A Case Report of Gliosarcoma in Young Female Adult

Maghous A, Marnouche EA, Zaghba N, Andaloussi K, Elmarjany M, Hadadi K, Sifat H and Mansouri H

Published on: 2018-12-28

Abstract

Gliosarcoma (GS) is a rare central nervous system (CNS) malignancy composed of both glial and sarcomatous components. It is considered as rare variant of glioblastoma multiforme (GBM) managed with the same sequencing of surgery, radiotherapy and chemotherapy. It is more common in fifth and sixth decade of life with slight male preponderance. Here we present a case report of young female adult treated at our institute.

Keywords

Gliosarcoma; Young female adult; Surgery; Chemotherapy; Radiotherapy

Introduction

Gliosarcoma (GS) is a rare primary tumor of the central nervous system (CNS) composed of both glial and malignant mesenchymal elements. It is considered as an uncommon histopathologic variant of glioblastoma multiforme (GBM) traditionally associated with a poor prognosis despite the same standardized maximal-safe surgical resection and adjuvant radiotherapy with concurrent and adjuvant temozolomide (TMZ)based chemotherapy. It is more common in fifth and sixth decade of life with slight male preponderance [1,2]. Here we present a case report of 36 years old female who presented with brain tumor which turned out to be GS after surgical resection and histopathological evaluation.

Case report

A 36-year-old female presented to neurosurgery department of our institute, with complaints of headache, vomiting and one history of recent generalized seizures. Magnetic resonance imaging (MRI) of brain showed heterogeneously enhancing right temporoparietal mass measuring 41 × 41 × 46 mm with perilesional edema and moderate mass effect shifting the left lateral ventricle (Figure 1). A left-side temporoparietal craniotomy and maximal macroscopic resection were performed without inducing neurological deficit. Then, immunohistochemistry (IHC) examination revealed gliosarcoma. Post-operative MRI showed a residual tumor with necrotic center and peripheral enhancement (Figure 2). The patient was then referred to our department for post-operative radiotherapy treatment using volumetric modulated arc therapy (VMAT) to a total dose of 60 Gy in 30 fractions (2 Gy per fraction). Based on the EORTC recommendation The Clinical
Target Volume (CTV) is defined as the resection cavity and residual enhancing regions on T1-sequences with the addition of a 20 mm margin including all regions of abnormal T2/FLAIR and edited to take into account anatomical barriers to tumor spread. The planning target volume (PTV) was created by adding geometric margins of 5 mm to the CTV (Figure 3). Concomitant temozolomide therapy at doses of 75 mg/m2 per day was administered with radiation. Now she is planned to adjuvant chemotherapy with temozolomide in dose of 150 mg/m2 for 5 days every 28 days for six cycles. Unfortunately, our patient died after 5 months before completion of adjuvant chemotherapy.

Figures

Figure 1: Axial T1-weighted contrast-enhanced MRI (a) and Flair MRI (b) showing a heterogeneously enhancing mass in the right temporoparietal region.

Figure 2: Axial T1-weighted contrast-enhanced (a) and Flair (b) early post-operative MRI showing a residual tumor with necrotic center and peripheral enhancement.

Figure 3: Sequential images of MRI and computed tomography scan dosimetry showing a conformal volumetric dose distribution by RapidArc radiotherapy.

Discussion

GS was first reported by Strobe in 1895 but did not gain wide acceptance until 1955 when Feigen and Gross defined as a subtype of glioblastoma [3, 4]. It represents a rare adult brain tumor and constitutes approximately 2% of all malignant gliomas [1]. Histologically it is an uncommon variant of glioblastoma characterized by biphasic compositions which are glial and malignant mesenchymal elements. While exact pathogenesis remains poorly understood, many studies have revealed shared mutations and cytogenetic abnormalities between gliomatous and sarcomatous elements of individual tumors [4-11]. GS is more common in fifth and sixth decade of life with slight male preponderance, although some cases of young and infantile gliosarcoma have also been described [1,12-15]. It is usually located in the supratentorial region with a small preference for the temporal lobes, followed by the frontal, parietal and occipital lobes [16,17]. Distant metastasis to lung, bone and lymph nodes has also been reported in the literature [18,19]. The standard treatment is the same as that administered for glioblastoma, especially maximal-safe surgical resection followed by adjuvant radiotherapy with concurrent and adjuvant temozolomide (TMZ)-based chemotherapy to improve overall survival [20,21]. Unfortunately, the prognosis for GS is generally poor with the mean survival of less than 8–24 months [16].

Abbreviations list

GS: Gliosarcoma; CNS: Central Nervous System; GBM: Glioblastoma Multiforme; TMZ: Temozolomide; MRI: Magnetic Resonance Imaging; IHC: Immunohistochemistry; VMAT: Volumetric Modulated Arc Therapy; CTV: Clinical Target Volume; PTV: Planning Target Volume

Conclusion

Gliosarcoma is a rare CNS malignancy. Here we have reported one case of 36 years old female gliosarcoma treated similarly to GBM but optimum treatment recommendations still not yet defined.

Journal of Cancer Research and Clinical Practice