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Table of Contents
CASE REPORT
Year : 2019  |  Volume : 2  |  Issue : 2  |  Page : 122-125

A rare case of gliosarcoma after acute hemorrhage


1 Department of Neurology, Faculty of Medicine, Sriwijaya University, Mohammad Hoesin Hospital, Palembang, Indonesia
2 Department of Neurosurgery, Faculty of Medicine, Sriwijaya University, Mohammad Hoesin Hospital, Palembang, Indonesia
3 Department of Internal Medicine, Hemato-Oncology Division, Faculty of Medicine, Sriwijaya University, Mohammad Hoesin Hospital, Palembang, Indonesia
4 Department of Radiology, Faculty of Medicine, Sriwijaya University, Mohammad Hoesin Hospital, Palembang, Indonesia
5 Department of Pathology Anatomy, Faculty of Medicine, Sriwijaya University, Mohammad Hoesin Hospital, Palembang, Indonesia
6 Department of Radiation Oncology, Faculty of Medicine, Sriwijaya University, Mohammad Hoesin Hospital, Palembang, Indonesia

Date of Web Publication27-Jun-2019

Correspondence Address:
Dr. Yunni Diansari
Department of Neurology, Faculty of Medicine, Sriwijaya University, Mohammad Hoesin Hospital, Palembang
Indonesia
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/glioma.glioma_7_19

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  Abstract 

Gliosarcoma (GSM) is a rare primary neoplasm of the brain, characterized by a biphasic tissue pattern with alternating areas displaying glial and mesenchymal differentiation. GSM is usually treated as glioblastoma multiforme (GBM) and the prognosis is poor. Here, we discuss a 50-year-old male presenting with sudden decreased consciousness and a history of recent headache. A noncontrasted computed tomography of the brain revealed a well-circumscribed, hyperdense lesion in the left frontal lobe with significant perilesional edema, suggestive of an intracranial hemorrhage. The patient underwent emergency craniotomy to evacuate the hematoma. Intraoperatively, a mass was present, and fluid around the mass was collected and analyzed. Cytology revealed the presence of malignant cells. A 1-week postmagnetic resonance imaging (MRI) revealed a heterogenously enhancing mass in the left frontal lobe, suggestive of a high-grade glioma. The patient was then treated with radiation followed by adjuvant treatment with temozolomide plus bevacizumab. One year after the surgery, the patient was readmitted with headache, right-sided hemiparesis, and seizure. A follow-up MRI found a residual mass in the same region. A second surgery was performed. Histopathology examination showed GSM, and this was confirmed with an immunohistochemistry panel including glial fibrillary acid protein and vimentin. GSM is a rare variant of GBM. Intracranial hemorrhage is an uncommon clinical presentation of GSM. Diagnosis is established by histopathology. Treatment is surgery, followed by radiotherapy and chemotherapy. The study was approved by the Health Research Review Committee of Mohammad Hoesin Central General Hospital and Faculty of Medicine Sriwijaya University (No. 089/kepkrsmhunsri/2019) on March 1, 2019.

Keywords: Biphasic pattern, glioblastoma multiforme, gliosarcoma, histopathology finding, intracranial hemorrhage, poor prognosis, progression


How to cite this article:
Diansari Y, Mutiara S, Baraqbah H, Syahrir M, Marsinta H, Erwin A, Andriani D. A rare case of gliosarcoma after acute hemorrhage. Glioma 2019;2:122-5

How to cite this URL:
Diansari Y, Mutiara S, Baraqbah H, Syahrir M, Marsinta H, Erwin A, Andriani D. A rare case of gliosarcoma after acute hemorrhage. Glioma [serial online] 2019 [cited 2022 Nov 28];2:122-5. Available from: http://www.jglioma.com/text.asp?2019/2/2/122/261677


  Introduction Top


Gliosarcoma (GSM) is a glioblastoma multiforme (GBM) variant. Histopathology of GSM reveals a combination of a glial component and a sarcomatous component (biphasic pattern).[1],[2] GSM is reported to comprise 2%–8% of all cases of GBM.[3],[4] GSM is more frequently found in the age group between 50 and 70 years and is more common in males.[3],[4],[5],[6] Clinical symptoms are varied depending on location and tumor size. GSM is an aggressive tumor and is usually treated similar to GBM, with tumor resection, followed by postoperative radiation and chemotherapy.[7]

Intracranial hemorrhage can be a presentation in high-grade glioma, with varied reported frequency. However, there are only a few reported cases of GSM presenting with acute intracranial hemorrhage. Here, we report a rare case of GSM presenting with intracranial hemorrhage.


