74例颅内生殖细胞瘤放疗疗效分析

目的 分析颅内生殖细胞瘤的放疗疗效.方法 搜集2007年11月前接近18年内收治的颅内生殖细胞瘤病例74例,其中男35例,女39例,中位年龄15(5～45)岁.放疗前病理诊断9例,余65例20 Gy放疗后MRI显示病灶均明显缩小(>50%)或消失为临床诊断.应用6 MV X线全脑全脊髓放疗加局部补量照射、全脑放疗加局部补量照射、全脑室放疗加局部补量或肿瘤区局部照射,原发病灶区38.5～50.0 Gy,全脑或全脑室18～25 Gy,全脊髓21～25 Gy,分割剂量1.6～2.0Gy/次,5次/周.结果 中位随访时间80(12～168)个月,总随访率为97%,10年随访例数和随访率分别为14例和19%.1、5、10年总生存率和无复发生存率分别为99%和97%、96%和90%、93%和83%.共9例患者治疗失败,6例为照射野内复发,3例为照射野外转移.照射野内复发者中肿瘤剂量<40 Gy者3例.38例患者放疗后有不同程度的垂体前叶功能低下,部分需强的松等激素替代治疗.结论 放疗是颅内生殖细胞瘤的主要治疗手段,其照射剂量、范围要根据病灶数目、脑脊液检查等结果 来决定.     

Radiation therapy for benign central nervous system disease

The most common indication for the use of radiation therapy in the treatment of benign central nervous system disease is for the treatment of benign brain tumors, such as meningioma, pituitary adenoma, acoustic neuroma, arteriovenous malformation, and craniopharyngioma. Other less common benign intracranial tumors treated with radiation include chordoma, pilocytic astrocytoma, pineocytoma, choroid-plexus papilloma, hemangioblastoma, and temporal bone chemodectomas. Benign conditions, such as histiocytosis X, trigeminal neuralgia, and epilepsy, are also amenable to radiation treatment. There have also been reports of radiosurgery being used for the treatment of movement disorders and psychiatric disturbances, such as obsessive-compulsive and anxiety disorders. For benign brain tumors, radiation therapy as either primary or adjuvant therapy plays an integral role in improving local control. In the treatment of trigeminal neuralgia, epilepsy, tremor, and some psychiatric disturbances, radiosurgery may help ameliorate or eliminate some symptoms. Patients with benign central nervous system disease are expected to live a long time. As such, treatment should be highly conformal and based on three-dimensional planning using magnetic resonance imaging, computed tomography, or both. It is critical that damage to normal brain be minimized.     

原发性中枢神经系统淋巴瘤的治疗

原发性中枢神经系统淋巴瘤(PCNSL)是一种较少见的中枢神经系统恶性肿瘤,总体预后欠佳,主要治疗方法包括手术、放疗和化疗.立体定向活检术以其微创、便捷的优点,已经成为确诊PCNSL的常规方法.全脑放疗是多病灶性PCNSL的标准化治疗方法,可短期内延缓肿瘤进展.以大剂量甲氨蝶呤为基础的治疗方案大大改善了PCNSL的治疗效果,成为PCNSL的有效治疗措施.有效的综合治疗是延长PCNSL患者生存期和改善生命质量的关键.     

Combined-modality therapy for primary central nervous system lymphoma: long-term data from a Phase II multicenter study (Trans-Tasman Radiation Oncology Group)

PURPOSE: To assess, in a multicenter setting, the long-term outcomes of a brief course of high-dose methotrexate followed by radiotherapy for patients with primary central nervous system lymphoma (PCNSL). METHODS AND MATERIALS: Forty-six patients were entered in a Phase II protocol consisting of methotrexate (1 g/m(2) on Days 1 and 8), followed by whole-brain irradiation (45-50.4 Gy). The median follow-up time was 7 years, with a minimum follow-up of 5 years. RESULTS: The 5-year survival estimate was 37% (+/-14%, 95% confidence interval [CI]), with progression-free survival being 36% (+/-15%, 95% CI), and median survival 36 months. Of the original 46 patients, 10 were alive, all without evidence of disease recurrence. A total of 11 patients have developed neurotoxicity, with the actuarial risk being 30% (+/-18%, 95% CI) at 5 years but continuing to increase. For patients aged>60 years the risk of neurotoxicity at 7 years was 58% (+/-30%, 95% CI). CONCLUSION: Combined-modality therapy, based on high-dose methotrexate, results in improved survival outcomes in PCNSL. The risk of neurotoxicity for patients aged>60 years is unacceptable with this regimen, although survival outcomes for patients aged>60 years were higher than in many other series.     

