Biswas Ahitagni, Kumar Narendra, Kumar Pankaj, Vasishta Rakesh Kumar, Gupta Kirti, Sharma Suresh C, Patel Firuza, Mathuriya Suresh Narain
Department of Radiotherapy & Oncology, Postgraduate Institute of Medical Education & Research, Chandigarh, India.
Br J Neurosurg. 2011 Dec;25(6):723-9. doi: 10.3109/02688697.2011.570881. Epub 2011 May 18.
We intended to assess the clinicopathological features and treatment outcome in patients of primary gliosarcoma, a rare malignant brain tumour.
Medical records were reviewed and data collected on primary gliosarcoma over an 8-year period (2002-2009) from the departmental archives. Overall survival (OS) was analysed by Kaplan-Meier method.
Seventeen patients met the study criterion (male:female = 9:8). Median age and performance status at presentation were 50 years and Karnofsky performance scale (KPS) 70, respectively. Symptoms of raised intracranial tension (in 100%) and motor impairment (in 64.7%) were commonly observed. Tumour location was frontal in four patients, temporal in three, parietal in three, thalamic in one, multilobed in five and multicentric in one. All patients underwent maximal safe surgery (total excision-10, near-total excision-2, subtotal excision and decompression-5). On histopathology, all tumours showed biphasic pattern, glial component positive for glial fibrillary acidic protein (GFAP) and mesenchymal component positive for vimentin and reticulin. Atypia, mitoses, necrosis and endothelial proliferation were identified in the glial component. Post-operative radiotherapy (median dose--60 Gy/30#/6 weeks) was used in 15 patients (88.2%). Concurrent and adjuvant chemotherapy with temozolomide (TMZ) were used in two patients depending upon affordability. After the completion of treatment, 35.3% patients were asymptomatic, 23.5% had symptomatic improvement, while 41.2% deteriorated. Salvage therapy for local recurrence was used in three patients (temporal lobectomy-1; total excision-1; TMZ+bevacizumab-1). At last follow-up (FU), eight patients were alive, seven patients dead and two patients lost to FU with symptom. Median overall survival in the evaluable patients (N = 15) was noted to be 8.27 months (6 month survival 60.76%).
Primary gliosarcoma, a variant of glioblastoma poses clinical challenge because of rarity, poor prognosis and limited experience. In our centre, principle of therapy is akin to that of glioblastoma--surgery followed by radiation along with concurrent and adjuvant TMZ. However, chemotherapy is often cost-prohibitive in our setting as mirrored by limited use (17.6%). Median survival of only 8.27 months in our series is in concert with the existing survival result of primary gliosarcoma in world literature (6.25-11.5 months).
我们旨在评估原发性胶质肉瘤患者的临床病理特征及治疗结果，原发性胶质肉瘤是一种罕见的恶性脑肿瘤。
回顾病历，并从科室档案中收集了8年期间（2002 - 2009年）原发性胶质肉瘤的数据。采用Kaplan-Meier法分析总生存期（OS）。
17例患者符合研究标准（男∶女 = 9∶8）。就诊时的中位年龄和体能状态分别为50岁和卡氏功能状态评分（KPS）70分。常见颅内压升高症状（100%）和运动障碍症状（64.7%）。肿瘤位于额叶4例，颞叶3例，顶叶3例，丘脑1例，多叶5例，多中心1例。所有患者均接受了最大安全手术（全切10例，近全切2例，次全切及减压5例）。组织病理学检查显示，所有肿瘤均呈双相模式，胶质成分胶质纤维酸性蛋白（GFAP）阳性，间充质成分波形蛋白和网硬蛋白阳性。在胶质成分中发现异型性、核分裂象、坏死和内皮细胞增生。15例患者（88.2%）术后接受了放疗（中位剂量 - 60 Gy/30次/6周）。根据经济承受能力，2例患者接受了替莫唑胺（TMZ）同步及辅助化疗。治疗结束后，35.3%的患者无症状，23.5%的患者症状改善，41.2%的患者病情恶化。3例患者接受了局部复发的挽救治疗（颞叶切除术1例；全切1例；TMZ + 贝伐单抗1例）。在最后随访时，8例患者存活，7例患者死亡，2例患者失访且有症状。可评估患者（N = 15）的中位总生存期为8.27个月（6个月生存率60.76%）。
原发性胶质肉瘤作为胶质母细胞瘤的一种变体，因其罕见性、预后差及经验有限而带来临床挑战。在我们中心，治疗原则与胶质母细胞瘤相似——手术 followed by radiation along with concurrent and adjuvant TMZ。然而，在我们的环境中，化疗通常费用高昂，如有限的使用情况（17.6%）所示。我们系列研究中仅8.27个月的中位生存期与世界文献中原发性胶质肉瘤现有的生存结果（6.25 - 11.5个月）一致。
最后一句中“surgery followed by radiation along with concurrent and adjuvant TMZ”翻译可能不太准确，原英文表述不太清晰，推测意思可能是“手术，随后是放疗以及同步和辅助的替莫唑胺治疗” 。
