Primary CNS Lymphoma in India: A 17-Year Experience From the All India Institute of Medical Sciences

Abstract

PURPOSE The information about the outcome of primary CNS lymphoma (PCNSL) in India is scarce, because there is no population-based or large hospital-based data. MATERIALS AND METHODS This is a retrospective study that spanned 17 years (2001 to 2017) to study the outcome of PCNSL at the All India Institute of Medical Sciences (AIIMS), which is a tertiary care center in Northern India. RESULTS Only one of 99 patients was positive for HIV serology. Diffuse large B-cell lymphoma was the most common histology (97.7%). The median patient age was 50 years (range, 13 to 70 years), and the ratio of men to women was 1.9. The median duration of symptoms before diagnosis was 3.5 months (range, 0.5 to 48 months), and 58.5% had a performance status (PS) of 3 or more. Multiple intracranial lesions were present in 81.8% of patients. Surgical resection was performed in 45%, and approximately 22% of patients were ineligible for treatment. Most patients (n = 73) were treated with high-dose methotrexate (HDMTX)–based regimens (ie, methotrexate, vincristine, and procarbazine with or without rituximab). Pharmacokinetic monitoring of methotrexate was not available at our center. HDMTX-related mortality was 3.9%. The median follow-up duration, event-free survival (EFS), and overall survival (OS) were 34 months, 20.4 months, and 31.7 months, respectively. Addition of rituximab (n = 27) to MVP resulted in a higher objective response rate (88.9% v 73.9% without rituximab; P = .12), complete remission (81.5% v 56.5%; P = .03), 2-year EFS (57.3% v 40.4%; P = .02), and 2-year OS (61.6% v 53.4%; P = .056). CONCLUSION This is the largest study of PCNSL from India. The patients were immunocompetent and young but presented with a high-burden disease that precluded treatment in approximately 22%. The treatment with HDMTX appears safe without pharmacokinetic monitoring. The outcome is comparable to those observed in the West, and rituximab use showed additional benefit. There are notable barriers with respect to management of PCNSL in the real world, and efforts are required to improve the outcome more.

Primary Central Nervous System Lymphoma: Focus on Indian Perspective

“…In Western settings, non-HIV infected PCNSL is typically diagnosed at 45-65 years of age (median age of diagnosis in the fifth decade) with no gender predilection [6,[17][18][19]. However, the Indian demographic differs in having a younger affected population (median age at diagnosis ranging from 42 to 59 years) with slight male preponderance [13,14,[20][21][22][23][24][25][26][27][28][29][30][31]. These changes are more likely from different population demographics rather than inherent disease biology, given that individuals aged more than 60 or 65 consistently amount to less than 9% and 7% of the total Indian population respectively [32].…”

“…In the Indian subcontinent, delayed health-seeking behavior combined with high initial misdiagnosis rates leads to a high disease burden at presentation. Median time from symptom onset to diagnosis is reported around 3.5-5 months in Indian studies [20,24,29,44], compared to 2.5-3 months in western studies [7,41].…”

“…Delayed health-seeking behavior is evident if we compare clinicoradiological features at index presentation. While two studies report focal neurological deficits as the most common presenting feature [20,29], most studies report headache from raised ICP as the index presentation [14, 21-23, 27, 28, 30, 31], which is typically a late feature in the western literature. Furthermore, while most western patients are ambulatory and capable of self-care at presentation [7], roughly 2/3rd of Indian patients are ECOG performance status (ECOG-PS) three or worse in retrospective [22,25,29] and prospective [44] studies.…”

“…Erroneous evaluation and emergent management are also common. Indian studies report misdiagnosis rates as high as 54% [20] to 100% [29], and inadvertent open surgical resection in 36-57% [20,44] of PCNSL patients compared to 25% in western literature. In addition, inadvertent steroids are given before diagnosis in up to 2/3rd patients referred from primary care [20] although prospective studies document only 9% requiring corticosteroid therapy for life-threatening indications [44].…”

“…Indian studies report modest outcomes. Up to 20% of PCNSL patients never receive therapy [20,22,29]. A single-institution reported a median EFS of 20.4 months and a median OS of 31.7 months at a median follow-up of 34 months with HD-MTX-based multiagent regimes and Rituximab and consolidative WBRT.…”