原发中枢神经系统淋巴瘤研究进展

原发中枢神经系统淋巴瘤(PCNSL)是原发于颅内的结外非霍奇金淋巴瘤,是一种罕见的高侵袭性淋巴瘤,预后较差.近年来,关于PCNSL的治疗方案尚无定论,以往的治疗包括手术、放疗、化疗等.目前大多认为综合治疗可以提高患者的生存率,而联合化疗药物的选择和预防性鞘内注射化疗药物在其治疗中占有重要地位.     

CNS germinoma: disease control and long-term functional outcome for 12 children treated with craniospinal irradiation

Purpose: To provide evidence that radiation therapy alone in the form of craniospinal irradiation (CSI) and a boost to the primary site of disease provides effective disease control and limited additional morbidity for patients with CNS germinoma.

Methods and Materials: Twelve patients with a median age of 12 years (range 9–16 years) with CNS germinoma were treated with CSI (median 25.6 Gy, range 23.4–32 Gy) and a boost to the primary site of disease (50.4 Gy, range 45–54 Gy) between January 1987 and June 1998. All patients were biopsied prior to radiation therapy and none received chemotherapy. No patients were lost to follow-up and the majority had long-term (> 45 month) pre- and postirradiation endocrine and psychology assessment.

Results: All 12 patients are alive and no failures have occurred with a median follow-up of 69 months (range 14–143 months). Preirradiation endocrine deficiencies were present in 6 of 6 suprasellar tumors and 1 of 6 pineal tumors; with follow-up there was no substantial difference between age and gender adjusted pre- and postirradiation stature and weight. With long-term follow-up, there were no significant differences between pre- and postirradiation full-scale, verbal, and performance IQ scores.

Conclusions: This study confirms the ability of radiation therapy alone to achieve disease control with a high rate of success in pediatric patients and demonstrates that the treatment toxicity faced by these patients may be less than anticipated. Because these patients present with substantial preexisting morbidity at diagnosis and may be of an age where the potential for radiation-related side effects is relatively small, the superiority of treatment alternatives may be difficult to prove.     

原发性中枢神经系统淋巴瘤的临床特征分析

 目的　分析原发性中枢神经系统淋巴瘤（PCNSL）的临床特征,探讨影响疾病的预后因素,并对不同的治疗方案进行评价。方法　回顾性分析初发PCNSL患者的临床资料、治疗经过及随访结果,应用Log-rank进行单因素分析,应用COX回归模型进行生存资料的多因素分析。结果　共收集PCNSL初发病例64例,中位年龄54.9岁,男性多于女性,肿瘤单发62 %（40／64）,深部病变占54 %（33／61）。在我科诊治的具有完整治疗资料的患者26例,其中19例患者初始治疗为单纯化疗,6例为全颅放疗（WBRT）后1个月进行化疗,1例患者初治时仅行WBRT。中位生存时间为17个月,血红蛋白≥9 g／L患者的生存时间长于血红蛋白＜9 g／L患者。年龄>60岁、性别、体能状态、病变部位等因素对预后无明显影响。应用含大剂量甲氨蝶呤（HD-MTX）或替尼泊苷的方案化疗者的预后优于未使用者,化疗联合放疗可能有助于改善患者的预后（χ2＝3.24,P＝0.07）,应用CHOP方案（环磷酰胺、多柔比星、长春新碱、泼尼松）、利妥昔单抗、鞘内注射化疗药物等与预后关系不大;多因素分析提示HD-MTX是影响PCNSL患者生存时间的独立有利因素,颅内病灶部位、病灶的多少、是否联合放化疗等均不是影响预后的独立因素。结论　PCNSL预后较差,应用HD-MTX、替尼泊苷等药物可改善患者的预后,贫血尤其中重度贫血患者预后不良。     

