Liverpool Hospital, Elizabeth Street, Liverpool, NSW 2170, Australia.
Neurological Intervention and Imaging Service (WA), Sir Charles Gairdner Hospital, Perth, WA, Australia.
J Clin Neurosci. 2014 Mar;21(3):478-81. doi: 10.1016/j.jocn.2013.07.042. Epub 2013 Oct 30.
Gliosarcoma (GSM) is a rare primary malignant brain tumour accounting for less than 0.5% of all intracranial tumours. It has a biphasic histological composition, demonstrating both gliomatous and sarcomatous elements. In clinical practice GSM are generally managed similarly to glioblastoma multiforme (GBM). However, unique features including its clinical propensity for extra-cranial metastasis, distinct radiological features and possible worse prognosis than GBM suggest that GSM may be a distinct clinico-pathological entity. Hence we reviewed patterns of care and outcomes for a series of Australian patients diagnosed with GSM in the era of combined chemo-radiotherapy. Patients were identified by searching the Australian Genomics and Clinical Outcomes of Glioma (AGOG) database and the Western Australian Interhospital Neurosurgical database. Nineteen patients with GSM were identified. Of these, 15 patients were diagnosed with primary GSM and four patients developed secondary GSM after radiation therapy for primary GBM. For comparative purposes, 408 primary GBM patients were identified from the AGOG database during the same study period. The overall median survival for all primary GSM patients was 9.7 months. In comparison the overall median survival for GBM patients recruited to the AGOG database over the same period was 12.2 months. The median survival for secondary GSM patients from the time of diagnosis was 5 months. Primary and secondary GSM pose a great clinical challenge due to their rarity. Our study adds further evidence to support GSM as a unique clinical entity with a likely worse prognosis than GBM.
胶质肉瘤（GSM）是一种罕见的原发性恶性脑肿瘤，占所有颅内肿瘤的比例不到 0.5%。它具有双相组织学组成，表现出神经胶质瘤和肉瘤成分。在临床实践中，GSM 通常与多形性胶质母细胞瘤（GBM）相似。然而，包括其向颅外转移的临床倾向、独特的影像学特征以及比 GBM 预后可能更差等独特特征表明，GSM 可能是一种独特的临床病理实体。因此，我们回顾了在联合化疗-放疗时代，一系列澳大利亚 GSM 患者的治疗模式和结局。通过搜索澳大利亚基因组学和神经胶质瘤临床结局（AGOG）数据库和西澳大利亚医院间神经外科数据库来识别患者。共确定了 19 例 GSM 患者。其中，15 例诊断为原发性 GSM，4 例患者在原发性 GBM 放疗后发展为继发性 GSM。为了进行比较，在同一研究期间，从 AGOG 数据库中确定了 408 例原发性 GBM 患者。所有原发性 GSM 患者的总体中位生存期为 9.7 个月。相比之下，在同一时期招募到 AGOG 数据库的 GBM 患者的总体中位生存期为 12.2 个月。继发性 GSM 患者从诊断时起的中位生存期为 5 个月。原发性和继发性 GSM 因其罕见性而带来了巨大的临床挑战。我们的研究进一步证明了 GSM 作为一种独特的临床实体，其预后可能比 GBM 更差。