  Case Report Top


A 50-year-old male was hospitalized due to a sudden decrease of consciousness. There was a history of previous headache. Moreover, noncontrasted head computed tomography (CT) revealed an intraparenchymal hemorrhage with a volume of around 90 mL in the left frontal lobe [Figure 1]A. An emergency craniotomy was performed to evacuate the hematoma. A mass was found during the operation, and tumor fluid was obtained for analysis. Cytology examination showed a population of cells consisting of cells with a high nucleus: Cytoplasm ratio and an eosinophilic cytoplasm concerning for malignant cells. One week after the operation, a follow-up magnetic resonance imaging (MRI) showed a heterogenously enhancing lesion in the left frontal lobe with unclear border and peritumor edema indicative of a GBM [Figure 1]B.
Figure 1: Neuroimages of the patient with gliosarcoma. (A) Noncontrast head computed tomography scan at the first presentation shows a large hemorrhage in the left frontal lobe (black arrow) with significant mass effect. (B) Axial T1 postcontrast head magnetic resonance imaging shows a lobulated mass (black arrows) with heterogenous enhancement a week after hematoma evacuation. (C) Coronal T1 postcontrast magnetic resonance imaging 1 year after the first presentation with new clinical symptoms shows a heterogenous enhancing cystic mass (white arrow). (D) Head computed tomography scan after reresection shows residual lesion (black arrow) with enhancement

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The patient underwent 30-fraction radiotherapy with total doses of 60 Gy, followed by chemotherapy with temozolomide at 150 mg/m2 in combination with bevacizumab at 5 mg/kg for six cycles. During follow-up, a head CT was done, and the tumor mass was still visible. The patient condition was stable without any new neurological deficits. Chest X-ray and abdominal ultrasound were performed to evaluate for extracranial metastasis.

Less than 1 year after the first diagnosis, the patient complained of recurrent and worsening headache associated with seizure. Right-sided hemiparesis was appreciated on examination. An MRI revealed a heterogeneously enhancing mass with cystic component and associated perimass edema in the right and left frontal lobes [Figure 1]C. A second surgery was performed, and follow-up contrast CT showed residual tumor [Figure 1]D. Histopathology examination revealed GSM appearance [Figure 2]A, which was supported by the immunohistochemistry examination, including glial fibrillary acid protein (GFP) [Figure 2]B and vimentin [Figure 2]C. The diagnosis of GSM was established. After the operation, the patient could perform daily activities with help from a caregiver. Twelve months after the diagnosis, the patient died of tumor progression.
Figure 2: Photomicrographs of the tumor. (A) Tumor characterized by a biphasic tissue pattern, showing alternating glioma tissue (red arrows) and sarcomatous tissue (black arrow) (hematoxylin-eosin staining, original magnification, 200×). (B) The gliomatous component is positive for glial fibrillary acid protein immunochemistry stain (original magnification, 200×). (C) The sarcomatous component is vimentin positive (original magnification, 200×)

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The study was approved by the Health Research Review Committee of Mohammad Hoesin Central General Hospital and Faculty of Medicine Sriwijaya University (No. 089/kepkrsmhunsri/2019) on March 1, 2019. The patient signed the written informed consent.


  Discussion Top


GSM is a rare primary malignant brain tumor, occurring in approximately 0.5% of all intracranial tumors.[6] GSM could be found as primary malignancy (de novo) or secondary tumor from dedifferentiation from a previous intracranial neoplasm. Patient characteristics and management of GSM are quite similar to those of GBM. Both malignancies are more frequent in older males. GSM tends to have a more temporal lobe predilection compared to GBM. A cohort study reported that the incidence of GSM is more frequently found in frontal and temporal regions.[8],[9],[10],[11]

GSM is clinically indistinguishable from GBM. The clinical profile of GSM is also similar to GBM. The most common symptoms are focal neurological deficits, tonic or clonic seizures, and other symptoms reflecting the location and increased intracranial pressure such as weakness, headache, confusion, nausea, vomiting, blurry vision, lethargy, ataxia, altered mental status, and others.