Cisplatin-based chemotherapy in primary central nervous system germ cell tumors

We report a retrospective review of our experience with cisplatin-based chemotherapy in eight patients (ages 9–44 years) with histologically confirmed primary central nervous system germ cell tumors. Five patients received chemotherapy as the primary treatment, radiation therapy being administered either at completion of chemotherapy or between chemotherapy courses. Three patients received cisplatin-based chemotherapy for recurrent disease after prior radiation therapy and/or surgery. Four of five patients treated with chemotherapy at diagnosis are in complete remission at 11–14 months from diagnosis. The remaining patient twice achieved complete remission prior to dying of progressive disease 16 months after diagnosis. Two of three patients treated with chemotherapy for recurrent disease are in complete remission at 20 and 26 months; the remaining patient deteriorated after the first cycle of chemotherapy and expired six months thereafter. Overall, of seven patients evaluable for response, five achieved complete remission with chemotherapy alone, and two with chemotherapy and radiation therapy. Our results confirm previous reports of high complete remission rates utilizing cisplatin-based chemotherapy in conjunction with radiation therapy. Prospective evaluation of cisplatin-based chemotherapy followed by radiation therapy is warranted.     

原发性中枢神经系统淋巴瘤预后相关因素研究进展

 原发性中枢神经系统淋巴瘤（PCNSL）是一种少见的结外恶性淋巴瘤,好发于免疫缺陷人群,但近年来在免疫功能正常人群中发病率逐年提高。其临床治疗缓解期短,易复发,预后不佳。研究发现PCNSL的预后相关因素包括：年龄、体力状态、放化疗的方式、初治对糖皮质激素的反应、组织病理和分子生物学标志等。     

原发中枢神经系统淋巴瘤诊疗进展

原发中枢神经系统淋巴瘤是一种少见的中枢神经系统恶性肿瘤,以大剂量甲氨蝶呤为基础方案诱导化疗是目前一线治疗,随后全脑放疗作为巩固治疗.迟发神经认知功能障碍出现,尤其是老年人,使缓解患者是否需要全脑放疗或减量放疗需要进一步明确.利妥昔单抗治疗PCNSL,初步研究表明美罗华可能给患者带来获益,但研究证据水平较低.HDC/ASCT对于治疗复发性或难治性PCNSL虽有疗效但毒副作用较大,临床价值尚存争议.     

原发中枢神经系统淋巴瘤放疗作用观察

目的 探索放疗在原发中枢神经系统淋巴瘤治疗中的作用。方法 回顾分析2010年9月至2017年12月确诊的免疫功能正常的原发中枢神经系统淋巴瘤60例资料,其中50例经由手术或立体定向活检后病理诊断,10例影像学临床诊断。52例患者接受了化疗,其中45例为大剂量甲氨喋呤为主方案,25例为含利妥昔单抗方案。27例患者行计划性放疗,33例未行计划性放疗,其中治疗失败后9例接受了挽救性放疗。结果 中位随访时间28个月(5~70个月)。全组中位生存、中位无进展生存期分别为22个月(5~65个月)、13个月(5~55个月),4年总生存率、无进展生存率分别为61%、33%。计划性放疗组、非计划性放疗组4年总生存率分别为68%、54%(P=0.083),无进展生存率分别为47%、20%(P=0.014)。挽救性放疗组与计划性放疗组4年总生存率差异无统计学意义(P=0.398),全脑放疗≤36Gy、>36Gy组4年总生存率差异也无统计学意义(P=0.136)。结论 放疗是原发中枢神经系统淋巴瘤的综合治疗的一部分,计划性放疗可能使患者在综合治疗中获益,较高的照射剂量不能使患者获益。     

原发中枢神经系统淋巴瘤的治疗

原发中枢神经系统淋巴瘤(PCNSL)是一种少见的非霍奇金淋巴瘤(NHL),其治疗观念在过去的20年中发生了巨大的改变,现总结探索其治疗方式的研究工作.     

原发性中枢神经系统淋巴瘤治疗进展

原发性中枢神经系统淋巴瘤(PCNSL)的发病率近年来上升明显,如何提高其治疗效果备受关注.随着放疗策略和化疗策略的改变以及新化疗药物的应用,PCNSL的治疗取得了新的进展.     

