====== Primary central nervous system lymphoma outcome ====== {{rss>https://pubmed.ncbi.nlm.nih.gov/rss/search/1XKgVAYRUbsED-vBBl80BcS32jzt6PRK9zozqubxqyJ-SE7wgc/?limit=15&utm_campaign=pubmed-2&fc=20230915143312}} The [[IPCG]] criteria are widely used in [[routine]] [[practice]] for the [[assessment]] of [[treatment]] [[response]] in [[Primary central nervous system lymphoma]]. However, the value of the IPCG criteria for ultimate [[clinical outcome prediction]] is largely unclear, mainly due to the uncertainty in delineating complete from partial [[response]]s during and after treatment. Lauer et al. explored various [[MRI]] [[feature]]s including semi-automated 3D [[tumor volume]] [[measurement]]s at different disease milestones and their association with [[survival]] in 93 CNSL patients undergoing curative-intent treatment. At diagnosis, patients with more than three [[lymphoma]] lesions, [[periventricular]] involvement, and high 3D tumor volumes showed significantly unfavorable [[PFS]] and [[OS]]. At first interim MRI during treatment, the IPCG criteria failed to discriminate outcomes in responding patients. Therefore, they randomized these patients into [[training]] and [[validation]] cohorts to investigate whether 3D tumor [[volumetry]] could improve outcome prediction. They identified a 3D tumor volume reduction of ≥97% as the optimal threshold for risk stratification (=3D early response, 3D_ER). Applied to the validation cohort, patients achieving 3D_ER had significantly superior outcomes. In multivariate analyses, 3D_ER was independently prognostic of PFS and OS. Finally, we leveraged prognostic information from 3D MRI features and circulating biomarkers to build a composite metric that further improved outcome prediction in CNSL. They developed semi-automated 3D tumor volume measurements as strong and independent early predictors of clinical outcomes in CNSL patients. These radiologic features could help improve risk stratification and help guide future treatment approaches ((Lauer EM, Riegler E, Mutter JA, Alig SK, Bleul S, Kuehn J, Ranganathan L, Klingler C, Demerath T, Würtemberger U, Rau A, Weiß J, Eisenblaetter M, Bamberg F, Prinz M, Finke J, Duyster J, Illerhaus G, Diehn M, Alizadeh AA, Schorb E, Reinacher PC, Scherer F. Improved early outcome prediction by MRI-based 3D tumor volume assessment in patients with CNS lymphomas. Neuro Oncol. 2023 Sep 15:noad177. doi: 10.1093/neuonc/noad177. Epub ahead of print. PMID: 37713267.)) The outcome of [[primary central nervous system lymphoma]] (PCNSL) varies and depends on several factors such as: Patient [[age]] and overall [[health status]] The size and location of the tumor The type of lymphoma The response to treatment The presence of other medical conditions. Generally, PCNSL is considered an aggressive form of cancer, and treatment usually involves a combination of chemotherapy and radiation therapy. The 5-year survival rate for PCNSL ranges from 20-50%. With improved treatment options and early diagnosis, the prognosis for PCNSL has improved in recent years. ---- The [[survival]] [[time]] for patients with [[Primary central nervous system lymphoma]] may be longer than previously thought, especially for patients with [[leptomeningeal seeding]] and lesions with hemorrhagic components Also, non-enhancing tumors may be less aggressive than enhancing tumors ((Huntoon K, Makary MS, Shah VS, Aquino A, Pandya V, Giglio P, Slone HW, Elder JB. Pretreatment findings on magnetic resonance imaging in primary central nervous system lymphoma may predict overall survival duration. Neuroradiol J. 2023 Jan 30:19714009231154681. doi: 10.1177/19714009231154681. Epub ahead of print. PMID: 36715098.)) ---- A difference in [[clinical response]] and [[PFS]] favored a more aggressive [[protocol]], but the [[toxicity]] of the multi[[agent]] combinations was significantly higher. The [[prognosis]] in younger was better than in older patients, with higher rates of clinical response, PFS, and [[OS]], although not statistically significant. Overall treatment outcomes are encouraging; however, there is a real need for an adaptive approach for older patients and balancing between the [[effectiveness]] and [[side effect]]s ((Zektser M, Rabinovich A, Grinbaum U, Porges T, Gozlan A, Gourevitch A, Al-Athamen K, Barrett O, Peles I, Kaisman-Elbaz T, Levi E. [[Primary Central Nervous System Lymphoma]]: Clinical Characteristics, [[Treatment]] [[Option]]s and Therapeutic [[Outcome]] in 36 Patients. A Single [[Center]] [[Experience]]. Isr Med Assoc J. 2022 Oct;24(10):654-660. PMID: 36309861.)). ---- With no treatment, median survival is 1.8–3.3 months following diagnosis. With [[radiation therapy]] ((O'Neill BP, Illig JJ. Primary Central Nervous System Lymphoma. Mayo Clin Proc. 1989; 64:1005–1020)) median survival is 10 months, with 47% 1-year median survival, and 16% 2-year median survival. 3-year survival is 8%, and 5-year survival is 3–4%. With intraventricular MTX, the median time to recurrence was 41 mos ((DeAngelis LM, Yahalom J, Thaler HT, et al. Combined Modality Therapy for Primary CNS Lymphomas. J Clin Oncol. 1992; 10:635–643)). Occasionally, prolonged survival may be seen ((Hochberg FH, Miller DC. Primary Central Nervous System Lymphoma. J Neurosurg. 1988; 68:835–853)) About 78% of cases recur, usually ≈ 15 months after treatment (late recurrences also are seen). Of these recurrences, 93% are confined to the CNS (often at another site if the original site responded well), and 7% are elsewhere. In AIDS-related cases, the prognosis appears worse. Although complete remission occurs in 20– 50% following XRT, the median survival is only 3–5 months ((Baumgartner JE, Rachlin JR, Beckstead JH, et al. Primary Central Nervous System Lymphomas: Natural History and Response to Radiation Therapy in 55 Patients with Acquired Immunodeficiency Syndrome. J Neurosurg. 1990; 73:206–211)) ((Formenti SC, Gill PS, Lean E, et al. Primary Central Nervous System Lymphoma in AIDS: Results of Radiation Therapy. Cancer. 1989; 63:1101–1107)) usually related to AIDS-related opportunistic infection. However, neurologic function and quality of life improve in ≈ 75% ((Baumgartner JE, Rachlin JR, Beckstead JH, et al. Primary Central Nervous System Lymphomas: Natural History and Response to Radiation Therapy in 55 Patients with Acquired Immunodeficiency Syndrome. J Neurosurg. 1990; 73:206–211)) Although there are individual studies that show trends, there are no prognostic features that consistently correlate with survival. The overall response rates and long-term survival of primary central nervous system lymphoma (PCNSL) are still significantly inferior to the results achieved in similar subtypes of extranodal non-Hodgkin's lymphoma. It is clearly necessary to investigate new therapeutic methods on PCNSL.