Das Shasanka, Bagal Bhausaheb, Jain Hasmukh, Kashyap Lakhan, Anbarasan Sekar, Abhishek Sharma, Bondili Suresh, Nayak Lingraj, Thorat Jayshree, Mirgh Sumeet, Gokarn Anant, Punatar Sachin, Ayushi Sahay, Epari Sridhar, Tembhare Prashant, Shetty Prakash, Khanna Nehal, Goda Jayant, Aliasgar Moiyadi, Gupta Tejpal, Sengar Manju, Khattry Navin, Laskar Siddhartha, Menon Hari
Department of Medical Oncology, Tata Memorial Centre, Mumbai, Maharashtra India.
Homi Bhabha National Institute, Mumbai, India.
Indian J Hematol Blood Transfus. 2023 Jan;39(1):57-70. doi: 10.1007/s12288-022-01557-7. Epub 2022 Sep 6.
Primary CNS lymphoma (PCNSL) is a rare subtype of non-Hodgkin lymphoma with the worst outcomes amongst all extranodal lymphomas. There is a scarcity of data on real-world outcomes of primary CNS lymphoma (PCNSL) owing to the rarity of the disease. This study analyzed the demographic patterns, risk stratification, treatment regimens used, & outcomes of patients treated at Tata Memorial Center Mumbai, India. This is a retrospective analysis of newly diagnosed primary CNS lymphoma patients treated at our centre over seven years from January 2013 to December 2019. A total of 142 patients with PCNSL were diagnosed during this period. Thirty (21.1%) patients were deemed ineligible for any systemic or local therapies,ten patients were referred to other hospitals, two patients had relapsed disease, and one was excluded because age less than 18 years. Finally 99 patients were included in the final analysis. Among these 99 patients,72 patients (72.7%) were < 60 years,70 (70.7%) patients had Eastern cooperative oncology group (ECOG) performance status (PS) less than equal to 2. DLBCL was the most common histology (86.4%) while rests were high grade B cell NHL NOS (11.4%),Burkitt's Lymphoma(1%),Peripheral T-cell Lymphoma NOS (1.2%). Only one of 99 patients was positive for HIV serology. Multiple intracranial lesions were found in 59.5%. Surgical resection was performed in 28.4% of patients. Out of 63 patients in whom the International extranodal lymphoma study group (IELSG) score is available, 34(54%) were IELSG high-risk groups. As per Memorial Sloan Kettering Cancer Center (MSKCC) risk grouping, patients were almost equally distributed in all the risk groups, with 32(32.3%) patients in risk group 1 (age < 50 years), 36(36.4%) patients in risk group 2 (age > 50 years, KPS > = 70), and 31(31.3%) patients in risk group 3 age > 50 years, KPS < 70). First-line treatment with high dose methotrexate (HD-MTX) based regimens was administered to 92 (92.9%) patients, and 72.8% of these patients received rituximab. Of these 92 patients, 59 (64.1%) patients could complete induction, and 52 patients received consolidation. Thirty-one patients received high dose cytarabine based chemo consolidation, one patient underwent high dose chemotherapy followed by autologous stem cell transplantation (ACST), and 19 patients received whole-brain radiotherapy (WBRT) and 1 patient received temozolomide as consolidation regimen. Thus only 52 patients completed the entire course of induction with consolidation therapy. The response to treatment was assessed using International PCNSL Collaborative Group Criteria. Post completion of consolidation, 49(94.2%) patients had a complete response. With a median follow-up duration of 39.2 months, the median progression-free survival (PFS) and the median overall survival (OS) of the patients taken into the analysis (N = 99) were 21 and 37 months respectively. On multivariate analysis, age < 60 yrs, > = 5 HD-MTX cycles received & the use of rituximab predicted better OS.Outcomes of patients with PCNSL treated with HD-MTX based therapy are comparable to reported literature however a large proportion of patients do not undergo required treatment despite the curable nature of disease.
The online version supplementary material available at 10.1007/s12288-022-01557-7.
原发性中枢神经系统淋巴瘤（PCNSL）是一种罕见的非霍奇金淋巴瘤亚型，在所有结外淋巴瘤中预后最差。由于该疾病罕见，关于原发性中枢神经系统淋巴瘤（PCNSL）真实世界预后的数据匮乏。本研究分析了在印度孟买塔塔纪念中心接受治疗的患者的人口统计学模式、风险分层、使用的治疗方案及预后。这是一项对2013年1月至2019年12月期间在我们中心新诊断的原发性中枢神经系统淋巴瘤患者的回顾性分析。在此期间共诊断出142例PCNSL患者。30例（21.1%）患者被认为不适合任何全身或局部治疗，10例患者被转诊至其他医院，2例患者有复发疾病，1例因年龄小于18岁被排除。最终99例患者纳入最终分析。在这99例患者中，72例（72.7%）年龄小于60岁，70例（70.7%）患者东部肿瘤协作组（ECOG）体能状态（PS）小于或等于2。弥漫大B细胞淋巴瘤（DLBCL）是最常见的组织学类型（86.4%），其余为高级别B细胞非特指型淋巴瘤（NHL NOS，11.4%）、伯基特淋巴瘤（1%）、外周T细胞非特指型淋巴瘤（1.2%）。99例患者中仅1例HIV血清学阳性。59.5%的患者发现有多个颅内病变。28.4%的患者接受了手术切除。在可获得国际结外淋巴瘤研究组（IELSG）评分的63例患者中，34例（54%）为IELSG高危组。根据纪念斯隆凯特琳癌症中心（MSKCC）风险分组，患者在所有风险组中分布几乎均等，风险组1（年龄小于50岁）有32例（32.3%）患者，风险组2（年龄大于50岁，KPS大于或等于70）有36例（36.4%）患者，风险组3（年龄大于50岁，KPS小于70）有31例（31.3%）患者。92例（92.9%）患者接受了基于大剂量甲氨蝶呤（HD-MTX）方案的一线治疗，其中72.8%的患者接受了利妥昔单抗治疗。在这92例患者中，59例（64.1%）患者能够完成诱导治疗，52例患者接受了巩固治疗。31例患者接受了基于大剂量阿糖胞苷的化疗巩固治疗，1例患者接受了大剂量化疗后行自体干细胞移植（ACST），19例患者接受了全脑放疗（WBRT），1例患者接受替莫唑胺作为巩固治疗方案。因此只有52例患者完成了诱导及巩固治疗的整个疗程。使用国际PCNSL协作组标准评估治疗反应。巩固治疗完成后，49例（94.2%）患者达到完全缓解。中位随访时间为39.2个月，纳入分析的患者（N = 99）的中位无进展生存期（PFS）和中位总生存期（OS）分别为21个月和37个月。多因素分析显示，年龄小于60岁、接受大于或等于5个HD-MTX周期治疗以及使用利妥昔单抗预示着更好的OS。接受基于HD-MTX治疗的PCNSL患者的预后与报道的文献相当，然而尽管该疾病具有可治愈性，但很大一部分患者未接受所需治疗。
在线版本补充材料可在10.1007/s12288-022-01557-7获取。
