surgical resection followed by radiotherapy/chemotherapy depending on the clinical status of the patient.
The aim to present this case report is to make the clinicians/pathologists aware of this rare and highly neoplastic variant of GBM so that early diagnosis and treatment can be planned and the age of survival of the patients increased.
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Conflicts of interest
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]