Primary gliosarcoma: key clinical and pathologic distinctions from glioblastoma with implications as a unique oncologic entity

Abstract

This report presents the historical experience, clinical presentation, treatment, prognosis, and pathogenesis of gliosarcoma described to date in the English literature. PubMed query of term “gliosarcoma” was performed, followed by a rigorous review of cited literature. Articles selected for analysis included: (1) case reports of gliosarcoma, (2) review articles of gliosarcoma, and (3) studies of the pathogenesis or genetics of gliosarcoma in humans. Our review identified 219 cases of gliosarcoma in 34 reports and eight articles addressing the pathogenesis. Survival in larger series ranged 4–11.5 months. Features unique to gliosarcoma compared to glioblastoma (GBM) include their temporal lobe predilection, potential to appear similar to a meningioma at surgery, repeated reports of extracranial metastases, and infrequency of EGFR mutations. Published experience is limited to small case series, and the pathogenesis remains unclear. Clinical and pathologic characteristics distinct from GBM suggest that they may warrant specific treatment, separate from conventional GBM therapy.

Established and emerging variants of glioblastoma multiforme: review of morphological and molecular features

“…GS manifestation in the spinal cord has also been reported [23]. Survival may be greater for GS with meningioma-like features vs. GBM-like features [48]. Also in this meta-analysis, GS tumours showed infrequent EGFR mutations unlike their GBM counterpart and suggested that the role for radiotherapy and chemotherapy treatments continues to be uncertain due to limited data and poor understanding of this entity.…”

“…Also reminiscent of GS, lipidized glioblastoma, termed lipoglioblastoma, has also been described as a rare malignant tumour with significant foamy cell presence [62,159]. While early reports of GS suggested the concept of a "collision tumour" with vascular dysplasia resembling sarcomatous features, current models suggest a monoclonal cell of origin or CSC with distinct genetic drivers of GS [35,48].…”

Neurosurgical Management of Gliomas

“…There are distinct gross macroscopic features described: a firm, well circumscribed lesion; and an infiltrative poorly defined lesion. Treatment and prognosis is similar as for GBM (Han et al, 2010). Giant cell GBM, another rare subtype, is treated in a similar fashion.…”

A rare central nervous system tumor: Gliosarcoma, Case report

“…In 1955, Feige et al was defined gliosarcoma as a variant of the glioblastoma which have sarcomatous areas with vascular proliferation. 6,7 . In our study, located in the right frontal intracranial tumor which was diagnosed as gliosarcoma according to morphological and immunohistochemical findings, are presented.…”