GBM and other metastatic brain tumors, such as melanoma, can bleed spontaneously. GSM can present with intracranial hemorrhage, but few cases have been reported. The diagnosis of a brain tumor can be delayed in the setting of intracranial hemorrhage, especially if the hemorrhage is large. In such cases, the tumor mass is often masked by the hemorrhage on imaging, and definite diagnosis can only be made after surgery and histopathological examination. The degree of vasogenic edema surrounding an acute hematoma with underlying neoplasm may be more pronounced at initial imaging than in the setting of hemorrhage associated with hypertension, which is typically located in the basal ganglia. In high-grade malignancy, the extensive and abnormal vascularity are considered predisposing factors for spontaneous hemorrhage.[12],[13],[14]

Imaging characteristic of GSM is dependent on the predominant tissue component. With a predominant gliomatous component, the imaging features are similar to those of GBM. Imaging (CT or MRI) usually reveals large and irregular lesions with indistinct margins and surrounding edema and mass effect. Foci of hemorrhage, necrosis, and cyst formation are common. However, several cases with a sarcomatous predominant, presented as a well-demarcated hyperdense mass and homogenous contrast enhancement, mimicking meningioma.[7],[9],[15]

A definite diagnosis of GSM is established after histological examination of the surgical specimen. Histologically, GSM has a biphasic tumor pattern consisting of gliomatous and mesenchymal regions of cells. The gliomatous region in GSM is similar to that found in GBM. GFP expression is high in gliomatous regions and low in sarcomatous regions. Vimentin is a marker for mesenchymal cells, and high vimentin expression is found in sarcomatous regions, whereas there is no staining in gliomatous regions.[1],[2]

Due to the similarity between GSM and GBM, the treatment of GSM is identical to GBM, which consists of surgical resection, postoperative radiotherapy and chemotherapy. When possible, a gross total resection is recommended in patients with a primary glial tumor and GSM. As in GBM, the more tumor resected in GSM leads to improved patient survival.[16]

Radiotherapy has been reported to improve survival following surgical resection of GSM, according to one of the earliest reviews of outcomes in a cohort of 24 GSM patients.[3] Total dose ranges from 40 to 80 Gy.[4],[5] Perry et al.[17] demonstrated improved survival in patients undergoing postoperative radiation compared to those who were kept under observation after surgery (10.6 months versus 6.25 months). Other series by Kevin et al.[8] suggested that tumor excision, as opposed to biopsy only, and adjuvant radiotherapy offered a better outcome.

Although chemotherapy with temozolomide (TMZ) is now the standard of care for GBM, the exact role of chemotherapy in GSM is still uncertain. In the study by Han et al.,[9] 10 patients who received TMZ combined with RT showed no significant difference in survival from another 10 patients who received radiotherapy with or without other chemotherapy. However, Salvati et al.[18] and Walker et al.[19] showed that TMZ was beneficial. Moreover, Singh et al.[20] reported 14 cases with a mean overall survival of 18.5 months with TMZ and suggested that TMZ should be included in GSM therapy. Several optional modalities such as gene therapy, angiogenesis inhibitors, and other antineoplasmatic agents are under investigation.[11],[15],[20]

GSM is one of the GBM variants that have a predilection for extracranial metastasis.[5],[21],[22],[23] Therefore, clinical systemic evaluation and several extracranial diagnostic examinations are needed to detect extracranial metastasis.

The prognosis for patients with GSM is equally poor as for those with GBM. The survival rate of GSM patients untreated is around 4 months.[3] The median survival in GSM patients ranges from 4 to 11.5 months from the previous studies.[4],[5],[21] Additional radiation therapy offered an improvement in median survival from 6.25 months to 10.6 months in one study.[17] Kozak et al.[8] reported worse prognosis in patients with GSM than GBM. Zhang et al.[7] reported a mean overall survival of 13.4 months for GSM compared to 15.8 months for GBM. This finding showed that the overall survival for GSM patients is worse than GBM as other retrospective studies have shown.[24],[25] However, the overall survival rate of our patient was 20 months after presentation.


  Conclusion Top


GSM is a variant of GBM. They have similar clinical presentation, imaging, and tumor characteristics. Unlike GBM, GSM infrequently presents with intracranial hemorrhage. Treatment of GSM is similar to that of GBM. A multidisciplinary approach in patients with GSM is crucial to maximize survival.

Financial support and sponsorship

Nil.

Institutional review board statement

The study was approved by Health Research Review Committee of Mohammad Hoesin Central General Hospital and Faculty of Medicine Sriwijaya University (No. 089/kepkrsmhunsri/2019) on March 1, 2019.

Conflicts of interest

There are no conflicts of interest.

Declaration of patient consent

The authors certify that they have obtained the appropriate patient consent form. In the form, the patient has given his consent for his images and other clinical information to be reported in journal. The patient understands that his name and initials will not be published and due efforts will be made to conceal his identity.



 
  References Top

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