Role of radiation therapy in primary central nervous system lymphoma

Journal of Neuro-Oncology - We analyzed patterns of care and outcomes for patients with primary central nervous system lymphoma (PCNSL) in this multi-institutional retrospective study. Between...     

Efficacy and safety of larotrectinib in TRK fusion-positive primary central nervous system tumors

BackgroundLarotrectinib is a first-in-class, highly selective tropomyosin receptor kinase (TRK) inhibitor approved to treat adult and pediatric patients with TRK fusion-positive cancer. The aim of this study was to evaluate the efficacy and safety of larotrectinib in patients with TRK fusion-positive primary central nervous system (CNS) tumors.MethodsPatients with TRK fusion-positive primary CNS tumors from two clinical trials ({"type":"clinical-trial","attrs":{"text":"NCT02637687","term_id":"NCT02637687"}}NCT02637687, {"type":"clinical-trial","attrs":{"text":"NCT02576431","term_id":"NCT02576431"}}NCT02576431) were identified. The primary endpoint was investigator-assessed objective response rate (ORR).ResultsAs of July 2020, 33 patients with TRK fusion-positive CNS tumors were identified (median age: 8.9 years; range: 1.3–79.0). The most common histologies were high-grade glioma (HGG; n = 19) and low-grade glioma (LGG; n = 8). ORR was 30% (95% confidence interval [CI]: 16–49) for all patients. The 24-week disease control rate was 73% (95% CI: 54–87). Twenty-three of 28 patients (82%) with measurable disease had tumor shrinkage. The 12-month rates for duration of response, progression-free survival, and overall survival were 75% (95% CI: 45–100), 56% (95% CI: 38–74), and 85% (95% CI: 71–99), respectively. Median time to response was 1.9 months (range 1.0–3.8 months). Duration of treatment ranged from 1.2–31.3+ months. Treatment-related adverse events were reported for 20 patients, with grade 3–4 in 3 patients. No new safety signals were identified.ConclusionsIn patients with TRK fusion-positive CNS tumors, larotrectinib demonstrated rapid and durable responses, high disease control rate, and a favorable safety profile.     

第一原发性舌癌和鼻咽癌放疗后第二原发性舌癌的比较分析

目的探讨放疗后第二原发性舌癌的临床特点及治疗效果。方法回顾性分析53例鼻咽癌放疗后第二原发性舌癌和252例第一原发性舌癌的临床和随访资料,比较其临床特点、治疗及预后。Kaplan-Meier方法计算累计生存率,Cox比例风险模型进行多因素分析。结果第二原发性舌癌与第一原发性舌癌患者的平均发病年龄、性别、原发灶大小、cTNM分期、肿瘤组织分化程度和远处转移发生率差异无统计学意义,而两组5年生存率、原发灶位置、临床和病理淋巴结转移率差异有统计学意义。多因素分析显示,患者年龄、颈淋巴结清扫、cTNM分期是影响舌癌预后的独立因素。结论鼻咽癌放疗后第二原发性舌癌在原发灶发生部位、临床和病理淋巴结转移率等方面有自身的特点,其预后差于第一原发性舌癌;但鼻咽癌放疗史不是影响舌癌患者预后的独立因素,对鼻咽癌放疗后第二原发性舌癌患者行手术或包含手术的综合治疗可能有利于其预后。     

Prognostic implication of p27 expression in primary central nervous system lymphoma

To clarify the role of p27/Kip1 (p27) in primary central nervous system lymphomas (PCNSLs), we examined p27 expression by immunohistochemical methods in a series of 22 patients with PCNSL. We attempted to correlate the expression of p27 with proliferation potential and prognosis. Although the MIB-1 labeling index (LI) was lower in tumors with low p27 expression (26.7% ± 17.2% vs 38.1% ± 16.3%), it was not significantly different from that of tumors with high p27 expression (P = 0.1253). Survival analysis revealed that high p27 expression was significantly associated with poorer overall prognosis (P = 0.0011); however, the MIB-1 LI were not associated with prognosis. Our results suggest p27 as a predictor of prognosis in patients with PCNSL.